CME Developmental Field Reassignment in Unilateral Cleft

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Developmental ﬁeld reassignment in unilateral cleft lip: Reconstruction of the premaxilla

Michael H. Carstens Division of Plastic Surgery, Saint Louis University, USA

Address for correspondence: Michael H Carstens, Division of Plastic Surgery, Saint Louis University, USA. E-mail: [email protected]

n the 21st century the greatest stimulus for progress identifiable developmental fields. Fields are discrete units in cleft surgery will come from more a more accurate composed of ectoderm, mesoderm, endoderm and neural model of facial development and how clefts originate. crest. At each level of the embryo all germ layers specific to I [1] Victor Veau accurately predicted this: “All cleft surgery that level can be referenced back to developmental zones is merely applied embryology.” The revolution in of the neuraxis known as a neuromeres. These are named developmental biology has not yet been incorporated into using the nomenclature of neuroembryology. The master surgical practice. The drawings and terminology used in plan of the entire embryo is determined by 6 prosomeres textbooks today are based on the work of Wilhelm His in (prosencephalon-for.com).ebrain), 2 mesomeres (mesencephalon the 1870’s.[2] Cleft repairs are therefore designed based on midbrain), 12 rhombomeres (rhomboencephalon-hindbrain the anatomy as it appears in the newborn. Measurements and 31 myelomeres (spinal cord).[5-7] are taken and the tissues are geometrically manipulated. But the anatomy of a cleft as seen at the time of birth is far RUBENSTEIN different from its original configuration in the embryonic face. From its onset at gastrulation, the clefting event.medknow Applications of neuromeric anatomy provide a potential unleashes pathologic processes that predictably alter the embryonic “map” of all craniofacial structures with original embryonic anatomy over time to produce what important implications for diagnosis and surgery. Exclusive we recognize at term as a cleft lip. Left uncorrected,(www these of the cranial base (basisphenoid and posterior) and processes will remain operative throughout facial growth. parietal bone, the craniofacial skeleton is made exclusively This explains why geometric cleft repairs relapse over from neural crest. Thus the cell populations producing the time, requiring Thisrevision. PDFa site is hosted available by forMedknow freeethmoid, download pr Publicationsesphenoid, pr fromemaxilla and vomer all originate in antero-posterior order from the neural folds in genetic Developmental field repair (DFR) is based upon the register with the 1st rhombomere (abbreviated r1). The neuromeric model of craniofacial embryology.[3-4] The inferior turbinate, palatine bone, maxilla, alisphenoid goals of DFR surgery are: (1) resolution of all pathologic (greater wing) and zygoma arise from the neural crest processes of clefting (deficiency, displacement, division of the 2nd rhombomere (r2). The squamous temporal, and distortion); (2) dissection along embryonic separation mandible, malleus and incus are r3 neural crest bones. planes (subperiosteal release); (3) preservation of blood supply to the alveolar mucoperiosteum; (4) primary Non-neural crest craniofacial bones come from paraxial unification of the dental arch; and (5) reassignment of all mesoderm (PAM) lying immediately adjacent to the neural developmental fields to their correct relationships. Before tube. PAM is divided into individual units shaped like describing the surgical technique, certain basic concepts popcorn balls called somitomeres, each one in register with of field theory should be understood. its corresponding neuromere. The first seven somitomeres (Sm) contain the myoblasts for all muscles of the orbit Craniofacial anatomy results from the assembly of specific, and the first three pharyngeal arches. For example, the

75 Indian J Plast Surg January-June 2007 Vol 40 Issue 1 Carstens mandible comes from r3 neural crest and all muscles presents the reconstructive application of these principles originating from it arise from Sm3. Beginning with Sm8 all to the surgery of labiomaxillary clefts. somitomeres undergo a further transformation into somites, each having a dermatome, myotome and sclerotome. The The pathologic anatomy of cleft formation first four somites (derived from Sm8-Sm11) produce the The pathologic anatomy of unilateral and bilateral cranial base posterior to the sphenoid, the muscles of the labiomaxillary clefts stems from a tissue deficiency state tongue and part of the sternocleidomastoid and trapezius. localized to the lower lateral piriform fossa. The tissue at These are called occipital somites. fault is the mesenchyme of the ipsilateral premaxilla. Neural crest stem cells responsible for synthesis of the presphenoid Disturbances at a particular neuromeric level can affect and ethmoid arise from the mesencephalic neural folds and individual or multiple fields to be deficient or absent. are genetically identified with the 1st rhombomere (r1). Note Thus, isolated cleft palate (unassociated with cleft lip) that the basisphenoid is not neural crest in origin; it comes represents a deficiency state of the vomer. This occurs as from paraxial mesoderm from somitomere 1 (Sm1). Sm1 lies a spectrum. As the vomer is progressively smaller, it lifts just outside the neural tube at level r1 and is in register with away from the plane of the palatal shelves and the cleft it. Immediately caudal to this population are neural crest extends forward toward the incisive foramen. In mild cells immediately above the rostral rhomboencephalon in cases of cleft palate associated with Pierre Robin sequence, register with the 2nd rhombomere (r2). The most rostral a reduction in the horizontal plate of the r2 palatine bone zone of r2 (herein referred to as r2’) is the likely source is seen. Soft palate muscles are consequently normal material for the vomer and PMx. The more caudal zone of but divided. As the pathology worsens, reduction in the r2 produces inferior turbinate (IT), palatine bone (P), maxilla horizontal plate of the r2 maxilla creates the well-known (Mx), alisphenoid (AS) and zygoma (Z). “horse-shoe” palate cleft. Submucous cleft palate, on the other hand, involves pathology in the 3rd pharyngeal arch. These cell populations migrate forward into the Somitomeres 6 and 7 contain the myoblasts of levator, developing.com). face in a strict temporo-spatial order. The uvulus, palatopharyngeus and superior constrictor. These sphenoid is laid down first, followed by the ethmoid. In can be globally affected. Frequently, persistent VPI follows like manner, formation of PMx is the prerequisite for the a seemingly simple palatoplasty, requiring further surgery. appearance of V. Formation of the PMx and V requires the Failure to stratify cleft palate by embryologic mechanism pre-existence of the perpendicular plate of the ethmoid explains much of the confusion currently extent in the speech (PPE). The function of the PPE is to provide a cellular and surgical literature. .medknowscaffold by which r2’ neural crest cells can reach the midline.[10] [Figures 1-2] In holoprosencephaly (HPE) the Finally. Treacher-Collins syndrome provides an example PPE can be absent. PMx and V cannot develop correctly; in which all r2 developmental fields of the midface(www are a wide bilateral cleft results.[11-13] affected: the maxillary, palatine and zygomatic bones are all small. The septum, vomer and premaxilla (being The piriform fossa of humans and some high primates r1 structures) arThise unaffected. PDFa site For isthis hosted available reason, the by central forMedknow freeis assembled download Publications as the fusion from of the frontal process of the midface projects normally while the dimensions of the premaxilla (PMxF) and the frontal process of the maxilla palate, maxilla and zygoma are constricted. (MxF). In all other vertebrate skulls PMxF and MxF are readily visible as two distinct entities.[12] Evolution Developmental fields form in a specific spatio-temporal foreshortened the human snout. The two fields became sequence. Each one builds upon its predecessors. superimposed, PMxF becoming telescoped internal to MxF. Making a face is much akin to assembling a house with This lamination is responsible for the strength of the magical pieces of Lego®, each one of which will grow piriform rim. Plating of the piriform “buttress” in fracture over time. Imagine a Lego house made from 20 pieces (4 repairs takes advantage of this bicortical anatomy. on the floor and 5 stories high). All pieces are growing independently. If a cornerstone piece is removed, the 19 PIRIFORM remaining pieces undergo a deformation and the house tilts into the deficiency site. The missing Lego piece in cleft The developmental field in which the PMx resides consists lip is the premaxilla. The physiologic basis of DFR is the of an epithelium and a mesenchyme. Formation of the reconstruction of the premaxillary field.[8-10] This chapter PMx results from interactions between these tissues. The

Indian J Plast Surg January-June 2007 Vol 40 Issue 1 76 Developmental ﬁeld reassignment in unilateral cleft lip

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Figure 1: Developmental ›eld map of the face. All neuromeres are color coded. Neural crest cells forming ethmoid, presphenoid, vomer and premaxilla share common embryologic origin from 1st rhombomere (violet). Maxilla, zygoma, alisphenoid come from 2nd rhombomere (blue). Nostril sill is union of r1 and r2. All craniofacial sutures contain neural crest cells. Craniofacial ›elds are neuroembryologic and follow suture boundaries premaxilla has several anatomic subcomponents, these are epithelium corresponding to PmxA and then “spills over” into assembled in a strict sequence.[14-15] In dental terminology zone PMxB. The time sequence of dental eruption (central the central incisor is called “A” and the lateral incisor is incisor A > lateral incisor B) is a manifestation of the relative called “B.” A erupts before B. Therefore the central incisor biologic “maturity” of the mesenchymal field within which field (PMxA) is biologically “older” than the lateral incisor each tooth develops. The frontal process field (PMxF) is a field (PMxB). Neural crest mesenchyme flows forward vertical offshoot of PMxB; this subfield is the biologically along the previously-established PPE. It first encounters the “newest” tissue.

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Pathology affecting the PMx occurs as a spectrum based mesoderm from the 2nd somitomere forms the anterior on this original developmental pattern. A deficiency half of the squamous temporal bone and the cranial half state of the PMx will first occur in the most distal aspect of the parotid gland. All derivatives from level r2 can be of the frontal process (ie. at its most cranial extent). As mapped out within the sensory distribution of V2, the the mesenchymal deficit worsens, frontal process will nucleus of which resides within r2.[19-24] be reduced in a cranial-caudal gradient. “Scooping out” of the piriform rim results; the nasal lining is pulled The developmental anatomy of the nose, prolabium down as well. This causes depression of the alar base and premaxilla has been previously described in terms and a downward-lateral displacement of the lateral crus. of neuromeric theory by this author.[3-4,8,9] In contrast to Biologic signals from PMxF regulate epithelial stability the rest of the body, all dermis and submucosa of the head and therefore affect lip formation (vide infra). When the originates from neural crest cells, not from a dermatome signal strength is minimally disturbed the lip is normal associated with a somite.[25] Pre-dermal neural crest arises despite the piriform distortion. Therefore the forme fruste from three distinct zones of the embryo. The dermis manifestation of premaxillary deficiency is a cleft lip nose of the forehead, nose and vestibular lining come from with a perfectly normal lip.[16] the caudal prosencephalon (above prosomeres p4-p1). This prosencephalic neural crest (PNC) migrates forward Once the frontal process is eliminated, the deficiency like a gigantic glacier to occupy the neural folds above state shows up in the lateral incisor field. Progressive prosomeres p6 and p5. The alar half of p6 and p5 creates degrees of PMx deficiency in the lateral incisor field the telencephalon (cerebrum). The basal halves of p6 and cause incremental loss of alveolar bone. Normal alveolar p5 plus all remaining prosomeres (p4-p1) synthesize the development follows a gradient. It begins at the incisive diencephalon (epithalamus, thalamus and hypothalamus). foramen and progresses forward. Mild deficiency causes The neural folds above p6 and p5 are “sterile.” They notching on the labial surface. As the deficiency worsens contain the pituitary, olfactory and optic placodes, but the notch deepens backward toward the incisive foramen. no neural cr.com).est. When PNC flows forward into this zone A critical lack of alveolar bone mass results in outright the placodes are activated and dermis is formed. Nasal failure of lateral incisor development. vestibular lining from the cribriform plate forward to the internal nasal valve comes from p6 PNC. All remaining BMP-4 signals from this field are directly implicated in the frontonasal dermis from the internal nasal valve forward mechanism of fusion between the lateral lip element and to the hairline comes from p5 PNC. the prolabium.[17-18] BMP-4 emanating from PmxL forms.medknow a cranio-caudal chemical gradient. The strength of this PNC skin shares sensory innervation with the dura of the gradient depends upon the total amount of available BMP- underlying frontal lobe. V1, the sensory nerve for this 4; this in turn is proportional to the overall mesenchymal(www common zone has its nucleus with the 1st rhombomere mass of PMxB. Reduction in mass of the lateral incisor (r1). The neuroanatomy is analogous for the rest of the field results in a diminution of the total BMP-4 signal. Lip face. Rhombomeres r2 and r3 contain neurons supplying fusion follows thisThis same PDF gradient.a site is Mildhosted available weakness by of theforMedknow freeall skin download and Publications dura inner vatedfrom by V2 and V3. This is the BMP-4 gradient will result in notching of the vermilion. As embryologic basis for the treatment of migraine headaches the situation worsens the extent of the lip cleft worsens with Botox® injected into peripheral trigger points. in a cranial direction. The clinical spectrum of the so- called minimal cleft lip deformity faithfully reproduces Design of surgical incisions for cleft repair follows this this biologic sequence.[16] embryology. The boundary between these two fields is sharply demarcated within the naris. The skin of the In summation, variations in clefts involving the primary anterior columella and philtrum is thus a p5 derivative. palate and the lip can be understood as interactions This skin is supplied by terminal branches of the between deep plane fields of the premaxilla, the maxilla internal carotid artery, the anterior ethmoid arteries and and superficial plane field of the lateral lip element. The innervated by V1.[3] The skin making up the floor of the mesenchyme of the lip has a different embryologic origin. nose has a different origin. It extends from the base of It is genetically identified with the 2nd rhombomere. the columella laterally and makes contact with the alar Neural crest cells from r2 provide the dermis and the base. The medial (terminal) branch of the sphenopalatine subcutaneous tissue of the alar base, while paraxial artery innervates this skin. The innervation is from V2 and

Indian J Plast Surg January-June 2007 Vol 40 Issue 1 78 Developmental ﬁeld reassignment in unilateral cleft lip is shared with the ipsilateral incisors. Continuity between a set of Lego® pieces, all growing over time. If one piece of the p5 skin of the lateral columella and the r2’ skin of the the set is missing, with subsequent growth the remaining nasal floor makes possible the elevation of a skin-cartilage pieces will collapse into the deficiency site. Only when flap containing the medial crura with long skin flaps. Many all the Lego pieces are in place can harmonious facial years ago Vissarianov described this flap as a means of development occur. secondary cleft reconstruction.[26-27] The premaxillary developmental field consists of lining Based on signals from the underlying premaxilla, the r2 (present in its totality) and mesenchyme (missing). alar base produces a tongue of tissue that makes contact Lining can be recreated by: (1) subperiosteal dissection with the r1 lateral prolabium. This skin bridge sets up the of the primary palate (r2’ premaxilla and r2 maxilla); (2) floor of the nose. It also provides a mechanical platform by subperiochondrial dissection of the p6 septum (sufficient which mesenchyme from the lateral lip element can make to reduce the septum into the midline; (3) subperiosteal contact with the p5 mesenchyme of the prolabium. Lip elevation of r2’ vomer to close the nasal floor (sufficient to closure thus occurs. This process involves mesenchymal reduce the septum into the midline); and (4) subperiosteal “flow” from the lateral lip element toward the prolabium. rescue of the r2’ nasal floor skin from the lateral prolabium. For this to occur, the epithelium covering the lateral lip When these flaps are elevated and sutured, the primary element, the r2-r1 skin bridge and prolabium must undergo palate becomes a box lined with neural crest stem cells, a genetically-induced breakdown. BMP-4 produced by the all of which carry membrane-bound BMP receptors.[29-31] premaxillary field causes de-repression of Sonic hedgehog (SHH), a gene localized to these overlying epithelia. The Mesenchymal replacement can be undertaken using protein product of SHH causes epithelial breakdown. Thus, two basic mechanisms. Autogenous bone graft from absence or deficiency of an appropriate BMP-4 signal will rib or hip provides mesenchymal cells originating from lead to restricted expression of SHH, abnormal persistence paraxial mesoderm. Incorporation of the graft into the of epithelium and failure of mesenchymal fusion.[28-29] primary palate.com). occurs by osteoconduction. Implantation of recombinant human bone morphogenetic protein-2 The volume of the PMx determines whether or not lip (rhBMP-2) in combination with an activated collagen closure can occur. First, a small premaxillar field manufactures sponge (ACS) results in morphogen-based recruitment of small amounts of BMP-4. The amount of BMP-4 produced is stem cells from the environment into the sponge. Stem cell critical to produce the epithelial breakdown necessary to concentration and differentiation into osteoblasts results permit mesenchymal merger. Second, when the premaxilla.medknow in the formation of bone native to the site. This process is too small, the physical distance between it and maxilla is known as osteoinduction.[32] Extensive pre-clinical work will exceed a critical dimension. Epithelial bridge formation by Boyne demonstrated the ability of rhBMP-2 to form between the alar base and the prolabium cannot(www occur. If this membranous bone, including reconstitution of surgically- critical distance exists at the level of the incisive foramen, created cleft palate defects in primates.[33-34] Studies a cleft of the secondary palate will form. This is because demonstrating efficacy in maxillary lift combined with the horizontal repositioningThis PDFa ofsite the is palatalhosted available shelf frbyom theforMedknow freeabsence download of Publicationsdonor site morbidity from r esulted in FDA approval maxilla must make contact with the vomer just posterior for this indication.[35] to the incisive foramen. The process is just like a zipper. If initial contact is not made, fusion of the palatal shelf to the The technique of facial bone reconstruction using vomer cannot take place. Even if initial contact is made, a rhBMP-2/ACS implantation is known as in situ osteogenesis secondary palatal cleft can still result due to displacement (ISO) and has been previously reported by this author of the vomer away from the midline. The vomer can become and co-workers.[36] Resynthesis of a 12 cm mandibular warped by the inequality of growth forces on either side of defect in a 9 year old demonstrated the ability of ISO to the cleft. Thus, the zipper may get started anteriorly but, as function effectively outside the range of blood supply the process proceeds posteriorly, when it encounters the associated with a critical-size defect.[37] BMP-2 mediated deviated vomer, a palatal cleft will open up.[3] osteoinduction is accompanied by extensive recruitment of blood vessels from local environment.[38] For this Reconstruction of the premaxilla reason selected bone defects can be resynthesized using Cleft surgery that does not reconstruct the missing PMx ISO alone, without recourse to microsurgical tissue does not solve the biologic problem. The pediatric face is transplantation.

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Figure 2: Developmental anatomy of premaxilla. Spatio-temporal distribution of neural crest (arrow) demonstrates frontal process as most —recent“ derivative. Clefting affects the premaxillary ›elds in reverse order; piriform fossa de›ciency is therefore the —forme fruste“ manifestation of a cleft .medknow Distraction techniques have been successfully applied to ISO-regenerated bone. In a number #7 lateral cleft with a foreshortened mandibular body and(www absent ramus, distraction of the recipient periosteal chamber in a posterior and superior direction permitted synthesis of 3.5This cm of mandibularPDFa site is ramushosted available with eventualby forMedknow free download Publications from articulation with the skull base via a pseudoarthrosis.[39] Distraction-assisted in situ osteogenesis (DISO) will be an alternative treatment to rib grafts in the reconstruction of the Pruzansky III mandibular defects in craniofacial Figure 3: Medial and lateral sections of the nasal cavity demonstrate the microsomia. The bone produced will be membranous. neuromeric origins of the bone ›elds. The biosynthetic pathway of the r2‘ MNC The dissection is less problematic. There is avoidance of lays down the premaxilla and vomer along the axis of the nasopalatine nerve and the medial branch of the sphenopalatine artery (the absolute terminus of unpredictable growth of the rib graft outside the natural the external carotid). Fields affects by the Tessier clefts are indicated by yellow periosteal environment. Finally, the chest wall donor site stars, eg. the #3 cleft is a selective knock-out of the inferior turbinate. Because the p5 lacrimal bone is built upon IT the #3 cleft destroys the lacrimal system. is obviated. Histology of ISO-produced membranous The cleft premaxilla is zone #2 bone is indistinguishable from that of the recipient site in both mandible and maxilla. excellent and the dimensions modest. For this reason, this author and co-workers reported 50 alveolar clefts Alveolar clefts are lined with mucoperiosteum successfully treated with rhBMP-2.[40] Precise surgical containing neural crest stem cells. Blood supply is technique of implant placement and of soft tissue

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Figure 4: Cleft site showing retraction of p5 lateral crus vestibular pulled downward into the premaxillary de›ciency site (just in front of the inferior turbinate). LLC is programmed by p5 nasal skin; ULC in programmed by p6 vestibular epithelium. If the LLC is not released and additional tissue provided, the nasal vestibule will be reduced in surface area 30-40% compared to the non-cleft side. Standard cleft repairs lead to respiratory dysfunction

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Figure 5a: Surgical marking and incisions for DFR. Point #3 is 6-10 mm from the normal philtral column. As medial incision for NPP-LCC fiap reaches the columellar base, it is interrupted by medially-based rectangular(www Reinisch fiap. Lateral nasal wall incision separates p5 vestibular mucosa (sky-blue) from r2 nasal skin (yellow) This PDFa site is hosted available by forMedknow free download Publications from closure was emphasized in the current series of 200 d cleft sites.[41] Long-term outcome of 43 cleft sites was assessed at one year post grafting with 3-D CT. The Figure 5b-d: DFR surgical design. Operative sequence demonstrates elevation and transposition of NPP-LCC (non-philtral philtrum/lateral columella series was comprised of 23 unilateral clefts (6 primary chondrocutaneous) fiap into de›ciency site created by release and cephalic and 17 secondary) and 12 bilateral clefts (3 primary rotation of p5 lateral crus. This reconstructs the soft tissue corresponding to and 9 secondary). Complete transverse fill (unification mssing lateral premaxillary ›elds of the dental arch) was achieved in all cases. Vertical fill was improved to 75-100% when an inert bulking Philosophy of Developmental Field Reassignment agent (tricalcium phosphate) was included. For these (DFR): the 4 D’s reasons, primary cleft repair using developmental field Faulty embryogenesis of the premaxillary field affects the reassignment technique can ideally be combined with position and shape of surrounding fields in four distinct ISO to achieve primary unification of the dental arch ways: Division of force vectors causes unequal growth, without donor site morbidity. Deficiency-related collapse of partner fields into the void,

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dental arch into a normal relationship. Fifth, reassign all developmental fields into correct relationships with each other. When properly executed, these principles result in the restoration of the functional matrix. When all the bone-forming soft tissue fields are spatially correct, all force vectors exerted upon bone will be correctly aligned. Subsequent growth of the face is directed back toward the normal.[44-46]

A new algorithm: Is lateral nasal wall deﬁciency relative or absolute? In keeping with Victor Veau, cleft repair is a constant exploration of nature’s experiments. Developmental field reassignment surgery is deliberately designed to address a tissue deficit of the lateral nasal wall and alveolus, the product of a congenitally small premaxillary field. That the lateral crus be entrapped cannot be in doubt. Its release into a normal position occasions a triangular tissue gap that must be filled. Proper airway reconstruction is the name of the game. At the same time, the alveolar cleft (a six-sided box) must be reconstructed. This requires flap coverage for its nasal surface (the “top” of the box). Can these two goals be accomplished with the same tissue? A skin graf.com).t alone (particularly anterior auricular skin and cartilage) will effectively support lateral alar crus advancement. It cannot provide vascular coverage for the Figure 6: Orbicularis oris is structurally and funcionally bilaminar. Anatomic alveolar cleft “roof.” At first blush, the LLC-NPP flap would dissection by Park demonstrates super›cial orbicularis oris (SOO) to attach to the philtral column as a dilator. Deep orbicularis oris (DOO is separated from SOO by seem big enough to accomplish both goals. After 7 years a plane of fat and axial vasculature. DOO is a sphincter. It runs in the plane deep of work with this technique this author has concluded that to the p5 mesenchyme of the philtrum and is thus continuous with itself across the midline. All pharyngeal arch muscles follow the same pattern: deep plane.medknow paring from the prolabium is not sufficient. Premaxillary muscles appear earlier than super›cial, caudal before cranial and lateral before field soft tissues are not only relatively deficient from the medial. The very last facial muscle to appear is the oblique head of SOO lateral nasal wall: an absolute tissue deficit exists. Use Displacement of partner fields away from the(www midline. of the premaxillary tissue mismatched to the prolabium Distortion over time of structures such as the septum. is not always sufficient to solve the problem. The new DFR repair addresses these “4 D’s” in reverse order. All algorithm of DFR surgery now calls for optional composite distortions shouldThis be corr PDFected.a site The is hosteddisplaced available sof tby tissues forMedknow freegrafting download of Publications the lateral wall,from followed by elevation and if the face should be centralized into the midline. The transposition of the LCC-NPP flap across the nasal surface deficiency site should be restored, using neighboring of the alveolar cleft. The decision to graft is based upon soft tissues and bone graft or ISO; and division should the relative size of the defect versus that of the flap. be closed via unification of soft tissues.[42] SURGICAL TECHNIQUE OF DFR The execution of DFR surgery is based upon the concepts of Sotereanos.[43] Its guiding principles are five.[6-7] (1) This operation consists of markings, a five-step dissection Correct all pathologic states in the first operation (as sequence and a five-step closure. above). (2) Respect embryonic developmental planes during dissection, ie. subperiosteal release of the soft Preparation and marking tissue envelope for a tension-free closure. (3) Conserve The DFR operation is comprehensive, providing blood supply to the periosteum, safeguarding it for future simultaneous correction of the lip, nose and primary membranous bone synthesis. Fourth, align and unite the palate. A more extensive dissection is required. The

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.medknow Figure 7: Four-step nasal incision sequence achieves advancement of both medial and lateral crura. I have recently modi›ed the infracartilagenous incision, making it discontinuous along the lateral crus to mimimize contracture. Nasal tip is reconstructed by simultaneous elevation of both the medial and lateral crura. The Delaire sutures are shown. #1 is placed in the levator insertion into the SOO one › nger-breadth lateral to the alar base. This centralizes the lip-cheek ﬁ ap. The non-cleft side is done ›rst to the anterior nasal spine (a drill hole is helpful). #2 is placed in the nasalis and is optional (decision made at the end of the case). It adjusts alar base position and nostril sill curvature. #3 suspends the oblique head(www of SOO from the columellar base. It establishes the —aesthetic drape“ of the lip operation takes longer to perform (about 3-4 hours for a pterygopalatine fossa using 0.25% Marcaine® (bupivicaine unilateral cleft).This For this rPDFeason,a site patients is hosted available come to bysurger forMedknowy freehydrochloride). download Publications Approximately from 3-5 cc per side is sufficient at 4-6 months of age. Prior to operation the dental arch (maximum dose for 0.25% bupivicaine being 1 cc/kg). At is prepared with splinting (a form of infant presurgical the end of the case, the central block is reinforced by orthopedics); this is begun as early as two weeks after blocking the infraorbital nerves with bupivicaine, 1-2 cc birth. The emphasis of presurgical splinting is: (1) per side. The child returns to recovery pain-free. The initial promoting anterior growth of the retro-displaced cleft block ensures that substance P (a critical mediator in the maxilla; and (2) maintenance of the space in the alveolar pain cascade released in response to surgical trauma) will cleft. If satisfactory maxillary shelf repositioning does not not be produced. In the absence of substance P the entire result by age 3 months, a lip adhesion procedure is carried postop pain response is altered. out. When satisfactory dental arch correction is achieved, DFR repair is performed. This usually occurs 3-4 months The surgical fields of the unilateral cleft are defined as after the lip adhesion. follows. FIGURE (In neuromeric terms: A =p6 (red) + p5 (turquoise), B = r2’ (pale gold), C is r2 (yellow) and D The patient receives antibiotics and corticosteroids is r2 + r4 (yellow + orange). The prolabium is divided for swelling. A central V2 block is performed at the into two zones. Zone A is the true philtrum; it measures

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UCL Double Layer Periosteoplasty: Step 1

Figure 8: NPP-LCC fiap has dual blood supply. Behind the surgical tape are: (1) lateral branches from the ipsilateral anterior ethmoid artery of the columella; Figure 10a: Anterior closure of unilateral alveolar cleft using sliding sulcus fiaps. and (2) vessels running along the dorsal surface of the lower lateral cartilage On the cleft side two incisions are required. (1) A medial-to-lateral L-shaped that anastomose the nasopalatine artery with the lateral nasal artery. In front releasing incision (red line) is placed in the gingival sulcus. It runs from the cleft of the tape, the nasopalatine (medial sphenopalatine) artery emerges from the lateral to the buttress. There, it ascends vertically to the zygomatico-maxillary septopremaxillary junction. This tissue, otherwise called the B fi ap, reconstructs junction. The subperiosteal fiap is elevated widely up to the infraorbital foramen. the nasal fioor (roof of the alveolar cleft) and the lateral nasal wall. This Because the periosteum is an intact sheet, the fi ap is stiff, being tethered to the corresponds to the missing premaxillary ›eld orbital rim. (2) A counter-incision (green line) is begun at the level of the nasal fioor and brought transversely from the piriform to the buttress. This releases the lower (alveolar) mucoperiosteal fiap. It freely translates two tooth units medially. This closes the anterior wall of the alveolar cleft

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Figure 9: Dissection of alveolar cleft. Elevation and division of mucoperiosteal © 2007 Carstens © 2007 Walters fiaps provides closure of nasal fioor and oral aspect of primary palate. R2‘ premaxillary fiaps (violet)This are sutured PDFa tosite r2 maxillary is hosted available fiaps (yellow), by providing forMedknow free download Publications from 4 walls of the box. The cleft-side sliding sulcus fiap closes off the front of the Figure 10b: Bilateral sliding sulcus fiaps are illustrated. Although I started out box. Backwall closure occurs when vomerine mucoperiosteum is elevated and with this technique, I no longer do a gingival release on the non-cleft side. sutured to the palatal shelf MxP. Septum is reduced and secured to midline. Cleft A one-sided release is suf›cient. On the non-cleft side, wide subperiosteal site —box“ ›lled with osteoinductive cytokine rhBMP-2 or autogenous bone graft. mobilization is combined with a full-thickness vertical releasing incision up This reconstructs the missing premaxillary Lego® piece the buttress. This creates a huge, mobile bipedicle fiap. The entire soft tissue complex becomes centralized without tension. Nota bene: Surgeons working the width of the columella. It contains paired anterior under extreme condition (very wide clefts in remote areas with no access to ethmoid arteries (the terminal branches of the internal orthodontic management) bilateral sliding sulcus fiaps are very effective. They will close any size defect carotid system) and paired terminal branches of V1. Thus zone A contains two neurovascular developmental D. It contains a sphincter layer, the deep orbicularis oris fields. Zone B of the prolabium is all tissue lateral to the (DOO) and the dilator layer, the superficial orbicularis oris philtrum: the non-philtral probium (NPP). The nasopalatine (SOO) muscles. DOO develops in conjunction with the oral (medial sphenopalatine) artery, the terminal branch from mucosa while SOO develops with the skin. A layer of fat the internal maxillary system, supplies the NPP. Bilateral conveniently separates these two layers.[47-48] clefts have two NPP zones. The cleft-side alar base is zone C. The lateral lip element below the alar groove is zone The true destination of NPP soft tissue is in the nasal

Indian J Plast Surg January-June 2007 Vol 40 Issue 1 84 Developmental ﬁeld reassignment in unilateral cleft lip

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Figure 11 (Case1): Bilateral cleft lip with right unilateral cleft of primary palate and bilateral clefts of secondary palate. Soft tissue relationships one year after DFR demonstrating stability of nasal tip projection. Comparison of CT scans at 3 months and 9 months show bony union of primary palate. Periapical view of alveolar cleft site shows reconstitution of the periodontal ligament, a neural crest structure. This is not seen with conventional iliac crest graft. Serial 3-D skulls taken 9 months apart support the Delaire dissection concept: wide subperiosteal undermining repositions the osteogenic soft tissue ›elds into a correct, centralized relationship. Subsequent osteosynthesis of membranous bone now occurs in a correct centric distribution. Developmental ›eld reassignment promotes normal membranous bone formation after the surgical procedure floor and the lateral nasal wall. NPP represents the soft the lateral crus corresponds to the frontal process field, tissue envelope corresponding to the lateral zone of PMxF. Lateral wall soft tissue corresponds to the frontal the premaxilla, PMxB + PMxF. Nasal floor soft tissue process field, PMxF. Nasal floor soft tissue corresponds corresponds to the lateral incisor field, PMxB. Soft tissue to the lateral incisor field, PMxB. The rationale of DFR of the lateral nasal wall between the inferior turbinate and surgery is to reassign the misplaced fields of the premaxilla

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Figure 12 (Case 2): Post op CT scans of piriform fossa in this challenging cleft.medknow demonstrate leveling out of the nasal ﬁoor to support the alar base. Consolidation of the alveolar cleft during primary DFR surgery maintains dimensions of the dental arch without collapse even after palatoplasty. Note symmetry of columellar-alara relaltionships seen in lateral views into correct position. When the NPP flap is(www combined tissue. The cymba graft, in combination with the NPP with the reflected mucoperiosteum from the margins of flap, provides more than enough tissue to reconstruct the bony cleft a surgical “pocket” is created. This site is the missing premaxilla. subsequently filledThis with PDFbonea site graf ist hostedor available a bone-pr oducingby forMedknow free download Publications from cytokine (rhBMP-2) such that missing premaxillary fields Markings are carried out using a modification of the are re-synthesized. Millard system. (Paired anterior ethmoid neurovascular bundles define the true philtrum; this equals the width of The NPP flap functions just like a “boxtop” to cover the the columella.). First to be marked is point 2, the junction alveolar cleft. It is almost always adequate. I am convinced of the normal philtral column with the white roll. The that the “secret identity” of NPP is the original PMxB. width of the columella at its base is then measured. This Because of the cleft this tissue is “shipwrecked” on the distance (usually 6-10 mm) is subsequently marked along premaxilla. Unfortunately, NPP is frequently insufficient the white roll medial to the philtral column as point 3. to replace PMxF. Every time the lateral crus is released Distance 2-3 is the width of the philtrum; this contains and advanced a soft tissue defect appears. The defect the two anterior ethmoid arteries (approximately 4-6 mm represents missing PMxF. In my clinical experience, apart). The midpoint of the philtrum (point 1 in the Millard this is almost always the size of the auricular cymba. A system or nadir of cupid’s bow) is irrelevant. Point 13, composite graft of anterior auricular skin and cartilage defined by the bulge of the footplate of the medial crus on from the cymba is the most reliable replacement for this the non-cleft side, marks the “shoulder” of the columella.

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Figure 13 (Case 3): MD Secondary deformities resulting from displacement of tissue from the midline corrected with subperiosteal centralization. Note changes in the non-cleft alar base after DFR. Residual sag in left alar contour will need cartilage batten graft in the future. The straight-line scar in DFR repair simulates philtral column.

The corresponding point 12 on the cleft side will be found the inferior turbinate. Point 9 is the tip of flap D while point displaced caudally and internally with respect to point 13. 11 is the tip of the future nostril sill C’, so-called because it Total philtral height 2-13 should equal 3-12. Points 4 and is in continuity with the alar base C. The dimensions of C’ 10 mark the midpoint of the alar bases on non-cleft and can be roughly mapped out by measuring the nostril sill on cleft sides, respectively. the non-cleft side. This is the distance from the midpoint of the non-cleft alar base (point 4) to the ipsilateral footplate On the lateral side, points 9 and 11 mark the terminus of point 13. Point 8 marks the natural transition of the white skin within the nasal cavity. This is located just anterior to roll. Distance 8-9 should match 3-12.

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layer of fat. The caudal margin of the two muscles defines the white roll and contains the labial artery.[47]

Step 2. Medial dissection: the NPP-lateral columellar advancement flap The medial margin of the cleft (zone B in our diagram) consists of prolabial tissue lateral to the true philtrum. This is in continuity with lateral columellar skin and the medial crus of the alar cartilage. Conveniently, the blood supply of these two units is a watershed permitting disection of a very long flap. The B flap has two parts: (1) a skin/mucosa flap of the non-philtral philtrum (NPP) and (2) a chondrocutaneous flap containing the lateral columellar skin and medial crus (LCC). B = NPP + LCC

Figure 13a: Vissiarnarov ﬁap harvested from scar in case 3 simulates non-philtral philtrum and ›lls lateral piriform defect created by advancement of the lateral crus. B has a rich blood supply. The NPP component is Note large pedicle from the external carotid-based nasopalatine artery. These irrigated by the nasopalatine artery. The LCC component ﬁaps can be harvested in virtually all secondary cleft reconstructions gets supply from 2-3 lateral branches of the ipsilateral anterior ethmoid. In addition, vessels in continuity with Dissection sequence the lateral nasal system from the facial artery run along Step 1. Lateral dissection: rescuing the nostril sill the surface of the alar cartilage itself. Once elevated, B The lateral lip is tensed with a single hook and the skin- is surprisingly long, reaching all the way across the cleft mucosa margin is incised proceeding upward from 8 to 9, to the lateral nasal rim. [Figure B] is inset into the gap located just below and anterior to the inferior turbinate. created by advancement.com). of the p5 lateral vestibular lining From here the incision swings around laterally to 10, (and the lateral crus). This achieves two important surgical the midpoint of the alar base, (but not beyond it at this goals: (1) cephalic advancement of the medial crus with point). In this manner, the lateral lip flap D is separated increased tip projection; and (2) soft tissue augmentation from the alar base C. This step separates orbicularis of the lateral nasal wall. Inset of B permits release of from the nasalis. From point 9, a second, more internal, the tethered lateral vestibular lining. Repositioning the incision defines the triangular flap C’. The base width of.medknow lateral crus now occurs in what would otherwise be a C’ can be deduced by measuring the dimensions on the Y-V pattern without the Y-V closure. Natural alar cartilage non-cleft side. anatomy is accomplished without pinching together an (www already-deficient lining. From the lip a lateral vermilion flap (L) is pared off and dissected down to the alveolar cleft margin. Proper paring The future cleft-sided philtral column is determined by of L includes a smallThis strip PDF ofa lipsite skin is 2-3hosted available mm wide becauseby forMedknow freeunderstanding download Publications the embr fromyology of the prolabium. The it is “rolled-in,” with an abnormal relationship to the true philtrum lies between points 2 and 3 and consists underlying muscle. If the surgeon does not do this, the of two fields (each supplied by a separate branch of the skin will pucker inward at the final closure. The L flap is anterior ethmoid artery). The philtral dermis comes from the most optional tissue of the entire DFR operation. It is p5 prosencephalic neural crest and is innervated by V1. best brought medially and interposed between the nasal The width of the philtrum is roughly equal to that of the vestibular lining and the B flap. It can also be used as a columella, generally measuring 6-10 mm. Because the free graft to the lateral nasal wall. No tissue is sacrificed philtrum develops on top of the r2’ premaxilla, it receives in DFR. additional blood supply from the terminal branches of the sphenopalatine artery (SPA) emerging at the level of With its vermilion stripped off, the lateral lip margin is the septopremaxillary junction. Thus, the philtrum has a now entered and the orbicularis is split into its deep dual blood supply. All remaining prolabial skin and mucosa (constrictor) and superficial (dilator) components. The (the NPP) is an r2’ derivative, supplied by the nasopalatine deep orbicularis oris (DOO) is shaped like the letter J and artery and innervated by V2. The prolabium of a bilateral separated from the superficial orbicularis oris (SOO) by a cleft thus consists of four developmental units based on

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Figure 14 (Case 4): Developmental ›eld reassignment of soft tissue ›elds in bilateral lip/palate cleft repair demonstrates normalization of Cupid‘s bow. Contrary to current dogma the philtrum and columella are not short in the cleft condition; they are mismatched. By recognizing and reconstructing the missing premaxillary ›eld, DFR corrects de›ciency-induced › eld mismatch embryology, blood supply and innervation: two central septum. Just beneath the lateral columellar pillars lie the philtral A fields and two lateral B fields. medial crura of the lower lateral cartilages (LLC). Because these pillars serve as the biologic “template” for the cartilage The embryology of the columella is as follows. The anterior to form, dissection of skin away from the crura is extremely centrally-located skin comes from p5 and contains the paired difficult. The LLCs are thus p5 neural crest derivatives. The anterior ethmoid arteries. On either side of that central upper lateral cartilages lie above the p6 vestibular lining and swatch, the columellar skin extends backwards toward the are therefore of p6 neural crest origin.

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Correction of the cleft lip nose requires releasing the alar The NPP-LCC flap now resembles a long boot, shaped like cartilage from two points of entrapment.[49] The deficiency Italy. The “toe” of the boot extends along the prolabial state of the premaxilla causes a mechanical deformation margin. Beneath the “heel” of the boot lies the footplate of perfectly normal p5 skin on both sides of the nasal of the alar cartilage. This landmark corresponds to point introitus. In the lateral nasal wall, the r2’ skin overlying the 12. Grasping the heel of B provides instant access to the premaxillary frontal process (PMxF) is reduced or absent. medial aspect of the medial crus. This is a safe plane Consequently, p5 skin containing the lateral crus of the permitting dissection of the alar cartilage right into the alar cartilage is dragged down into this “sinkhole” located nasal tip, with the following caveat. The B flap gets blood just in front of the inferior turbinate. The lateral crus is supply from the nasopalatine artery via 2-3 branches therefore flattened. On the medial side, the deficiency emerging at the junction of the premaxilla and vomer. of the r2’ premaxillary lateral incisor field (PmxB) creates Gentle spreading along the medial border of the cartilage a “sinkhole” into which the p5 lateral columellar skin will reveal these branches and preserve them. Additional is displaced. This displaces the medial crus of the alar blood supply descends along the skin. Formerly I would cartilage downward and inward compared with the non- elevate these NPP-LCC flaps completely, never having an cleft side. issue with ischemia. Now however, I think it prudent to preserve the nasopalatine branches when possible. This The LCC-NPP flap is elevated as follows. Under tension, the type of blunt dissection is more than sufficient to advance NPP skin is elevated in continuity with the lateral columellar the nasal tip. wall. The lateral (internal) incision is made first, separating the skin from the mucosa all the way to the junction The septum is now dissected out and freed from the between the premaxilla and vomer. Although this incision maxillary crest until it sits passively in the midline. As will eventually be carried up in front of the septum, it is growth proceeds the centralized septum will no longer advisable to stop here. Tension can thus be maintained on constitute an abnormal force vector tethering the nasal the NPP while it is separated from the philtrum proper. tip. .com).

Next, a second incision, parallel to the first, ascends from Step 3. Nasal dissection: “open-closed” rhinoplasty point 3 to the base of the columella. It then extends At this juncture, a standard infracartilagenous incision is into the nose along the lateral sidewall of the columella. made. This incision is brought all the way to the piriform Previously I would continue this incision along the rim, following the natural fold between the nasal skin side of the columella (just anterior to the edge of the.medknow and the vestibular skin. The caudal extent of this incision medial crus) and thence bring it directly into the nose terminates at the internal border of the triangular nostril as an infracartilagenous incision. This design made sill flap B’. The exposure gained via the lateral columella- me concerned about possible scar contractur(wwwe. At the infracartilaginous incision allows a complete dissection of suggestion of Dr. John Reinisch, I began to break up the the dorsal nasal skin envelope as described by McComb.[50] incision by elevating a medially-based rectangular skin flap The success of the McComb dissection is really an occupying the caudalThis half PDFa of sitethe columella.is hosted available Just beneathby forMedknow freeembr yonicdownload Publicationsfield separation. from The deep layer comprises the Reinisch flap lies the footplate of the lateral nasal the p6 vestibular epithelium and p6 neural crest upper cartilage. At its cephalic margin, the incision is resumed lateral cartilages, the blood supply to which comes from along lateral columella. At the level of the intermediate below via the ICA. The superficial layer is p5 nasal skin crus the incision transitions beneath the soft triangle and the lower lateral nasal cartilages, the blood supply to into a standard infracartilagenous incision and is then which is also of ICA derivation. Interposed between these continued all the way to the piriform margin. two layers, like a sandwich, is the SMAS layer of facial muscles derived from the 2nd pharyngeal arch (r4-r5). The The anterior incision of the NPP-LCC flap is not sufficient to myoblasts come from somitomere 4. The nasal muscles are advance it. A second parallel incision is required. FIGURE It compartmentalized by r4 neural crest fascia (the SMAS). starts in the membranous septum about halfway up. It is The blood supply to this intermediate 2nd arch layer is then carried downward to the vomer and then re-directed from the facial artery (ECA). This mesenchyme provides forward to meet the lateral margin of the B flap. Note an additional source of blood supply to the alar cartilages. that the membranous septal incision provides immediate Careful dissection of the lateral columellar walls from exposure of the cartilaginous septum. the columella discloses vessels running along the medial

Indian J Plast Surg January-June 2007 Vol 40 Issue 1 90 Developmental ﬁeld reassignment in unilateral cleft lip surface of the medial crura. These anastomose the lateral these solutions made use of an anteriorly-based inferior nasal vessels with the nasopalatine vessels. turbinate flap as described by Noordhoff.[53-54] Although this tissue worked adequately, I had several reservations about Along the lateral nasal rim the dissection is carried it: (1) the dissection is subtle and difficult to teach; (2) right down to the piriform margin. The proper plane the tissue type is distinct and not native to the nasal rim; for separation is achieved by hugging the surface of the and (3) healing of the donor site can be accompanied by cartilage. The overlying r4 SMAS layer and the p5 skin crusting and bleeding; and (4) it did not make embryologic layer are left in anatomic continuity. Very little bleeding is sense. Proper dissection and inset of flap NPP combined occasioned by this approach. As one reaches the piriform with use of a composite for FTSG made the turbinate flap rim the periosteum is incised and stripped vertically. unnecessary. The key to getting the most out of NPP is to Subperiosteal elevation of the soft tissues lateral to the include the prolabial vermilion with the skin. piriform rim preserves the facial artery arcade. The nasal skin envelope is then liberated cephalically all the way to Step 4. Intraoral dissection: sliding sulcus S flap the nasal bones. The rationale and design of the sliding sulcus mucoperiosteal flap stem directly from pioneering work at the University Despite these maneuvers, the vestibular lining is still of Pittsburgh by Sotereanos.[43] This technique involves tight! The media crus has been completely released and a gingival release on the cleft side carried out from advanced into the nasal tip but the lateral crus being the alveolar cleft to the buttress.[42] This permits wide remains splayed out and tethered. Releasing the lateral subperiosteal dissection over the entire face of the maxilla crus from the vestibular lining has been advocated in the below the infraorbital foramen as described by Delaire.[55 past. This is technically difficult because it violates the 56] A 45 degree backcut up the buttress is performed. The embryology (recall that the alar cartilage arises as a neural attached gingival is released. The flap is covered on its crest response to a pattern embedded in the vestibular undersurface by a sheet of periosteum, rendering it rather epithilium). The size and shape of the cleft-side alar stiff. The Soter.com).eanos maneuver is a mobilization of the S cartilage has been demonstrated to be normal compared flap using a counter-incision in the periosteal sheet itself with the non-cleft side.[51] Consequently, the p5 vestibular parallel to the gum line. The counter-incision, located half- lining “program” must be normal as well. Studies at UNC way from the gingival margin to the infraorbital foramen, demonstrate that the overall surface area of the repaired is made by just scoring through periosteum. It extends cleft nostril is reduced by 30%.[52] The site and dimensions from the piriform margin straight lateral to the buttress. of the deficit correspond to the soft tissues of PMxF. .medknowAt this point it joins up with the previous backcut. These two incisions make a right angle: the periosteal counter- The soft tissues of the alveolar cleft PMxB can be found incision is transverse across the maxilla and the buttress on the prolabium. The LCC-NPP flap effectively(www provides incision is vertical. The combination of these two incisions a vascularized “roof ” for the bony cleft. Lateral nasal releases the lower mucoperiosteal flap S from the upper wall release “uncovers” a defect that frequently exceed mucoperiosteum attached to the orbit. the reach of LCCThis-NPP. The PDFa r eleasesite is beginshosted available at the byinferior forMedknow free download Publications from turbinate and continues along the junction of vestibular When S is released, it advances mesially about two tooth and nasal skin. At the apex of the lateral crus it becomes units. The margin of S that was freed from the lateral V-shaped. Due to the prior McComb dissection the lateral border of the cleft now extends across the alveolar cleft crus is advanced cephalically into symmetry with the without tension and is sutured to the mucoperiosteum of normal side. The internal nasal valves will appear equal. the premaxilla. In this way, like a sliding door, the S flap Left behind is a raw spot roughly the size of the ear seals up the anterior aspect of the alveolar cleft. cymba. A composite graft using anterior skin and cartilage from the cymba provides a sturdy reconstruction for the On the noncleft side, a similar subperiosteal dissection airway. The LCC-NPP flap, inset without tension across the is done without recourse to a gingival release. A large alveolus, comes to rest at the foot of the cymba graft. bipedicle flap is created, permitting centralization of the previously lateralized soft tissue envelope. Note that when Exit the turbinate ﬂap cleft lip occurs without an alveolar cleft, bilateral S flaps, As the DFR evolved, I employed various solutions to elevated without gingival release, permit proper tension- patch the defect created by the vestibular release. One of free centralization of midface soft tissues.

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The S flap then comes in two varieties, each of which has a mucoperiosteal flap elevated directly off the lateral incisor. specific benefit for the patient. The simple S flap is elevated The space will fill in nicely. If one desires, the flap donor in the subperiosteal plane across the entire face of the site can be grafted with Alloderm®. maxilla between two points of vertical release: the alveolar cleft and lateral wall of the buttress. It remains attached Limited resources and difficult logistics in economically to the teeth. A gingival releasing incision is not required. developing countries force surgeons treating cleft patients By virtue of its ability to centralize the soft tissues of the to adopt different and creative strategies, particularly entire midface, the simple S flap fulfills two important when the alveolar cleft is wide. These children often have functions: (1) the creation of an esthetic soft tissue “drape”; no access to pre-surgical orthopedics. They may never and (2) the biologically active osteogeneic potential of the receive orthodontics. They do possess a ready source of mucoperiosteal stem cells is transferred forward, thus bone graft from the rib. In such cases, achievement of a enabling bone deposition to take place in centrically for consolidated arch and elimination of oronasal fistula are better facial skeletal projection. The drawback of the simple reasonable goals for primary cleft surgery. The two key S flap is that it cannot produce a mucoperiosteal flap to factors for success in these patients are: (1) ability to make cover over the anterior aspect of the alveolar cleft. In such a simple acrylic splint and secure it--2 mm screws work cases, a rectangular mucoperiosteal flap can be harvested perfectly well; and (2) meticulous dissection of the alveolar immediately lateral to the alveolar cleft and brought over cleft with attention to its vascular anatomy. for closure. This design works for narrow clefts. If a dental splint can be fabricated, lip adhesion can narrow The compound S flap (as described by Sotereanos) the cleft down to dimensions permitting simultaneous implies a full gingival releasing incision all the way from DFR repair and alveolar grafting with a simple S flap. the alveolar cleft back to the tuberosity. This involves When this is not possible, a compound S flap will close additional operating time. Gingival release from an the gap. In some very wide primary unilateral clefts I have alveolus containing erupted primary or secondary used bilateral.com). compound S flaps to successfully close gaps dentition is technically simple. The bone is solid and of 14-16 mm. Of course, this maneuver disconnects the the surgical plane easy to follow. Infants are a different frenulum from the midline. Postoperative remodeling of matter. Prior to the 4th month of life maxillary and alveolar the alveolus reestablishes symmetry. In such cases, arch bone is too soft to work with. DFR should be performed stability justifies the extensive intraoral dissection. between ages 4-6 months. Dissection is facilitated using loupe magnification, a #15C or Beaver blade, an amalgam.medknow Secondary cleft reconstruction follows the same rules packer and Molt (#9) or Louisville periosteal elevators. but with the proviso that gingival release incisions are A compound S flap can be mobilized two full tooth fast and easy to accomplish. The floor of the alveolar widths. The main advantage of the compound(www S flap is reconstruction has already been provided by a pre-existent the dramatic increase in mucoperiosteal flap length it palatal repair. When orthodontic capability exists, such provides. This is generally the width of two dental units. clefts should first be expanded to the original width prior The anterior aspectThis of thePDFa alveolar site is hostedclef availablet is thus coverby foredMedknow freeto graf downloadting. Publications But when the from patient has no access to ortho, with stem cell-containing mucoperiosteum. Disadvantages S flap alveolar reconstruction can be combined with of S flap relate to the temporary anatomic disruption of DFR to produce a permanent, aesthetically pleasing and periocoronal attachments. physiologic result in one stage.

The S flap has distinct indications for use in primary and Step 5. Dissection of the primary palate secondary cleft repair. These are of vital importance, Beginning with the lateral nasal wall, the mucoperiosteum particularly when one must operate under conditions that is elevated off the maxillary alveolar bone. The palatal are less than ideal. In a primary cleft, whenever possible, margin is undermined as well. This results in a large pre-surgical orthodontic control of the arch should be mucoperiosteal envelope. This must be separated into accomplished. This may also involve a lip adhesion. nasal and oral components. This is done at the level of the The entire effort is directed toward normalizing arch “shoulder” of the alveolus. The incision can be extended dimensions. In these cases, compound S flaps are not backward a few mm along the edge of the palatal shelf. required. Closing the anterior wall of the alveolar cleft On the medial side, the septal mucoperichondrium and can be accomplished with a rectangular superiorly-based the vomerine mucoperiosteum are elevated in continuity.

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The surgeon then proceeds forward to elevate the to the GPA. This permits downward dissection of the mucoperiosteum off the premaxilla. Separation of the mucoperiosteum from the shoulder of the premaxilla or envelope occurs at the “shoulder” of the premaxilla. A upward dissection from the oral margin. The premaxilla vomer mucoperiosteal flap is elevated and sutured to the remains alive, based on its other sources. In bilateral nasal mucosa of the cleft maxilla. palate clefts, no such palatal anastomosis exists. The mucoperiosteum can only be reflected upward. If this is A six-sided “box” is created. The medial and lateral walls done on both sides and if the prolabium is simultaneously are raw bone. The floor is oral mucoperiosteum. The elevated, premaxillary necrosis can ensue. anterior wall is the sliding sulcus mucoperiosteum. The roof is the nasal mucoperiosteum + the NPP flap. The This principle is exactly the same as in prolabial death posterior wall is the vomer flap. These flaps are loaded reported by Millard, a combination of bilateral rotation with undifferentiated mesenchymal stem cells (MSC). The backcuts (destroying the anterior ethmoid supply to cambium layer of the periosteum is especially rich in stem the philtrum) and elevation away from the underlying cells. All neural crest MSCs contain membrane-bound premaxilla.[55] In bilateral cleft lip and palate, the anatomy receptors for BMP. This pocket is now ready to be filled of both prolabium and premaxilla consists of paired with rib graft (iliac crest graft in older children) or with angiosomes. Disruption can occur in one or the other an rhBMP-2/ACS implant. with impunity, but not both.

Alveolar reconstruction, both primary and secondary, Fortunately, the robust blood supply of the maxilla comes demands precise knowledge regarding the vascular to our rescue. Injection studies demonstrate the maxilla anatomy of mucoperiosteum bordering the alveolar to be supplied by the pharyngeal branch of the facial cleft. This is a neglected area of cleft surgery. The blood artery and facial branch of the ascending pharyngeal supply to the premaxilla is the most complex (and artery.[56] It is independent of its mucoperiosteal cover. potentially treacherous). It is derived from three sources. Meticulous .com).dissection of the lateral alveolar cleft creates (1) Premaxilla is an r2’ derivative. Like all mesenchymal a continuous mucoperiosteal sheet uniting the lateral structures caudal to r1, premaxilla is supplied from the nasal wall with that of the hard palate. This contains a external carotid. The vascular axis of the vomer and mirror-image external carotid anastomosis, this time premaxilla is the medial sphenopalatine (nasopalatine) between the medial sphenopalatine and greater palatine artery. PMx mucoperiosteum supplied by the SPA envelops arteries. This can be stripped downward with impunity. It the bone from above-downward, like a cloak thrown over.medknow a is a long flap, extending from just in front of the inferior chair. (2) From below, PMx mucoperiosteum is continuous turbinate all the way to the hard palate. The lateral alveolar with that of the r2 maxillary palatal shelf (MxP), the mucoperiosteal flap is of sufficient size to close the entire arterial axis of which is the external carotid-based(www greater floor of the alveolar cleft. palatine artery. (3) a second anastomosis exists within the mucoperiosteum covering the anterior superior aspect of What then should be done for children BCL(P)? Must the premaxilla. ThisThis zone PDF isa in site contact is hosted withavailable the sof tby tissues forMedknow freeone sacrifice download Publications arch reconstruction from to facial aesthetics or of the prolabium, a p5 derivative supplied by the internal vice versa? The answer (no!) lies in a staged approach carotid-based anterior ethmoid arteries. with careful dissection. Primary lip reconstruction must not violate the tissue plane separating the prolabium Premaxillary survival depends upon maintenance of and mucoperiosteum. Even in a bilateral dissection, sufficient contact between the bone and its enveloping 50% of the premaxilla will remain perfused by both SPA mucoperiosteum. Alveolar cleft reconstruction cannot be and AEA vessels. Lateral nasal wall reconstruction with accomplished without stripping away the vascular coverage the cymba composite graft is appropriate, Liberation of PMx, to a greater or lesser degree. Thus, premaxillary of the medial crura must be done without entering the vascular anatomy dictates the direction in which the territory of the medial sphenopalatine artery. If this mucoperiosteum must be elevated. Surgical strategy for appears difficult, nasal elevation and lip revision may this maneuver is a direct consequence of the cleft type. have to be staged later. Alveolar cleft closure should be done by elevating the premaxillary mucoperiosteal In unilateral palate clefts, the medial alveolar cleft margin flap upward and the maxillary mucoperiosteal flap is a continuous zone of external carotid from the SPA downward.

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Wide bilateral clefts require alternative strategies. Two The tip is positioned anatomically using the Cronin nasal principles to avoid trouble in these patients are: (1) retractor (Padgett Instruments). The medial crura are meticulous primary closure of the nasal floor; and (2) battened together with 5-0 PDS. Suture suspension and delayed grafting of the alveolar cleft. A wide alveolar modification of the alar cartilage can be readily executed “roof ” defect requires big nasal floor flaps. These are best by means of the open-closed approach as per the surgeon’s harvested at the primary surgery, before other tissues get preference. The author’s approach is predicated on in the way. The key point here is to not cut the lateral the establishment of normal field relationships. Wide mucoperiosteal flap until the medial flap is harvested dissection of the nasal soft tissues combined with release from the premaxilla. Once the dimensions of medial flap from their “piriform prison” allows all fields to be passively are known, a superiorly-based lateral flap of sufficient held in position by a nasal stent inserted at the conclusion size is elevated to complete the anterior nasal closure. of the procedure. The author finds the Koken-type silicon At this point we are still left with a wide alveolar “floor” stents (Porex Corp, Newman, Georgia) easy to use. The defect and not enough tissue to close it. What does one stent is placed at the end of the surgery. Closure of the do? One waits it out, using a palatal acrylic splint (secured nostril incision starts at the intermediate crus working with pins or 2 mm screws) to maintain the transverse arch medially down to the Reinisch flap. One then proceeds dimensions and prevent collapse. laterally to the margin of the lateral nasal wall. This involves placing 2-3 sutures over 5 mm. 6 months later, at palatoplasty, the long mucoperiosteal flaps elevated from the hard palate provided ideal coverage The remainder of the lateral nasal incision will be filled by for the alveolar cleft “floor.” Bilateral compound S flaps are inset of the B flap. Reconstruction of the frontal process raised. These will adequately seal off the anterior face of of the premaxilla adds the missing tissue to the lateral the alveolar cleft. The graft (using rhBMP-2 or rib) is placed. nasal wall (see below). This frequently involves placement Once again, care must be taken to prevent transverse of a composite for full thickness graft into the defect. The collapse until the bone graft has hardened. Intraoral medial crural.com). complex is then elevated with respect to the splinting is thus continued for another 2-3 months. septum with 4-0 vicryl. This also closes the membranous septum counter-incision. Secondary grafting in bilateral cases follows the same principles. The vascular anatomy may have been altered Step 2. Soft tissue reconstruction of the premaxilla (primary surgery may have elevated the prolabium The roof of the missing premaxillary field is reconstructed away from the premaxilla). In such cases some degree.medknow based on meticulous closure of the nasal floor. This is of revascularization to the premaxilla occurs over carried out using a mouth gag, starting anteriorly at time. Nonetheless, dissection of the premaxillary the incisive foramen. Because the space is tight using mucoperiosteum must be sparing. All that (wwwis required 5-0 Vicryl® on a small P-2 needle is helpful. The medial is elevation of sufficient tissue to close the soft tissue vomerine and lateral nasal mucoperiosteal flaps are closed defect and place the graft. Nasal tip elevation can be all the way posterior to the end of the vomer flap. This safely accomplishedThis using PDFa siteDFR istechnique hosted available and by cymba forMedknow freeprovides download corr Publicationsect orientation from for inset of the B flap. The grafts. The key point is to preserve soft tissue continuity posterior margin of B is sutured from medial to lateral along between the vomer, the septal mucoperichondrium and the newly-united nostril floor. The tip of B is eventually the premaxilla: this is the site of entry of blood supply inset into the donor site of the lateral crural advancement into the premaxilla. flap. Next, the alar nasal skin flap C is sutured to the anterior margin of B. The surface area of the lateral nasal Closure sequence wall is now restored. Step 1. Elevation of the nasal tip The lateral crus is stabilized into symmetry with the The floor of the missing premaxillary field is reconstructed normal side using a transcutaneous 4-0 chromic mattress when the medial and lateral mucoperiosteal flaps stitch. The V-shaped limbs of the releasing incision are harvested from the walls of the alveolar cleft are turned closed. The donor site is reconstructed using a full- down into the mouth using 4-0 vicryl. The cleft side thickness retroauricular graft or a composite graft of sliding sulcus flap S is advanced and secured using 4-0 anterior ear skin and cartilage. By trial and error I prefer Vicryl PS-2 around the dental units. Three or four such the cymba as the donor site. sutures will suffice. Optional suspension of the sulcus to

Indian J Plast Surg January-June 2007 Vol 40 Issue 1 94 Developmental ﬁeld reassignment in unilateral cleft lip maintain height can be done by passing a 3-0 vicryl up Delaire 2: The purpose of this suture is to control the to the nasal floor and then back down into the sulcus as height and curvature of the cleft side nostril. This is a mattress suture. accomplished by connection of the nasalis to the anterior nasal spine However this step is optional at this point. If Step 3. Osseous reconstruction of the premaxilla: In situ performed, care should be taken to not tighten this and osteogenesis (ISO) inadvertently narrow the nostril. When the remaining The primary palate is reconstructed with Infuse® bone sutures are placed a, decision can be made if a Delaire 2 graft (Memphis, Tennessee Sofamor-Danek). The implant is is warranted. I usually decide upon a Delaire 2 at the end prepared by soaking a Helistat® activated collagen sponge of the case to accentuate curvature (ACS) (Plainsfield, N.J. Integra Life Siences) of preselected size with reconstituted rhBMP-2 applied uniformly over the Orbicularis closure: Three or 4 sutures of 4-0 PDS are ACS. The minimum time required for binding is 15 minutes; required for the DOO layer. The SOO layer is closed with however, I usually wait 30 minutes. The Infuse® comes in 5-0 PDS making sure the loop is at the level of the dermal- three kit sizes 4.2 cc, 5.6 cc and 8.4 cc. These are used as epidermal junction. follows: primary unilateral (small), primary bilateral (medium), secondary unilateral (medium) and secondary bilateral (large). Delaire 3: The oblique head of SOO sets the aesthetic drape The volume of the implant should match that of the defect. of the lip. The 5-0 PDS is obliquely passed upward from Mastergraft® tricalcium phosphate is useful for “bulking up” the cephalic edge through the base of the columella and the implantation site. It acts passively, a space-occupying then back down to the SOO as a mattress suture. agent. One could use freeze-dried bone as well (the ideal bulking agent does not yet exist.) The Mastergraft is wrapped Step 5 Final adjustments: finessing the nostril floor with the ACS collagen sponge like a fajita or sushi roll and After closure of the lip, the alar base C is, at time, placed into the defect. I like to place a vertical component compressed medially by the movement of the neighboring into the alveolar walls and a horizontal component below D flap (lateral.com). lip element). If so, the alar base must the nasal floor. The pocket created by DFR dissection can be be translocated laterally. This can be accomplished by filled with iliac crest graft as well. excision of a crescent of skin from the lateral lip element. C is then elevated and secured to the lateral via a buried Step 4. Closure of the lip: Delaire concepts modified 5-0 PDS suture. At times it is necessary to elevate the Many years ago pioneering work by Delaire demonstrated ala completely in order obtain sufficiently lateralization. that wide subperiosteal dissection yield significant aesthetic.medknow p The tip of the nostril sill flap C’ is now sutured just benefits of tissue draping and, at the same time, did not posterior to the columellar shoulder. Continuity between impair maxillary growth.[57-58] Indeed, when compared with the columellar shoulder and the nostril sill is now supraperiosteal release, this approach is developmentally(www reestablished. Perialar suturing with inverted 5-0 PDS correct because it separates the osteogenic functional sutures restores the alar crease very nicely. matrix (the periosteum and overlying soft tissues) from the product (the premaxillaThis andPDFa maxilla).site is hosted available by forMedknow freeA final caveatdownload Publications concerns the from lining of the lateral nasal defect. Experience with this technique shows that prevention of The suture sequence is as follows: post operative contraction is paramount. This requires Delaire 1: Centralization of the bilaterally displaced soft placement of a Porex® silicon conformer sized to the tissues is accomplished with 4-0 nylon sutures placed from patient. The stent should be sutured in place with 4-0 below the flap to the paranasalis (levator) insertion into the nylon for 8 weeks. It is important that the B flap not be superficial orbicularis oris (SOO). This insertion does not sutured to the sidewall of C with any tension. If tension involve the alar base. It is generally located one fingerbreadth exists I have found it prudent to use a small pinch graft lateral to the alar base. The non-cleft side is sutured first of retroauricular skin. to below the anterior nasal spine. This suture serves as a reference point for a similar suture from the cleft side. DISCUSSION

Suspension of the DOO: This suture sets the depth of the Whenever a surgical procedure for a congenital condition sulcus on the cleft side. At the most cephalic margin the in a growing child leads to a predictable pattern of relapse DOO is suspended from the septum with a 4-0 PDS. over time two, inescapable conclusions must be drawn: (1)

95 Indian J Plast Surg January-June 2007 Vol 40 Issue 1 Carstens the biologic rationale of the procedure is incorrect; and The membranous bones of the maxilla and premaxilla (2) the anatomic pattern of relapse points an accusatory are just a product of the soft tissue functional matrix finger at the pathology. DFR surgery represents a new that surrounds them. As the soft tissue grows, new bone form of thinking about clefts by identification and is deposited and old bone resorbed according to the rearrangement of specific developmental fields. The mechanical forces placed on the bone. The purpose of primary pathology of cleft lip is hypoplasia or absence of subperiosteal centralization in DFR is to change the biologic the distal premaxilla. DFR is designed to reconstruct the relationship of the bone product to the functional matrix. missing premaxilla using osteoinductive technologies for Over time, the former will adapt to the latter in its new, stem cell concentration and differentiation such as rhBMP centralized position. Appreciation of the subperiosteal 2 or by conventional osteoconductive technique (bone plane as the correct approach to surgically separate the grafting). DFR incisions are made on the basis of vascular functional matrix from its product (bone) ensures tension- supply and embryology, not geometric manipulation. free release and accuracy of muscle repair (individual For this reason the design of DFR presents a series of muscle units can be identified by tugging on them from specific solutions that speak to problems in cleft surgery below). Thirty years of work by Delaire and others confirm hitherto inadequately addressed: distortion of the nasal the clinical accuracy and safety of this approach. Readers envelope and septum, displacement of soft tissues away are also advised to study the pioneering work on primary from the midline, the entrapped position of the medial bone grafting done by Rosenstein.[59-60] This 30 year follow- crus and the “hidden” nostril sill. DFR treats mechanisms, up of dental development in patients treated with careful not appearance. presurgical orthodontics and rib grafting demonstrates clearly the safety and advantages of gaining control over The extraoral design of DFR is invariable; it is the same for the alveolar cleft at primary surgery. both unilateral and bilateral clefts. Developmental fields are recognized, separated and rearranged into correct The concepts and techniques of DFR are applicable to all anatomic relationships. The intraoral design varies with forms of clef.com).ts. Because it separates out osseous pathology cleft type. If no alveolar cleft is present, subperiosteal from soft tissue pathology, DFR is a true “cut as you go” release and zygomaticomaxillary buttress backcut are technique. In secondary cleft surgery, DFR is capable of sufficient to achieve centralization of the soft tissue rescuing previously violated fields and reuniting them envelope. Gingival release is not necessary. If a primary into their correct functional relationships. Sliding sulcus palate cleft exists, a complete sliding sulcus dissection flaps function as elastic flaps as defined by Goldstein[61-62] with gingival release will allow for mesial translocation.medknow and can readily be brought into the midline. Abbe flaps and coverage of the alveolar cleft. are virtually unnecessary, even in salvage cases involving total loss of the philtrum. DFR includes a straight-line repair, resulting(www in an anatomically correct philtral column. The distorted and DFR is a practical application of developmental anatomy. It seemingly foreshortened philtrum in clefts is familiar provides a means to analyze membranous bone formation to all plastic surThisgeons. PDF aFor site many is hostedyears, available the r obytation- forMedknow freeand periosteal download Publications physiology from. It embodies concepts of facial advancement technique has been used to reorient the growth central to orthodontics. It makes use of neuromeric philtrum. So why is philtral rotation not required in DFR? theory to map out and manipulate developmental fields. The appearance of the philtrum in clefts stems from the Proper implementation and study of cleft repair using overall malposition of the soft tissue in a falsely lateralized DFR will provide a forum for dialog among craniofacial state. Thus, the philtrum and columella appear to be surgeons orthodontists and developmental biologists. short, but are actually displaced into the nasal tip. DFR achieves de-rotation of the philtrum and columella by REFERENCES the following: (1) field separation; and (2) subperiosteal mobilization. The entire soft tissue complex in a unilateral 1. Veau V, Recamier J. Bec-de Lievre: Formes Cliniques-Chirugie. cleft is laterally displaced “around the corner” of the Masson et Cie: Paris; 1938. premaxilla. When mobilized correctly off the bone it is 2. His W. Beobachtungen zur Geschichte und Gamenbildung beim menschlichen Embryo. Kgl. Akad Wiss: 1901. p. 27. brought forward around the curve of the bone and drops 3. Carstens MH. Development of the facial midline. J Craniofac Surg right into place. 2002;13:129-90.

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4. Carstens MH. Neural tube programming and craniofacial cleft prophylaxis of cosmetic deformities and de›ciencies. Institute of formation. I. The neuromeric organization of the head and neck. Cosmetology: Moscow; 1982. p. 118-21. Eur J Paediatr Neurol 2004;8:181-210. 27. Vissarionov VA. Correction of the nasal tip deformity following 5. Puelles L, Rubenstein JL. Development of the central nervous unilateral repair of clefts of the upper lip. Plast Reconst Surg system. In: Rossant J, Tam PP. Mouse development: Patterning, 1989;83:341-7. Morphogenesis and organogenesis. Academic Press: San Diego; 28. Zhang Z, Song Y, Zhao X, Zhang X, Fermin C, Chen Y. Rescue of 2002. p. 37-54. cleft palate in Msx-1 de›cient mice by transgenic Bmp4 reveals a 6. Puelles L, Rubenstein JL. Forebrain gene expression domains and network of BMP and Shh signaling in the regulation of mammalian the evolving prosomeric model. Trends Neurosci 2003;26:469- palatogenesis. Development 2002;129:4135-40. 76. 29. Gilbert S. Developmental Biology, 8th ed. Mass, Sinauer: 7. Rubenstein JL, Puelles L. Development of the nervous system. Sunderland; 2006. In: Epstein CJ, Erickson RP, Wynshaw-Boris A, editors. Inborn 30. Wozney J. Overview of bone morphogenetic proteins. Spine errors of metabolism: The molecular basis of clinical disorders of 2002;27:52-8. morphogenesis. Oxford University Press: Oxford, England; 2004. 31. Ebara S, Nakayama K. Mechanisms for the action of bone p. 75-88. morphogenetic proteins and regulation of their activity. Spine 8. Carstens MG. Functional matrix cleft repair: A common strategy for 2002;27:S10-5. unilateral and bilateral clefts. J Craniofac Surg 2000;11:437-69. 32. Valentin-Opran A, Wozney J, Csiima C, Lilly L, Reidel GE. Clinical 9. Carstens MH. Function matrix cleft repair: Principles and evaluation of recombinant human bone morphogenetic protein-2. techniques. Clin Plast Surg 2004;31:159-89. Clin Orthop Related Res 2002;395:110-20. 10. Lemire RJ, Cohen MM Jr, Beckwith JB, Kokich VG, Siebert JR. 33. Boyne PJ. Animal studies of application of rhBMP-2 in maxillofacial The facial features of holoprosencephaly in anencephalic human reconstruction. Bone 1996;19:83S-92S. specimens. I. Historical review and associated malformations. 34. Boyne PJ, Nath R, Nakamura A. Human recombinant BMP-2 in Teratology 1981;23:297-303. osseous reconstruction of simulated cleft palate defects. Br J Oral 11. Siebert JR, Kokich VG, Beckwith JB, Cohen MM Jr, Lemire RJ. Maxillofac Surg 1998;36:84-90. The facial features of holoprosencephaly in anencephalic human 35. Boyne PJ, Marx RE, Nevins M, Triplett G, Lazaro E, Lilly LC, et specimens. II. Craniofacial anatomy. Teratology 1981;23:305-15. al. A feasibility study evaluating rhBMP-2/absorbable collagen 12. Liem KF Jr, Tremml G, Roelink H, Jessell TM. Dorsal differentiation sponge for maxillary sinus ﬁoor augmentation. Int J Periodontic of neural plate cells induced by BMP-mediated signals from Restorative Dent 1997;17:11-25. epidermal ectoderm. Cell 1995;82:969-79. 36. FDA Dental Advisory Board hearing on Infuse® bone graft 13. Liem, KF, Bemis WE, Walker WF, Grande L. Functional anatomy for maxillary sinus and alveolar extraction socket defects. of the vertebrates: An evolutionary perspective, 4rd ed. Thompson: Gaithersburg:.com). Maryland; November 9, 2006. Belmont, California; 2006. 37. Carstens MH, Chin M, Li J. In situ osteogenesis (ISO): Regeneration 14. Barteczko K, Jacob M. A re-evaluation of the premaxillary bone of 10-cm defect in porcine model using recombinant human bone in humans. Anat Embryol (Berlin) 2004;207:417-37. morphogenetic protein-2 (rhBMP-2) and Helistat ® absorbable 15. Mooney MP, Siegel IP, Kimes KR, Todhunter J. Premaxillary collagen sponge. J Craniofac Surg 2005;16:1033-42. development in normal and cleft lip and palate human fetuses 38. Chao M, Donovan T, Sotelo C, Carstens MH. In situ osteogenesis using three-dimensional computer reconstruction. Cleft Lip Palate of hemimandible in a 9-year-old boy: Osteoinduction via stem cell J 1991;28:49-54. concentration. J Craniofac Surg 2006;17:405-12. 16. Carstens MH. The spectrum of minimal clefting: Process-oriented.medknow 39. Ruberte J, Carretero A, Navarro M, Marcucio RS, Noden D. cleft management in the presence of an intact alveolus. J Morphogenesis of blood vessels in the head muscles of avian Craniofac Surg 2000;11:270-94. embryo: spatial, temporal, and VEGF expression analyses. Dev 17. Gong SG, Guo C. BMP4 gene is expressed at the putative site Dynam 2003;227:470-83. of fusion in the midfacial region. Differentiation 2003;71:228-36.(www 40. Carstens M, Chin M, Ng T, Tom WK. Reconstruction of #7 facial 18. Ashique AM, Fu K, Richman JM. Endogenous bone morphogenetic cleft with distraction assisted in situ osteogenesis (DISO): Role of proteins regulate outgrowth and epithelial survival during avian lip recombinant human bone morphogenetic protein-2 with Helistat fusion. Development 2002;129:4647-60. activated collagen sponge. J Craniofac Surg 2005;16:1023-32. 19. Lumsden A, KrumThisﬁauf R.PDFa Patterning site is hostedthe available vertebrate byneuraxis. forMedknow free41. Chin download M, PublicationsNg T, Tom WK, Carstensfrom MH. Repair of alveolar clefts Science 1996;274:1109-15. with recombinant human bone morphogenetic protein. J Craniofac 20. Noden DM. Patterning of avian craniofacial muscles. Dev Biol Surg 2005;16:778-89. 1986;116:347-56. 42. Chin M, Ng T, Tom WK, Carstens MH. 5th International Conference 21. Noden DM. Interactions and fates of avian craniofacial on Distraction Osteogenesis in Craniofacial Surgery. Paris, mesenchyme. Development 1988;103:121-40. France; June, 2006. 22. Noden DM. Origins and patterns of craniofacial mesenchymal 43. Carstens MH. The sliding sulcus procedure: Simultaneous repair tissues. J Craniofac Genet Dev Biol 1991;11:192-213. of unilateral clefts of the lip and primary palate-a new technique. 23. Noden DM, Trainor PA. Relations and interactions between cranial J Craniofac Surg 1999;10:415-29. mesoderm and neural crest populations. J Anat 2005;207:575- 44. Demas PN, Sotereanos GC. Closure of alveolar clefts with 601. Corticocancellous block grafts and marrow: A retrospective study. 24. Helms JA, Cordero D, Tapadia MD. New insights into craniofacial J Oral Maxillofac Surg 1988;46:682-7. morphogenesis. Development 2005;132:851-61. 45. Delaire J. The potential role of facial muscles in monitoring 25. Carlson BR. Human embryology and developmental biology, 3rd maxillary growth and morphogenesis. In: Carlson DS, McNamara ed. Mosby: St. Louis; 2004. JA Jr, editors. Muscle Adaptation and Craniofacial Growth. 26. Vissarionov VA. Analysis of the results of reconstructive plastic (Center for Human Growth and Development, Craniofacial Growth surgery of unilateral clefts of the upper lip based on data of the Monograph No. 8). University of Michigan: Ann Arbor, MI; 1978. surgical section 1970-1977. In: Pathogenesis, treatment and p. 157-80.

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46. Markus AF, Delaire J, Smith WP. Facial balance in cleft lip and reference as to the anatomy of the premaxilla. Previous papers palate. I. Normal development and cleft palate. Br J Oral Maxillofac describing the non-existence of the human premaxilla debunked. Surg 1992;30:287-95. Butler, AB, Hodos W. Comparative Vertebrate Neuroanatomy. New 47. Moss ML, Vilmann H, Das Gupta G, Skalak R. Craniofacial growth in space-time. In: Carlson DS, McNamara Jr JA, editors. York: Wiley-Liss, 1997. Fundamental conservation of neuroembryologic Craniofacial biology. Center for Human Growth and Development, developmental units is described. Functional model relating motor and University of Michigan: Ann Arbor, MI; 1981. sensory neural structures serving between axial and pharyngeal arch 48. Park GC. The importance of accurate orbicularis repair of the orbicularis oris muscle in unilateral cleft lip. Plast Reconstr Surg musculature in alternating pairs of somitomeres. 1995;96:780-8. Carlson BR. Human Embryology and Developmental Biology 3rd ed. 49. Carstens MH. Correction of the bilateral cleft using the sliding Philadelphia: Mosby, 2004. Very clear account of how an organism sulcus technique. J Craniofac Surg 2000;11:137-67. forms with a good dose of modern biology. Exceptional illustrations. 50. Carstens MH. Correction of the unilateral cleft lip nasal deformity using the sliding sulcus procedure. J Craniofac Surg 1999;10:346- Francis-West PH, Robson I, Evans DJ. Craniofacial development: 64. the tissue and molecular interactions that control development of the 51. McComb H. Treatment of the unilateral cleft lip nose. Plast head. Adv Anat Embryol Cell Biol 2003; 169:1-138. New concepts of Reconstr Surg 1975;55:596-601. 52. Stenstrom SJ. The alar cartilage and the nasal deformity in developmental biology applied to the head are succinctly reviewed. unilateral cleft lip. Plast Reconstr Surg 1966;38:223-31. Futayama D. Evoution. Sunderland, Massachusetts: Sinauer, 2005. Up- 53. Drake AF, Davis JU, Warren DW. Nasal airway size in cleft and to-date discussion of the mechanisms underlying evolution, including noncleft children. Laryngoscope 1993;103:915-7. Hox genes and the dawn of modern evolutionary developmental 54. Noordhoff MS, Chen YR, Chen KT. The surgical technique for the complete unilateral cleft lip-nasal deformity. Op Tech Plast Surg biology. 1995;2:164-74. Gilbert S. Developmental Biology, 8th ed. Sunderland, Massachusetts, 55. Noordhoff MS. The surgical technique for the unilateral cleft 2006. Written for advanced undergrads, this outstanding text is lip-nasal deformity. Noordhoff Craniofacial Foundation: Taipei, Taiwan; 1997. sumptuously-illustrated; the accompanying DVD and website, www. 56. Millard DR. Cleft Craft II. Bilateral and rare deformities. Little, devbio.com, have a wealth of detail including real-time movies of key Brown: Boston; 1977. p. 259-65. developmental processes. This is the medical professional’s “go-to” 57. Siebert JW, Angrigiani C, McCarthy JG, Longaker MT. Blood supply of the Lefort I maxillary segment: an anatomic study. Plast source to understand.com). molecular embryology. Reconstr Surg 1997;100:843-51. Hall BK. The Neural Crest in Development and Evolution. New York: 58. Delaire J, Precious D, Gordeef A. The advantage of wide Springer, 1997. Very readable discussion of the 4th germ layer and the subperiosteal exposure in primary surgical correction of labial source of nearly all craniofacial bones. maxillary clefts. Scand J Plast Reconstr Surg Hand Surg 1989;22:147-51. Hanken J, Hall BK. The Skull. Chicago: University of Chicago Press, 59. Precious DA, Delaire J. Surgical considerations in patients with cleft 1993. This series includes 3 volumes (Development, Patterns of deformities. In: Bell WH, editor. Modern practice in orthognathic Structural and Systematic Diversity, Functional and Evolutionary and reconstructive surgery. WB Saunders: Philadelphia; 1992..medknow p. 390-425. Mechanisms) summarizing the entire literature. 60. Rosenstein SW, Grassechi M, Dado DV. A long-term retrospective Huang R, Zhi Q, Patel K, Wilting J, Christ b. Contribution of single assessment of facial growth, secondary surgery need and somites to the skeleton and muscles of the occipital and cervical regions maxillary lateral incisor status in a surgical-orthodontic(www protocol in avian embryos. Anat Embryol 2000; 202:375-383. Mapping study of for complete clefts. Plast Reconstr Surg 2003;111:1-13. 61. Rosenstein SW. Early bone grafting of alveolar cleft deformities. neural crest cells from a single somite leading was later expanded. J Oral Maxillofac Surg 2003;61:1078-81. Hu D, Marcucio RS, Helms JA. A zone of frontonasal ectoderm 62. Goldstein MH. A tissue expanding vermilion myocutaneous fiap This PDFa site is hosted available by forMedknow freeregulated download patterning Publications and growth from in the face. Development 2003; for lip repair. Plast Reconstr Surg 1984;73:768-70. 63. Goldstein MH. The elastic fiap for lip repair. Plast Reconstr Surg 130:1749-1758. Neural crest derivatives such as bone and cartilage 1990;85:446-52. involve exchange of information between epithelia and mesenchyme. The model reported here may explain frontal bone development as “programming” by p5 skin/dermis and underlying p5 brain covered by r1 dura. The presence of two distinct templates may “map” the frontal ANNOTATED BIBLIOGRAPHY bone into two distinct laminae, the interface between is a potential space, the frontal sinus. Basic science/embryology Jacobson AG. Somitomeres: mesodermal segments in the head and These references provide a basis for understanding the trunk. In: Hanken J, Hall, BK (eds). The Vertebrate Skull. Vol I. Pp. 42-76. neuromeric system and developmental fields. Chicago: Univ Chicago Press, 1993. Clear discussion of somitomeres. Both Carlson and Gilbert make use of this work in discussing how Barteczko K, Jacob M. A re-evaluation of the premaxillary bone in embryonic tissues are segmentally organized. Fate maps of craniofacial humans. Anat Embryol (Berlin) 2004; 207:417-437. The definitive mesenchymal derivatives are based on this concept.

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Kjaer I, Keeling JW, Fischer-Hansen B. The Prenatal Human Cranium– Puelles L, Rubenstein JLR. Forebrain gene expression domains and Normal and Pathologic Development. Copenhagen, Denmark: the evolving prosomeric model. Trends Neurosci 2003;26:469-476. Munksgaard, 1999. Slim volume packed with important ideas. Careful Revised version of prosomeric theory. analysis of craniofacial bone ossification is the conceptual basis for the Rubenstein, JLR, Regionalization of the prosencephalic neural plate. Lego® model of sequential developmental field assembly. Not afraid Annu Rev Neurosci 1998; 21:445-477. Gene product mapping used to speculate…ahead of its time. to defi ne prosomeres. LeDouarin NM, Kalcheim C. The Neural Crest, 2nd ed. Cambridge, UK: Tapadia MD, Cordero DR, Helms JA. It‘s all in your head: new insights Cambridge University Press, 1999. Gerard Couly and Nicole LeDourain into craniofacial development and deformation. J Anat 2005;207:461 - revolutionized neural crest embryology with the quail-chick chimera 477. Excellent synthesis of new ideas, including the premandibular model. This laboratory has mapped out the origins and fate of neural arch. Illustrations are fi rst rate. crest cells throughout the embryo. Cleft Lip and Palate Surgery Liem, KF, Bemis WE, Walker WF, Grande L. Functional anatomy of the These references cover the bases of developmental › eld theory Vertebrates: An Evolutionary Perspective, 3rd ed. Belmont, California: Carstens MH. The sliding sulcus procedure: simultaneous repair Thompson, 2001. No theory of human craniofacial development can of unilateral clefts of the lip and primary palate-a new technique. J exist without reference to other vertebrates. The 4th edition includes Craniofac Surg 1999; 10:415-434. Principles of periosteal anatomy are contributions of Hox genes and neuromeres. applied to surgical correction of soft tissues on both sides of the cleft. Lumsden A, Krumfiauf R. Patterning the vertebrate neuraxis. Science Bardach’s contributions are discussed in a new context. The resulting 1996; 274:1109-15. Key reference correlating neural content of centralization of the facial functional matrix leads to more anatomic rhombomeres. deposition of membranous bone over time. “Process theory” (a model Morriss-Kay GM, Wilkie AOM. Growth and the normal skull vault of cleft formation as a series of pathologic mechanisms/processes and its alteraion in craniosynostosis: insights from Human genetics acting over time) was presented. Surgical correction would therefore and experimental studies. J Anat 2005; 207:637-653. Morriss-Kay become a logical correction of each one of these processes such that demonstrated in previous papers the origin of the parietal bone from the patient would “grow into the repair.” PAM in mammals versus neural crest. Carstens MH..com). Correction of the unilateral cleft lip nasal deformity Muller F, O‘Rahilly. The timing and sequence of appearance of using the sliding sulcus procedure. J Craniofac Surg 1999; 10:346- neuromeres and their derivatives in staged human embryos. Acta 364. Contribution of the deficient piriform platform to alar cartilage Anat 1997;158:83-99. The master plan of cranio-caudal development malposition is combined with concepts of mechanical displacement of is described. This is the basis of homeotic-gene specifi c levels. the premaxilla and maxilla. Soft tissue deficiency of the lateral nasal O‘Rahilly R, Muller F. Developmental Stages in Human Embryos. wall not yet linked to the premaxillary field. Washington, D.C.: Carnegie Institution Publication 637, 1987. The.medknow Carstens MH. Correction of the bilateral cleft using the sliding sulcus Carnegie staging system for human embryos is described here. This technique. J Craniofac Surg 2000; 11:136-167. Commonality of reference is used by embryologists world-wide. pathology between unilateral and bilateral clefts is emphasized O‘Rahilly R, Muller F. The Embryonic Human Brain:(www An Atlas but incision design reflects lack of understanding of soft tissue of Developmental Stages, 2nd ed. New York: Wiley-Liss, 1999. developmental field anatomy. Embryonic origin of facial musculature Visualization of neuroaantomy in motion visually comprehensible for and the anatomy of the philtrum are discussed. any surgeon. This PDFa site is hosted available by forMedknow freeCarstens download MH. Publications The spectrum of from minimal clefting: process-oriented cleft Osumi-Yamashita N, Ninomiya Y, Doi H, Eto K. The contribution of management in the presence of an intact alveolus. J Craniofac Surg both forebrain and midbrain crest cells to the mesenchyme in the 2000;11:270-94. For the first time I was able to grasp the sequential frontonasal mass of mouse embryos. Dev Biol 1994;164:409-419. assembly of soft tissues around the cleft. The genetic signalling Timing of when PNC, MNC and RNC populations complete migration relationships between the underlying premaxillary field and the comes from this lab. overlying soft tissues was as yet unknown. By this time I was aware Osumi-Yamashita N, Ninomiya Y, Eto K. Mammalian craniofacial of neuromeric theory but had not yet envisioned the “layering” off facial embryology in vitro. In J Dev Biol 1997;41:187-194. soft tissue fields over bone fields. Puelles L. Brain segmentation and forebrain development in amniotes. Carstens MH. Sequential cleft management with the sliding sulcus Brain Res Bull 2001; 55:695-710. Luis Puelles and John Rubenstein technique and alveolar extension palatoplasty. J Craniofac Surg 2000; discovered and mapped out the segmental organization of the forebrain 11:437-469. As described by Delaire, cleft palate shares with cleft lip (prosencephalon). Neural crest in association with a given prosomere common pathophysiologic mechanisms that, over time, produce the creates external mesenchymal structures (forehead, nose, philtrum, eye), final anatomic state. Both buccal and lingual aspects of the alveolus the development of which coincides with the corresponding zones in the have a similar pattern of blood supply. Preservation of the entire brain. Anomalies are often shared. “The brain predicts the face.” alveolar mucoperiosteum as a osteosynthetic developmental field

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is a key point of the surgery. The additional length gained by the with references. AEP procedure prevents residual anterior fistula. The importance of Schendel SA, Tessier P, Tulasne J-F. Facial clefting disorders and Schweckendieck’s work is discussed. craniofacial dysostoses: skeletal considerations. In: Turvey TA, Vig Carstens MH. Functional matrix cleft repair. J Craniofac Surg KWL, Fonseca RJ. Facial Clefts and Craniosynostosis: Principles and Carstens MH. Function Matrix Cleft Repair: Principles and Techniques. Management. 1996. Philadelphia: WB Saunders, pp. 95-128. Good Clin Plast Surg 2004; 31:159-189. Detailed discussion of “how to do skull photos of the synostoses. it.” As my understanding of developmental fields improved the FMR Tessier P. Fentes orbito-faciales verticales et obliques (colobomas) became the DFR (Developmental Field Repair). All the principles of completes et frustes. Ann Chir Plast 1969;19:301-311. Original report FMR are preserved in the current technique. In DFR (as discussed based on his empiric observations helped to organize facial clefts into here), dissection of the philtrum and the lateral nasal wall are recognizeable patterns. combined with the correct placement of the A1 flap and the deliberate Tessier P. Anatomical classi›cations of facial, cranio-facial and laterofacial reconstruction of the premaxilla with rhBMP-2. clefts. J Maxillofac Surg 1976;4:69-92. Seminal contribution in English Carstens MH. Development of the facial midline. J Craniofac Surg establishing a geographic schematization of facial clefts. Developmental 2002;13:129-87. This paper discusses two topics, the neuroembryology field model categorizes clefts into a neuromeric distribution explains of the facial placodes and the neuromeric mapping of neural crest Tessier’s observations on a neuroembryologic basis. derivatives in the face. A three-dimensional model of facial development Vissiarionov VA. Analysis of the results of reconstructive plastic surgery based on Carnegie stages is presented. of unilateral clefts of the upper lip based on data of the surgical section Carstens MH. Neural tube programming and craniofacial cleft 1970-1977. In: Pathogenesis, treatment and prophylaxis of cosmetic formation. I. The neuromeric organization of the head and neck. Eur deformities and de›ciencies. Moscow: Institute of Cosmetology; 1982, J Paed Neurol 2004; 8:181-210. Save for some confusion about the pp. 118-21 number and relative contributions of occipital somites this paper Recombinant Human Bone Morphogenetic Protein-2 constitutes my first-pass introduction into the neuromeric system. Relevant preclinical studies and clinical applications in the maxilla Delaire J. The potential role of facial muscles in monitoring maxillary and mandible. growth and morphogenesis. In Carlson DS, McNamara Jr JA (eds). Boyne PJ. Animal.com). studies of application of rhBMP-2 in maxillofacial Muscle Adaptation and Craniofacial Growth. Ann Arbor, MI: University reconstruction. Bone 1996; 19(suppl):83S-92S. Early work proving of Michigan, 1978, pp.157-180 (Center for Human Growth and BMP resynthesizes membranous bone. Development, Craniofacial Growth Monograph No. 8). Boyne PJ. A feasibility study evaluating rhBMP-2/absorbable collagen Delaire J, Precious D, Gordeef a. The advantage of wide subperiosteal sponge for maxillary sinus fioor augmentation. Int J Periodontic exposure in primary surgical correction of labial maxillary clefts. Restorative Dent 1997;17:11-25. Fiirst clinical application in craniofacial Scand J Plast Recons Surg 1989; 22:147-151. Pioneering work.medknow skeleton was to support maxillary implants. on subperiosteal dissection directly following basic science of Boyne PJ. Application of bone morphogenetic proteins in the treatment membranous bone formation represented a functional approach to of clinical oral and maxillofacial osseous defects. J bone Joint Surg cleft repair. Delaire was the first to emphasize physiological(www principles 2001; 83A(suppl)S146-S150. Demonstrates potential breadth of of soft tissue-bone interaction over time. Reading Delaire opened the applications for Infuse®. door for me to visualize cleft formation as a 4-D process acting over Boyne PJ, Nath R, Nakamura A. Human recombinant BMP-2 in time. DFR surgery Thisis a direct descendentPDFa site is of hosted thisavailable work. by forMedknow freeosseous download reconstruction Publications of simulated from cleft palate defects. Br J Oral Markus AF, Delaire J, Smith WP. Facial balance in cleft lip and palate Maxillofac Surg 1998; 36:84-90. Immediate grafting with BMP I. Normal development and cleft palate. Br J Oral Surg 1992;30:287- maintains the dental arch in primates. This was a key idea for eventual 95. Force vector concept and its effects on bone structure presented incorporation into DFR. and illustrated with clarity. Boyne PJ, Nakamura A, Shabahang S. Evaluation of long-term effect Moss ML, Vilmann H, Das Gupta G, Skalak R. Craniofacial growth of function on rhBMP-2 regenerated hemimandibulectomy defects. Br in space-time. In Carlson DS, McNamara Jr JA (eds). Craniofacial J Oral Maxillofac Surg 1999; 37:344-352. biology. Ann Arbor, MI: Center for Human Growth and Development, Carstens MH, Chin M, Li J. In situ osteogenesis (ISO): Regeneration University of Michigan, 1981. Balanced facial growth requireds that all of 10-cm defect in porcine model using recombinant human bone soft tissue developmental fields become physiologically centralized to morphogenetic protein-2 (rhBMP-2) and Helistat ® absorbable collagen synthesize facial bone in the correct position over time. sponge J Craniofac Surg 2005; 16: 1033-42. Precious DA, Delaire J. Surgical considerations in patients with Carstens M, Chin M, Ng T, Tom WK. Reconstruction of #7 facial cleft with cleft deformities. In: Bell WH (ed.). Modern practice in orthognathic distraction assisted in situ osteogenesis (DISO): Role of recombinant and reconstructive surgery. Philadelphia, WB Saunders; 1992; p. human bone morphogenetic protein-2 with Helistat activated collagen 390-425. Good discussion of Delaire’s technique is presented along sponge. J Craniofac Surg 2005 16(6): 1023-1032.

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Chao M, Donovan T, Sotelo C, Carstens MH. In situ osteogenesis Ruberte J, Carretero A, Navarro M, Marcucio RS, Noden DM. of hemimandible in a 9-year-old boy: osteoinduction via stem cell Morphogenesis of blood vessels in the head muscles of avian concentration. J Craniofac Surg 2006; 17:405-412. 12 cm segmental embryos: spatial, temporal and VEGF expression analysis. Dev mandibular defect reconstructed with BMP-directed ISO. Free ﬁbula Dynam 2003;227:470-483. Important concept suggesting anatomic transfer was avoided. Response of the regenerate to surrounding soft basis of peripheral arterial system via Schwann cell induction from tissue forces was to remodel into a replica of the normal side. peripheral nerves. BMP-directed neural crest release of VEGF to Chin M, Carstens MH. Distraction osteogenesis with bone promote rapid vascularization of surgical site was a concept derived morphogenetic protein enhancement: facial cleft repair in humans. Proc from this work. 4th International Congress on Cranial and Facial Distraction Processes. Seeherman H, Wozney J, Li R. Bone morphogenetic protein delivery Paris, France, 197-200, 2003. DISO was invented with the original #2, systems. Spine 2002; 27(165):516-523. Carriers required to retain #7 cleft repair which we published 2 years later. BMP-2 in the target environment are reviewed. Chin M, Ng T, Tom WK, Carstens MH. Repair of alveolar clefts with Urist, MR, Strates BS. Bone morphogenetic protein. J Dent Res 1971; recombinant human bone morphogenetic protein. J Craniofac Surg 50:1392-1406. The original report. PJ Boyne studied under Urist. 2005; 16(5):778-789. Original study done using DFR concepts in Valentin-Opran, Wozney J, Csiima C, Lilly L, Reidel GE. Clinical secondary cleft cases. evaluation of recombinant human bone morphogenetic protein-2. Ebara S, Nakayama K. Mechanisms for the action of bone Clin Orthop Related Res 2002;395:110-120. Relevant clinical data morphogenetic proteins and regulation of their activity. Spine 2002 succinctly summarized. Very useful reference. 27(16S):S10-S15. Induction of osteoclasts from monocytes sets up Wozney J. Overview of bone morphogenetic proteins. Spine negative feedback loop. 2002;27(165):52-58. Physical characteristics of BMP discussed.

.com).

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EPILOGUE FOR COLLEAGUES IN INDIA AND DFR was born in post-war Nicaragua, under the harshest SOUTHEAST ASIA of conditions. I was assigned there to work for the Pan American Health Organization (WHO) as a surgical I should like to depart at this point from traditional consultant in 1990-1992. At that time, the backlog of academic writing to address the reader in a very personal plastic surgery cases was estimated at 10,000 patients. way. Many of you must treat children and adults with clefts Problems were not only logistical but also the creation under the most demanding of circumstances. Poverty, of manpower. Working with Professor Arturo Gomez at poor communication, distance from medical centers the Universidad Nacional de Nicaragua in Leon and with and difficulties in transportation negatively affect every a small group of lay people, the first medical foundation aspect of your treatment plan. The same is true for your in the history of Nicaragua was formed. Noel Icaza, an patients, no matter how motivated they might be. Time angricultural engineer, gave it the name Nicaplast. We taken to bring a child in for treatment from a remote traveled around the country, operating and working within location may present an intolerable stress on families various cities to create support for this apparently crazy living in these circumstances. For many, if not most, of the idea. Nicaragua had descended into chaos. The gross world’s children with clefts, these factors make careful, national product was just above that of Haiti, making well-regulated multidisciplinary care a distant mirage so this formerly active exporting nation the second-poorest easy to talk about, so difficult to achieve. of all the Americas. Given this situation, the creation of resources to resuscitate reconstructive surgery seemed For these patients the surgical team must set priorities an impossible task. to accomplish the maximum physiologic correction with the minimum of surgeries. The goals at the first stage At this point, Carlos and Vivian Pellas stepped in. These are: (1) a functional nasal airway; (2) a united dental arch remarkable people, from a highly educated and wealthy without collapse; (3) subperiosteal dissection designed family, survived a commercial airline crash in 1989. 170 to promote centric facial growth; and (5) soft tissue perished at.com). the scene. The Pellas were evacuated to correction using developmental field concepts to place Miami where they underwent numerous surgeries for scars into embryonic separation planes. The goals of the burns and fractures including free tissue transfer. Upon follow-up stage are: (1) meticulous three-layer closure recuperating they returned to Nicaragua determined of the soft palate; and (2) use of AEP (alveolar extension to help children with burns and deformities. Their palatoplasty) flaps to prevent anterior fistula. Should the support for Nicaplast resulted in the establishment patient be unable to return for further work, this should.medknow of new residency training at Unan-Leon, improved provide at the very least an intact oral cavity in some infrastucture and a complete modernization of the degree of alignment, a working airway, reasonable speech pediatric burn unit in the capital city, Managua. Partner and an appearance that is socially acceptable.(www relationships were constructed with Interplast, the University of Wisconsin, Physicians for Peace and the Developmental Field Reassignment cleft surgery was AO Foundation in Basel, Switzerland. Each one of these designed with theseThis cir cumstancesPDFa site is hostedin available mind. It is bya “high forMedknow freeentities download contributed Publications towar fromd expanded reconstructive intellect/low technology” method. The only structures surgical care and advanced education. In time, a new missing are a lateral nasal wall soft tissue defect (equal foundation, under the leadership of Vivian Pellas, was to the cymba) and an alveolar bone defect (equal to established. The Asociacion Pro Ninos Quemados de the housing of the lateral incisor). Reconstruction of Nicaragua (APROQUEN) now has direct responsibility these vital elements is within the hands of all cleft for burn care in that country. surgeons. There are only two requirements for DFR to be properly performed: (1) in-depth anatomic knowledge of The residency was particularly innovative. The longest in embryologic field anatomy; and (2) the technical ability Latin America, it demands full general surgery + 3 years of to make and secure an acrylic palatal splint. How one plastic surgery, preparing one graduate every three years. achieves alveolar reconstruction is irrelevant. One may use Participation by the resident in all activities with visiting cancellous graft (rib in young patients) + platelet enriched teams created a broad exposure. Mini-fellowships were plasma. One may use rhBMP-2. What really matters is to created. Each graduate undergoes final oral examination in follow biologic principles. English with the participation of national and international

Indian J Plast Surg January-June 2007 Vol 40 Issue 1 102 Developmental ﬁeld reassignment in unilateral cleft lip faculty. All three graduates remain in the public sector. 12 years ago I proposed this plan to Carlos and Vivian Dr. Gustavo Herdocia work at UNAN-Leon while Dr. Mario Pellas over rum on a muggy Managua night over several Perez and Dr. Leonel Briceno staff the APROQUEN burn rounds of Flor de Cana Rum. In 2005 the Hospital unit. Over 4000 surgeries and 100,000 outpatients have Metropolitano Vivian Pellas opened its doors. HMVP exists benefited from these efforts without a single dollar from because people cared deeply, refused to give up hope and abroad. worked unceasingly to make it happen. And the stunning fact of the entire Nicaragua experience is that it happened But human resources are not enough. While Nicaplast from within. Information regarding these foundations is continues its valuable work in Leon, APROQUEN has available to all surgeons on the Internet. surged forward as a modern-day model of a medical foundation with a bold strategy: the creation of a financial I write this to you, the reader, to propose that improvements “engine” to finance the clinical work and cost of running in cleft care can always be made, even under difficult a 1st-class burn center in an extremely poor country. In circumstances. Our patients speak for themselves. The 1972 a terrible earthquake destroyed much of the capital primary task is to make it possible for the national medical city, including the main hospital for the country. Extensive teams to achieve the logistical support required to provide outside aid was lost to corruption; the hospital was never the care. And, in this regard, innovative efforts in the rebuilt. 4 temporary structures built 35 years ago continue private sector, backed by lay people with the desire to to provide care to the population. A high quality “center help, to bypass roadblocks and provide better service. of excellence” was nonexistent. As I look backward and contemplate the evolution of Burns are a socioeconomic disease. Nicaragua has developmental field theory and the DFR repair, I realize 1000 new burn cases per month, 10% of which require that it followed a number of steps, some forward and some admission. Given the many needs in the health sector, backward. The driving force behind innovation comes public resources are grossly inadequate for these patients. from restless.com). curiosity and the capacity to demand that Knowing this, APROQUEN partnered with other investors anatomy must make sense. Nature is trying to tell us her to build the most modern (albeit small) medical facility in secrets if we can only learn to listen. As we understand the country, with state-of-the-art=radiology, surgery and how the face is constructed, we will learn the sequence intensive care. The facility would serve the international that leads to cleft formation. This inevitably will direct us community and private patients. It would be the very best toward better techniques and better results. As a cleft- in Central America. Trips to Miami would be avoided. But.medknow affected person I know the reality behind the surgeries the key concept is that that APROQUEN would maintain we perform for our patients. We must push forward to do 51% ownership…and a percentage of profits would better. Hopefully, DFR surgery will lead you to question old thus directed to the burn service and reinvested(www for the assumptions, strive for perfection and (most importantly) foundation. find your own innovations. This PDFa site is hosted available by forMedknow free download Publications from

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