Eye (\988) 2, 207-219

The Management of Congenital Malpositions of Eyelids, Eyes and Orbits

S. MORAX AND T. HURBLl Paris

Summary Congenital malformations of the eye and its adnexa which are multiple and varied can affect the whole eyeball or any part of it, as well as the orbit, eyelids, lacrimal ducts, extra-ocular muscles and conjunctiva. A classification of these malformations is presented together with the general principles of treatment, age of operating and surgical tactics. The authors give some examples of the anatomo-clinical forms, eyelid malpositions such as entropion, ectropion, ptosis, levator eyelid retraction, medial canthus malposition, congenital eyelid colobomas, and congenital orbital abnormalities (Craniofacial stenosis, orbi­ tal plagiocephalies, hypertelorism, anophthalmos, microphthalmos and cryptophthalmos) .

Congenital malformations of the eye and its as echography, CT-scan and NMR, enzymatic adnexa are multiple and varied. They can work-up or genetic studies (Table I). affect the whole eyeball or any part of it, as Surgical treatment when feasible will well as the orbit, eyelids, lacrimal ducts extra­ encounter numerous problems; age will play a ocular muscles and conjunctiva. role, choice of a surgical protocol directly From the anatomical point of view, the fol­ related to the existing complaints, and coop­ lowing can be considered. eration between several surgical teams Position abnormalities (malpositions) of (ophthalmologic, plastic, cranio-maxillo-fac­ one or more elements and formation abnor­ ial and neurosurgical), the ideal being to treat malities (malformations) of the same organs. Some of these abnormalities are limited to Table I The manag ement of cong enital rna/positions one organ and can be subjected to a relatively of eyelid s, eyes and orbits simple and well recognised surgical treat­ Ocular Findings: ment. But often they are more widespread, Eyeball, eyelids, lacrimal passages, orbit, socket, involving eye and adnexa, oculo-facial region motility. or eye, face and other organs, in some well known malformative syndrome. Family History

The clinical work-up must always include a Systemic Manifestations: description of the presenting signs and symp­ Face toms and an investigation of other signs General through a careful ophthalmic and systemic Radiologic investigations: examination. Echography, X-Rays, CT-Scan, NMR As a result of the clinical findings, some investigations will then be carried out: Laboratory investigations. radiologic work-up ranging from simple X-Ray to more sophisticated techniques such Genetic transmission and counselling.

Correspondence to: Fondation Ophtalmo)ogique A. de Rothschild 25, rue Manin 75019. Paris. 208 S. MORAX AND T. HURBLI the maximum amount of abnormalities in a Table II Ocular find ings single setting, hence decreasing the number of surgical interventions that will be needed. EYE Vision, visual fields, intraocular pressure, The mechanism of the congenital anterior segment and sclera, retina and choroid ophthalmologic disorder may be direct, in this optic nerve, size and malformations (anophthalmos, microphthalmos, cryptophth­ case the symptom can either be isolated or almos), malpositions: ocular dystopia, motility: associated with a more complex malformation muscular agenesis, muscular extorsion superior creating syndromes or diseases. On the other oblique fontalisation. hand, because of the intricate relationships between the eye and its adnexa, some dis­ SO CKET Dermoids, dermolipomas, lipomas, der­ matocoeles pterygoides ( = choristomas) orders may, indirectly, be a consequence of a Investigation for 1st brachial arch syn­ pre-existing malformation. For instance, con­ drome and facial cleft. genital microphthalmos may result in micro­ orbitism that will need a clinical and radiologi­ EYELIDS (a) Eyelid margin malpositions: • Congenital entropion/epible- cal work-up. Similarly, a severe cranio­ pharon stenosis will lead to malposition of the eye­ • Congenital ectropion. ball (exophthalmos), this secondary (b) Levator dysfunction (neurogenic, exophthalmos may induce corneal exposure myogenic): • Ptosis and keratitis. As no single symptom of the • Eyelid retraction. ocular area is pathognomonic alone, it is very (c) Medial canthus malpositions (epi­ important to describe precisely each symptom canthus, telecanthus, telorbitism. and link it to its true cause or mechanism. (d) Lateral canthus malpositions: anti­ mongoloid fissures. (e) Colobomas and ablephary. Classification A number of classifications have been pro­ LACRIMAL DUCTS ABNORMALITIES posed according to the type of lesion or tissue Colobomas, stenosis ... involvement (soft tissue only, combined soft CONGENITAL ORBITAL ABNORMALITIES tissue and underlying bone or indirect Micro/Macr()-{)rbitism---craniofacial stenosis, pla­ involvement of soft tissue as a result of bony giocephal-telorbitism. malformation). We prefer a classification which relates to often as a result of congenital bony or orbital the involved organ (Table II). contents abnormalities as encountered in cra­ niostenosis or Von Recklinghausen's disease. A-Eyeball Abnormalities The eye may stay normally formed and func­ These can be of size or of shape. Micr­ tional, the corneal reflexes well centred and ophthalmos or anophthalmos are very precise the motility normal. Strabismus is not found examples. True anophthalmos is rarely in all cases. Treatment must include reposi­ encountered and CT-scan often reveals that in tioning of the bony orbit. fact it is simply a severe microphthalmos with sometimes a colobomatous cyst. These iso­ C-Oculomotor Abnormalities in Orbital lated malformations can also be associated in Malformations more complicated syndromes, e.g. first These may be isolated or associated with branchial arch or facial cleft syndromes. The other abnormalities. Direct lesions of the small size of an unaesthetic and non functional muscles occur: agenesis (as the agenesis of the eyeball will result in lack of development of superior rectus in Apert syndrome), or mal­ the bony orbit, producing a relative micro­ position of the muscle insertion or direction orbitism and lid size reduction ranging from due to orbital malposition (often extorsion). simple microblephary to subtotal ablephary. The most frequent syndrome is macular pseudo-ectropia with extorsion of all four B-Position Abnormalities recti that act like oblique muscles, resulting in Ocular dystopias, are displacement of the eye­ a V-syndrome, a double upshoot and weak­ ball in a vertical, transverse or sagittal plane ness of both superior obliques. This is most THE MANAGEMENT OF CONGENITAL MALPOSITIONS OF EYELIDS, EYES AND ORBITS 209

frequently encountered in hypertelorism and • Congenital ectropion due to cutaneous Treacher-Collins syndrome with anti­ lack is either present alone or belongs to a mongoloid lids. blepharophimosis syndrome. Hypertropia by hyperaction of the inferior oblique and weakness of the superior oblique Levator muscle dysfunction by excess or by can be explained by excessive sagittalisation deficiency of neurogenous origin or of the inferior oblique or frontalisation of the muscular dystrophy. superior oblique, as in cases of plagiocephaly This gives rise to the congenital ptoses and where the frontal bone is less developed and muscular retractions. retruded as compared to the maxillary. The following elements will be examined: Exotropia is frequent in hypertelorism, primary position, muscular function, pres­ being sometimes the result of the divergence ence of syntines and associated signs. of orbital axes and can be reduced simply by repositioning of the orbit, although muscle • Medial canthus malpositions include: surgery may sometimes be needed to correct epicanthus, telecanthus and intercanthal any residual strabismus. space widening by soft tissue lesion. They can either be isolated or integrated in a D-Abnormalities of the Conjunctival blepharophimosis or Waardenburg syn­ Fornices drome. The most frequently encountered lesions are Hypertelorism (Grei syndrome) is an inter­ those belonging to the groups: dermoids, canthal widening by lesion of the bony parts, dermo-lipomas, dermatocoeles and ptery­ its degree is variable and two aspects are goides. All can be classified as choristomas encountered: hypertelorism by simple cranio­ (presence of a normal tissue in an unusual stenosis and hypertelorism by orbito-facial location) (Fig. la and b). cleft. 1.2 These non-evolving lesions must incite the search for a first brachial arch syndrome or • Lateral canthus malpositions are less facial cleft. They can also be associated with frequent. palpebral abnormalities (colobomas). The most encountered aspect is the anti- mongoloid fissure as seen in the E-Palpebral Malpositions and Congenital Franceschetti-Treacher-Collins syndrome Malformations of the lids and in telorbitism with extorsion. Several categories can be described: • Colobomas and ab\ephary. Congenital mal positions of the lid margin: As well as the rest of the face, lids can be • Congenital entropion and epiblepharon subject to clefts. Colobomas are either iso­ due to lack of adherence of the inferior lid lated or associated and belong to the facial retractor of the skin. cleft group (Fig. 2). One must therefore look

Fig. 1. a-Cong enital limbal dermoid . b-Cong enital dermolipoma of the lateral canthus (same patient) . 210 S. MORAX AND T. HURBLI

General Principles of Treatment The severe bony malformations which require cranio-facial surgery have become better classified and more easily treatable since the work of Tessier1.2 in the last 20 years. The interest in the treatment of congenital malfor­ mations of the visual apparatus lies more in the extremely variable clinical work up and the surgical management of the associated malformations rather than in the purely tech­ nical aspect of the surgery. The plastic surgeon will be faced with Fig. 2. Cong enitalsuper ior eyelid coloboma. several problems:

for all associated anomalies of a half-axis or a At what age should one operate? different axis according to Tessier's classifica­ Except for emergency cases as illustrated by tion,2 on skin, skeleton and lacrimal ducts. an upper ablephary causing corneal exposure or optic nerve compression by cranio-stenosis F-Congenital Orbital Abnormalities that may be an indication of an extremely One can describe abnormalities in size: micro­ urgent cranio-orbito facial surgery, we do orbitism related to microphthalmos, macro­ have the choice between an early surgery and orbitism as encountered in mesodermal dys­ a delayed one. Given the facts that surgical plasia with destruction of the greater wing of techniques are becoming more and more the sphenoId bone in von Recklinghausen's sophisticated, and that anaesthesia and disease, and malformative and position resuscitation are making much progress, the abnormalities as dystopias in all three planes: trend is to operate very early to avoid func­ plagiocephaly in the vertical plane, telorbi­ tional sequellae and psychological disorders tism in the transverse plane and cranio-facial for the child and his family. stenosis in the sagittal plane. The indication for such early surgery will of These primary or secondary bony malfor­ course depend on the importance of the mations induce sequellae of the visual appara­ lesion, its evolution, the demand of the family tus: positional sequellae (enophthalmos, and the calculated risks as compared to those exophthalmos); strabismus or visual for a delayed type of surgery. sequellae: optic nerve compression and cor­ Technically, early surgery of soft tissues or neal exposure. bony parts does not seem more difficult than To summarise, all congenital malforma­ surgery in adolescence. In some cases, tions of the visual apparatus described in each delayed surgery (planned toward the end of organ can either be found alone or associated the growing period), will appear more appro­ and integrated into other diseases; the clinical priate. We also believe that it is preferable to presentation being extremely varied. perform orbital enlargement osteotomies on well-grown orbits rather than on young ones. • Isolated forms: only one single symptom calls for treatment, orbital, ocular, pal­ What surgical tactic to use? pebral, lacrimal or muscular. This decision will depend on the clinical and • Associated forms that stay purely investigatory findings. ophthalmic, where one or two symptoms of In isolated forms, the treatment is simple the visual apparatus can be either directly and is directed towards a single symptom, or indirectly related. whereas in multi symptomatic forms, one • Ophthalmic and facial associated forms. must plan several surgical procedures done • More generalised associated forms where during one or more surgical intervention. ophthalmic, facial and general abnor­ They may include orbital osteotomies, pal­ malities can combine. pebral lengthening or correction of a pal- THE MANAGEMENT OF CONGENITAL MALPOSITIONS OF EYELIDS, EYES AND ORBITS 211 pebral coloboma as well as reformation of a auricular full thickness skin grafts inserted conjunctival fornix by a mucosal graft. into the lower lid via a para-marginal incision. As a general rule, bone will be treated first, The orbicularis muscle must be split just followed by soft tissue surgery. The surgical above the lower lateral orbital margin and the treatment must be carefully and precisely orbital septum divided with scissors along the planned as several operative settings may be inferior orbital margin. The upper lids will be often needed. reconstructed after an interval of 6 months by In many cases, surgery will be 'border-line', insertion of adequate split-skin grafts or thin calling for the assistance of several teams: full-thickness grafts, by a para marginal ophthalmic, plastic, cranio-maxillo-facial and incision. neurosurgical specialists will have to cooperate. 3-Ptosis (Fig. 3)

Indications and Methods According to Congenital ptosisS is due to a dystrophy of the Anatomo-c1inical Forms levator muscle. In some cases, there is an A-Eyelids Malpositions: aponeurotic defect and nerve palsy. The I-Entropion management of ptosis depends on the diag­ Entropion is characterised by turning in of the nosis and examination findings. Levator eyelid margin causing irritation of the eye and resection by an anterior approach is the cornea. It must be distinguished from epi­ method of choice. The importance of the blepharon that is an abnormal fold of skin resection depends on the degree of levator occurring in young children that retracts over function. In moderate ptosis with good leva­ the lower lid, pressing the lashes inwards. tor function (more than 10 mm), the The principle of treatment is to excise an Fassanella-Servat procedure is a good one. ellipse of skin and underlying hypertrophied The upper border of the tarsus is excised with orbicularisllmscle Lelow the inferior punc­ the lower part of Muller's muscle and overly­ tum. The skin edges are sutured to the lower ing conjunctiva. When the levator function is border of the tarsus to prevent the orbicularis poor or when the levator muscle cannot be from again overriding the lid margin.3 used, a brow suspension by connecting the frontalis muscle and eyebrow to the eyelid 2-Ectropion with a subcutaneous sling must be performed The congenitdl ectropion syndrome4 is a rare (Fig. 4). congenital deformity, with shortage of skin in alI 4 lids sometimes along with a degree of 4--Levator eyelid retraction6•7 ptosis but in which although there is quite a This congenital deformity is a rare condition marked telecanthus there are no epicanthal with a true levator eyelid retraction some­ folds of any sort. times associated to shortage of skin. The treatment includes extensive post- This upper lid retraction can be treated by

Fig. 3. a-Mod er ate left cong enital ptosis. Mod er ate b-Result after largelevator shor tening. levator function. 212 S. MORAX AND 1. HURBLI

Fig. 4. a-Sever e blephar ophimosis with ptosis, epi­ b-After cor r ection of the epicanthus-telecanthus and canthus, telecanthus. bilater alsu spension with autog enousfascialata .

lengthening of the levator muscle by the fol­ In the latter condition, the telecanthus or lowing technique (Fig. 5). the telorbitism is corrected when the orbital Incision through the skin of the upper lid in contents and the orbit themselves are moved the palpebral fold, opening of the septum, into anormal position. complete section of the levator muscle at the The important points in correction of con­ upper border of the tarsal plate, lengthening genital telecanthus are to arrange for the of the muscle by interposition of Iyophilised accommodation of the excess soft tissue in the sclera or a strap of synthetic material (Gore­ area by means of transposed flaps, to shorten Tex) at the upper border of the tarsus. This the medial canthal tendons, or in severe cases, lengthening must equal twice the excess to attach the medial canthal tendons perma­ retraction of the upper lid. Slight upper lid nently to each other across the nasal cavity by retraction can be treated by Miiller's muscle means of stainless steel wire. resection. The design of the flapsis established by first marking a point where the medial canthus 5-Medial canthus malpositionss.9 ought to lie and then, after drawing the nasal Congenital telecanthus may be encountered skin medially so as to obliterate any fold pres­ either as a primary condition in which the ent, marking the site of the actual canthus. interpupillary distance itself remains within These 2 salient points are joined and from normal limits, or it may be a secondary con­ the centre a line is drawn, slightly shorter than dition associated with an increase of the inter­ and at 60° to the original line. pupillary distance, indicating in case of Backcuts of the same length are drawn at absence of strabismus an increased distance 45°, and the design of the four quadrilateral between the eyeballs and the underlying bony flapsis completed by marking in paramarginal orbits. lines again of the same length as the verticals.

Fig. 5. a-Left cong enital levator eyelid retr action. b-Result after levator recess ion and scler al graft. THE MANAGEMENT OF CONGENITAL MALPOSITIONS OF EYELIDS, EYES AND ORBITS 213

The flaps are incised and raised along with plasty by four quadrilateral flaps or a V-Y some underlying orbicularis muscle, and the plasty, a reduction of the thickness of the elongated superficial component of the anterior lacrimal crest and medial orbital medial canthal tendon is rejected, and a 4--0 bony wall, and a medial canthopexy by a nonabsorbable suture is inserted through the transnasal wire fixation.8.9 stump of the tendon close to the canthus and through the periosteum underlying the medial &-Congenital eyelid colobomaslO stump. When this suture is tied, it draws the Congenital colobomas are clefts of varying medial canthus nasally, and the flapsare then configurationfound in the visual system. They easily transposed. Since they are quad­ can be found in all areas of the lids. More than rilateral, the flapsmay need to be trimmed to one lid may be involved in the same patient, or fit the triangular defects, and they are then there may be multiple coloborn as in the same sutured into place using 6-0 chromic catgut lid. They most often occur as isolated anoma­ (which avoids the need to remove the sutures lies, but are sometimes seen in patients with 4 or 5 days later). The scars of the transposed other congenital lesions, and ocular lesions, flaps are surprisingly obvious for several the most common being dermo"ids and der­ months but eventually become practically molipomas, facial lesions as other facial clefts. invisible. The aetiology of eyelid colobomas is not When telecanthus is present alone without clear. The predominant aetiologic theory any accompanying folds, as in Waardenburg's incriminates the mechanical action of amni­ syndrome, exactly the same formula is used, otic bands. Heredity does not seem to play a but no obliteration of folds has to be carried significant role in most colo bornas except in out before designing the flaps. Because of the lower-lid colobomas associated with man­ this, the whole of the flapdesign lies exposed, dibulo-facial dysostosis (Francheschetti­ but the technique is carried out exactly as Treacher-Collins syndrome). before (Fig. 6). Recently, the colobomas of the eyelids have A much more severe type of telecanthus is been classified with the new anatomic classi­ encountered in blepharophimosis where there fication of facial clefts. 2 may be up to 20 mm of increase of the inter­ Treatment of congenital colobomas will canthal width. In blepharophimosis, along depend on the extent of the defect as well as its with the telecanthus there are small inverted position.to.1I It is possible primarily to close epicanthal folds, a severe ptosis which is most defects of up to one-third of the lid. The difficult to correct by levator surgery alone, rounded edges of the coloboma are squared and an absence of laxity of the skin of the lids by a razor knife, creating fresh new surfaces to in a vertical direction, which may amount to help ensure the most precise closure. After actual ectropion in the outer half of the lower the edges of tarsus are brought together by lid. In these severe cases with telecanthus and placing 6-0 chromic sutures in the pretarsal epicanthal folds, the treatment includes a skin fascia, attention is focussed on the lid margin

Fig. 6. a-Sever e Waar d enbur g's synd rome with b-Result after med ial canthopexy by a tr ans nasal wir e telecanthus . fixation. 214 S. MORAX AND T. HURBLI which is aligned with three interrupted (}-() In some cases after excision of the lesion, a silk sutures. The remainder of the skin is then reconstruction of the defect must be per­ closed with interrupted 6-0 silk sutures. formed by conjunctival flaps or mucosal In some patients with slightly larger defects, grafts. the horizontal tension may still be too great so that a cantholysis must be performed. By cut­ B-Congenital Orbital Abnormalities16.l7 ting either the upper or lower wing of the Congenital orbital abnormalities can be classi­ lateral canthal tendon, depending on which fied as congenital anophthalmos and lid is being repaired, the surgeon will have microphthalmos as orbital dystopias or pla­ several more millimeters of tissue with which giocephalies due to a cranio facial cleft, or as to work. hemicranial stenoses, cranio facial stenosis as Colobomas involving much more than one­ the Crouzon and Apert syndromes, or hyper­ third of the lid will require a more extensive telorism and finallyas craniofacial and lateral lid reconstruction procedure. Upper-lid clefts. Cranio facial stenosis affect the sagittal defects must be repaired by using a composite diameter in Crouzon and Apert syndromes, graft from either the other upper lid or the the vertical diameter is abnormal in orbital opposing lower lid,12 a Mustarde rotation dystopia, and the transverse diameter is f1ap,13 or a Cutler-Beard bridge flap from the affected in hypertelorism. The general prin­ opposing lower lid.14 Extensive defects of the ciple of treatment is to displace the functional lower lid are best required by a large cheek part of the orbit, that is, that part of the orbit rotation f1apl3 or an orbito-naso-genial f1aplS that carries the globe. This part consists of the with a chondro-mucosal graft for the posterior orbital walls situated behind the equator of lamella, or a tarso-conjunctival flap from the the eyes. opposing upper lid doubled by a skin graft for the anterior lamella.ll I-Craniofacial stenosis18 (Fig. 7) Frequently congenital eyelid colobomas are The Crouzon and Apert syndromes are associated with adnexal dermoIds.16 DermoId characterised by stenosis of all the sutures of cysts are common congenital anomalies found the upper part of the skull and consequently in the brow, the eyelid or the orbit. Solid intracranial hypertension, papillary oedema dermoId tumors on the limbus and der­ and sometimes blindness. molipomas in the fornices are much less com­ The facial characteristics are the reduced mon than dermoId ·cysts. Complete operative size of the facial structures, exophthalmos and removal is the treatment of choice in all exotropia. These may be combined with dermoId tumors; however there are times nasopharyngeal atresia and deformity of the when the needs of the patient are best served maxilla. The aims of the operation are to give by a subtotal excision. the central portion of the face a normal pro­ A partial resection is recommended rather file, to re-establish dental occlusion, and to than endangering vital orbital structures. increase the depth of the orbit.

Fig. 7. a-Cr aniofacial stenoses, Cr ouzon's disease b-Result after Tessier -Lefor tIII os teotomy (s ur geon with exophthalmos and str abismus. Dr P Tessier ) and str abismussur gery . THE MANAGEMENT OF CONGENITAL MALPOSITIONS OF EYELIDS, EYES AND ORBITS 215

The method used is a cranio-facial dysjunc­ occlusion, but in the child, sagittal over-cor­ tion which is both interfronto-facial and rection of 6-8 mm is necessary in order to interpterygomaxillary, and to re-establish the provide space for future dental eruptions and vertical dimensions of the face. If possible, orthodontics. The intermaxillary fixation is respiratory pathways have to be restored. used only as a means of rectifying the final articulation. The central position of the face Procedure: remains without support in its new position The total osteotomy of the central portion of without the aid of intermaxillary fixation. the face is a complex operation consisting of several stages. We describe the mid-facial 2-Congenital orbital dystopias or orbital advancement by the Tessier-Lefort III type pi agio cephalies: . . osteotomy. When there is orbital dystopia, which. IS A coronal incision has many advantages in always the consequence of other malforma­ facilitating the osteotomies along the front­ tions, one must look for the causal malforma­ omalar region. One follows the lateral surface tion and its repercussions. of the orbit into the retromalar space and The operation consists of the monobloc performs a temporo-zygomatic dysjunction. raising of the orbit by a frontal and malar Then on the frontal bone, one can make either osteotomy, followed by repositioning in the a deep vertical frontal spur or a transverse proper position. In addition, hypoplastic and frontal crescent, behind which bone grafts can displaced parts should be remodelled by bone be placed to prevent relapse. grafts to smooth the exterior aspect. The Along the fronto-naso-orbital region, one monobloc raising of the orbit should prefer­ firstcarries out a vertical section of the medial ably be done by elevating it in its totalit�, i? walls down to the floor, 10 mm behind the one or two fragments. Again the coronal mCI­ posterior lacrimal crests. Then, centrally, an sion is used. The cranial base is exposed from infra-basilar osteotomy is made parallel to the the crista galli back to the posterior border of stage of the anterior cranial base. Sub­ the lesser wing. Oblique or step osteotomies sequently, an inter-pterygo-maxillary dys­ are traced on the frontal and on the malar junction is performed by an oral approach bone to ensure that the orbit is mobilised as a with a curved 8 mm osteotoma. monobloc. Dysjunction is performed with the Rowe The inferior facial osteotomy is done at two forceps or with right-angles grapnels, intro­ different levels. Laterally, it is oblique and duced behind the maxilla. The disengagement located relatively high on the malar bone. should be carried out to an overcorrection of Medially it is transverse and directed towards at least 10 mm. Consequently, seven gaps the inferior meatus. occur, a central fronto-nasal gap, two lateral During the repositioning one has to keep in gaps and two retromalar gaps, which should mind that transverse displacement corrects all be closed with bone grafts. hypertelorism and telecanthus. Anterio dis­ These grafts must support the repositioning � placement may result in exophthalmos If the of the face and, in particular must prevent any defects are not sufficiently occluded by bone backward relapse into the original position. In ' grafts. By relation of the bloc, the lateral addition, the grafts have to close the fissuresin quadrants can be elevated and the malar bone the orbital walls and are used to correct the may be advanced. The position of the orbital profile of the glabella, of the supra-orbital rim can be adjusted. If a unilateral reposition­ ridge, of the zygomatic arch and of the infra­ ing is done, the contralateral orbit should be orbital rim. used as a reference point. The osteosynthesis finally has to provide the permanent fixation of the reconstructed region. As regards the dental occlusion, there 3-Hypertelorism (Figs. 8-9): is a clearcut difference in procedure between Hypertelorism is a congenital malformation adults and children. characterised by an abnormally exaggerated In the adult one establishes only normal distance between the two orbits and, there- 216 s. MORAX AND T. HURBLl

Fig. 8. a-Hyper telor smi with a cr aniostenosis in the b-Result after displacement of the or bit tr ansversely inter or bitalspace . (Sur geon Dr P. Tessier ). fore, between the two ocular globes, with a on the fronto maxillary processes and on the cranio facial cleft or a craniostenosis in the ethmoId bone. The anterior resection is com­ interorbital space. When hypertelorism is pleted by resection of the lateral masses of the secondary to a craniostenosis, there is always ethmoId and of the structures within the nose. a brachycephaly and an elevation of the level The septum, vomer, inferior and medial turbi­ of pterion and generally a bifidnose. nates and the edges of the piriform aperture The abnormal appearance is much less the are severed. With medial canthopexies, the consequence of hypertelorism than of the orbits are brought to the midline and the malformations which cause the hypertelor­ aponeurosis of the temporal muscle is ism. To correct these malformations of the advanced to the lateral pillar of the orbit and interorbital space, one has to restore the inter­ to the temporal crest. Finally the nasal bridge orbital distance to their normal dimensions. and possible defects have to be grafted with The usual procedure by the intracranial route bone. If hypertelorism and orbital dystopia consists of the displacement of the orbit trans­ have to be treated, the cranial approach versely by a total osteotomy of the frame work makes it possible to correct the transverse as of the walls. A central resection is carried out well as the vertical displacement of the orbit. on the frontal bone on the bones of the nose, If it concerns orbital dystopia in the Crouzon or the Apert syndromes the craniofacial sten­ osis are often asymmetric, although in a cer­ tain way they are plagiocephalies. Although these facial retrusions are generally approached by an extracranial route, con­ siderable orbital dystopia can be an indication

b-Result after two major oper ations (Sur geon Dr P. Fig. 9. a-Hyer teloris m with craniofacial cleft. Tessier ). THE MANAGEMENT OF CONGENITAL MALPOSITIONS OF EYELIDS, EYES AND ORBITS 217 for simultaneous elevation of the orbit by the stabilised. We have, in fact, observed that intracranial route. If hypertelorism and cra­ medial orbital translocation in teleorbitism niofacial stenosis go together, the cranial and sagittal orbital translocation in craniofa­ route should be combined with ethmoidal cial stenosis induce a reduction of the orbital resection. divergence and so a reduction of the Craniotomy must be done by the neu­ exotropia. Orbital surgery can also induce an rosurgeon ·as soon as intracranial hyper­ oculomotor disturbance that did not exist tension is diagnosed. But in cases of facial before.2o.21 deformity the primary craniotomy ought to be This recommendation to wait for arranged according to what will be done in the strabismus surgery can be modifieddepending future, either on the orbitofrontal region or on the age of the patient when examined and on the facial abnormalities. The ages for the when the orbital translocation was first surgical stage should preferably be performed. between three and fiveyears. If the malformation is discovered very early Our experience in the surgery of craniofa­ and surgery takes place in the first years cial stenosis has demonstrated that the first (between 3 and 5 years for some authors), the operation always has the best chance. Second­ strabismus will be treated and operated on ary revisions in the orbitocranial area are later. If the malformation is to be treated much more difficult and hazardous than the later, after the eruption of permanent denti­ primary operation. Consequently, the plan­ tion or for any other reason, the strabismus ning for the primary intervention should aim surgery should be performed first for func­ at a complete redesigning of the facial tional reasons. architecture. We think that early correction of congenital In operations for hypertelorism, secondary deviation is more likely to result in attainment operations are unavoidable. These involve of binocularity. However, we know that after restoration of palpebral occlusion and the craniofacial reconstruction decompensation closure of maxillo palatine clefts, and rarely of strabismus is possible, especially when bin­ involve reinforcement of the dura. The three ocular vision is absent and revision of operations are satisfactory in hypertelorism strab�smus surg�ry is frequent. secondary to cranial or facial clefts. In cra­ If the Inalforniatiollis seen and operated on niofacial stenosis and orbital dystopias, aes­ in adults strabismus surgery will be done after thetic as well as functional objectives should orbital surgery because treatment is under­ be pursued to correct the nose, chin, lateral taken for only cosmetic reasons. canthus, dental occlusion, and motility dis­ orders such as ptosis and strabismus. 5-Anophthahnos and severe congenital We advocate that no corrective strabismus microphthalmos22 (Fig. 10) surgery is undertaken until at least six months In patients with congenital anophthalmos and after the major orbital translocation pro­ severe microphthalmos, we are faced with a cedures, by which time the strabismus is tiny orbit and socket and with little eyelids,

- Fig. 10. a-Sever e cong enital micr ophthalmos. b-Result after or bital os teotomy, socket expansion, infer ior eyelid reconstr uction, pr os thesis. 218 S. MORAX AND T. HURBLI frequently preventing retention of a standard conformer or prosthesis. Socket expansion is sometimes impossible with micro-orbitism, the retention of a prosthesis is also difficult when malformation of the eyelids exists. The treatment in these difficult cases includes, in the first stage, an orbital expansion which depends on the cephalometric studies of the patient: transverse osteotomy of the maxilla and zygomatic bone with lateral bar via an extra-cranial approach, vertical osteotomy of the roof of the orbit via an intracranial b-Result after socket reconstruction, eyelid recon­ approach. In some cases, the osteotomy will struction, prosthesis on the eyeball. include expansion in the transverse and verti­ cal diameter with bone grafts in the defects References and on the lateral and superior rims. At the 1 Tessier P, Guiot G, Rougerie J, Delbet P, Pasteriza same time, socket expansion will be per­ P: Osteotomies cranio-naso-orbito-faciales. Ann Chir plast 1967,12: 86. formed by incision of the conjunctival sac cir­ Hypertelorisme. 2 Tessier P: Anatomical classification of facial, cra­ cumferentially, with mucosal or split skin niofacial, and laterofacial clefts in Sy mposium on grafts on a conformer. The second stage plastic surgery in the orbital region. St Louis includes eyelid reconstruction by different 1976, The C. V. Mosby Co, 189-98. flaps. A third stage is frequently needed for 3 Collin JR: Congenital entropion in 'Manual of systematic eyelid surgery'. Churchill correction of eyelid malpositions on the pros­ Livingstone, 1983, 16. thesis: ptosis, entropion surgery. 4 Mustarde JC: Congenital ectropion syndrome. In 'Repair and Reconstruction in the orbital region'. Churchill Livingstone, 1980,351-5. 6-Cryptophthalmos23 (Fig. 11) 5 Morax S and Ruban JM: Les ptosis. J Fr ophthalmol. The cryptophthalmos syndrome is a very rare (In Press). 6 Morax S and Hurbli T: Choice of surgical treatment congenital defect with incomplete or com­ for Graves' disease. J crania-max Fac Surg 1987, plete failure in the development of one or 15: 174-80. both eyelids, skin covering the anterior seg­ 7 Morax S: Traitement des retractions palpebrales par ment. The eyeball is anatomically present but allongement du muscule releveur. Ann Chir Plast not functional. The orbital cavity is well devel­ Esthet 1983, 28: 361-8. 8 Mustarde JC: Epicanthal folds and the problem of oped. The surgical treatment includes a telecanthus. Trans Ophthalmol Soc UK 1963,83: socket reconstruction with eyeball con­ 397-411. servation if possible. At the same time, or 9 Mustarde JC: Telecanthus in 'Third international later, the eyelids are reconstructed by dif­ symposium of plastic and reconstructive surgery of the eye and adnexia'. Williams and Wilkins, ferent flaps. Baltimore 1982, 199-205. to Wilkins RB and Carroll RP: Adnexal dermoids and congenital eyelid colobomas in 'Symposium on plastic surgery in the orbital region'. St Louis 1976,The C. V. Mosby Co, 187-8. II Morax S and Benia L: Traumatismes et chirurgie plastique des paupieres. Encycl Med Chir (Paris France). Ophthalmologie, 21100 D 10, 11-1985, 18. 12 Hubner H: Kolobomverschluss Mittels fre er Tarsus lidrandiiberf Lanzung. Klin Monatsbl, Augenheilk 1976, 168: 677-82. I 3 Mustarde JC: Repair and reconstruction in the orbi­ tal region. Churchill Livingstone, Edinburgh, 1980. 14 Cutler ML and Beard C: A method for partial and .....11. a-Partial crvotophthalmos. total upperlid reconstruction. Am J ophthalmol 1955, 39: 1. THE MANAGEMENT OF CONGENITAL MALPOSITIONS OF EYELIDS, EYES AND ORBITS 219

20 15 Tessier R: B1epharopoieses inferieures. Bull Mem Morax S:Oculomotor disorders in cranio-facial mal­ Soc Fr Ophtalmol1960, 73, 213-54 . formations. In 'Cranio-facial surgery' Boston 16 Rougier J, Tessier R, Hervouet F, Woillez M, 1985 . Little, Brown and Company edited by Car­ Lekieffre M, Derome P: Colobome's pal­ onni P. , 97-107 . pebraux, dermoldes epibulbaires et du fornix. 21 Morax S: Changing of the eye position after cranio­ Ectropion congenital in 'Chirurgie plastique facial surgery in 'Cranio-facial surgery' Boston orbito palpebrale. Rapport Soc Fr Oph Paris 1985 . Little, Brown and Company edited by Car­ 1977, Masson ed, 209-22. onni P. . 2�10. 17 Morax S: Correction of congenital orbital abnor­ 22 malities. Orbit 1983, 2: 121-8. Morax S:Reconstruction orbito-palpebrale au cours 18 Tessier P: The definitiveplastic surgical treatment of des anophtalmies et microphtalmies severes con­ the severe facial deformities of cranio-facial dys­ genitales. AnnChir Plast Esther 1987,32: 255-61. 2 ostosis. Crouzon's and Apert's syndromes. Plast 3 Morax S and Herdan ML: Reconstruction orb ito reconstr Surg 1971, 48: 419 . palpebrale au cours de deux cas de cryp­ 19 Tessier P: Orbital hypertelorism. Scand J plast rec­ tophtalmies partielles. Ann Chir Plast Esthet onstr Surg 1972, 6: 135 . 1987, 32: 319-26 .