Infant Head Shape Evaluation Savannah Brown MPAS, PA-C Elizabeth Wetz MS, MPAS, PA-C April 6, 2019 Dallas, Texas Privileged and Confidential Objectives • Understand the common physical findings differentiating craniosynostosis and deformational plagiocephaly • Recognize patterns of single suture, non-syndromic craniosynostosis • Discuss treatment options for both craniosynostosis and deformational plagiocephaly Dallas, Texas Privileged and Confidential 2 Skull Growth • The skull grows in response to increasing brain and CSF volume • The brain size triples by 1 year and approximates 85% adult growth by 3 years of age • Normal growth occurs perpendicular and parallel to sutures. Dallas, Texas Privileged and Confidential 3 Skull Growth Dallas, Texas Privileged and Confidential 4 Parietal Bone Anterior Fontanelle Frontal (Metopic) Suture Coronal Suture Frontal Frontal Bone Bone Coronal Suture Dallas, Texas Privileged and Confidential 5 Anterior Fontanelle Coronal Suture Frontal Parietal Bone Bone Lambdoid Squamosal Suture Suture Dallas, Texas Privileged and Confidential 6 Posterior Coronal Fontanelle Suture Anterior Parietal Bone Fontanelle Frontal Bone Lambdoid Frontal Sutures (Metopic) Suture Frontal Bone Parietal Bone Coronal Suture Sagittal Suture Dallas, Texas Privileged and Confidential 7 Sagittal Suture Parietal Parietal Bone Bone Posterior Lambdoid Fontanelle Sutures Occipital Bone Dallas, Texas Privileged and Confidential 8 Skull Maturation • Normal suture fusion – Metopic: 6 to 8 months – Sagittal: 22 years – Coronal: 24 years – Lambdoidal: 26 years • Normal fontanelle closure – Posterior fontanelle: 3-6 months – Anterior fontanelle: 9-12 months Dallas, Texas Privileged and Confidential 9 Abnormal Head Shape Evaluation Dallas, Texas Privileged and Confidential 10 Infant Head Shape Changes • Macrocephaly à Neurosurgery • Microcephaly à Neurology • Scaphocephaly/Brachycephaly/Plagiocephaly à Plastic Surgery Dallas, Texas Privileged and Confidential 11 Plagiocephaly Plagio = “twisted” or “oblique” + Cephaly = head • Is a descriptor of head shape, not necessarily a diagnosis Dallas, Texas Privileged and Confidential 12 Head shapes • Scaphocephaly: “boat” – sagittal synostosis versus long term hospitalization positional changes • Anterior plagiocephaly – unicoronal synostosis versus positional plagiocephaly • Posterior plagiocephaly – lambdoid synostosis versus positional plagiocephaly • Trigonocephaly: “triangle” – metopic synostosis versus benign metopic ridge • Brachycephaly: “short” – bicoronal synostosis versus positional brachycephaly Dallas, Texas Privileged and Confidential 13 Basic Exam (NOT anthropometry) • Head size • Overall shape • Cephalic index • Suture ridging or mobility • Areas of flattening and prominence • Ear placement • Forehead shape and symmetry • Intercanthal distance • Orbit symmetry Dallas, Texas Privileged and Confidential 14 Craniosynostosis Dallas, Texas Privileged and Confidential 15 Craniosynostosis Definition • Premature fusion of one or more cranial sutures • Incidence is 1:2500 live births Dallas, Texas Privileged and Confidential 16 Craniosynostosis: Virchow • Inhibition of growth perpendicular to stenosed suture • Compensatory growth parallel to closed suture • Essentially, compensatory growth occurs at all patent sutures, especially the adjacent ones Dallas, Texas Privileged and Confidential 17 NORMAL CRANIOSYNOSTOSIS X Dallas, Texas Privileged and Confidential 18 Dallas, Texas Privileged and Confidential 19 Is this purely an aesthetic problem? Elevated Intracranial Pressure • Elevated intracranial pressure (ICP) • 13% incidence of increased ICP with isolated synostosis • 42% incidence of increased ICP with multiple suture synostosis Complications of Elevated Intracranial Pressure • Blindness: optic nerve atrophy, corneal exposure • Developmental delay • Attention issues • Learning and languaGe deficits • Decreased IQ in non-operated patients when compared to patients who have surGery before 1 year of aGe. Dallas, Texas Privileged and Confidential 20 Intracranial Pressure Symptoms • Headache • Nausea/vomiting • Visual problems • Motor, behavioral, and intellectual problems • Vault too small for growing brain • CSF production > egress Dallas, Texas Privileged and Confidential 21 Sagittal Synostosis • Incidence: 40-50% of craniosynostosis • Morphology: Scaphocephaly (“boat shaped”) • Ridging in the line of sagittal suture • Increased AP diameter and decreased biparietal width • Bitemporal to biparietal ratio is abnormal • Frontal bossing and occipital “coning” • Male/female ratio: 4:1 Dallas, Texas Privileged and Confidential 22 SAGITTAL NORMAL CRANIOSYNOSTOSIS Dallas, Texas Privileged and Confidential 23 NORMAL SAGITTAL CRANIOSYNOSTOSIS Dallas, Texas Privileged and Confidential 24 Dallas, Texas Privileged and Confidential 25 Treatment: Extended Strip Craniectomy Dallas, Texas Privileged and Confidential 26 BEFORE SURGERY 3 WEEKS AFTER SURGERY Dallas, Texas Privileged and Confidential 27 FRONT BEFORE SURGERY 3 WEEKS AFTER SURGERY Dallas, Texas PrivilegedBACK and Confidential 28 BEFORE SURGERY 4 MONTHS AFTER SURGERY Dallas, Texas Privileged and Confidential 29 Treatment: Open Vault Remodeling Dallas, Texas Privileged and Confidential 30 Treatment: Open Vault Remodeling Dallas, Texas Metopic Synostosis • 23-28% of craniosynostosis • Trigonocephaly • Most obvious with midline vertical forehead ridge and keel shaped forehead • A spectrum of severity Dallas, Texas Privileged and Confidential 32 NORMAL METOPIC CRANIOSYNOSTOSIS FRONT BACK Dallas, Texas Privileged and Confidential 33 Metopic Spectrum Benign metopic ridge versus metopic craniosynostosis Dallas, Texas Privileged and Confidential 34 Metopic Synostosis • Bitemporal narrowing • Hypotelorism • Epicanthal folds • Omega sign (CT) • Anterior cranial base increased in the AP dimension Dallas, Texas Privileged and Confidential 35 Treatment: Strip Craniectomy versus Open Cranial Vault Remodeling Dallas, Texas Privileged and Confidential 36 Dallas, Texas Privileged and Confidential 37 Dallas, Texas Privileged and Confidential 38 Unilateral Coronal Synostosis • Incidence: 20% of craniosynostosis • Morphology: Anterior plagiocephaly (Twisted Skull) • Ipsilateral frontal flattening and contralateral frontoparietal bossing • Ridging along the closed coronal suture (flat side of forehead) • Ipsilateral occipitoparietal flattening and contralateral occipitomastoid bossing (Trapezoid) • Ipsilateral temporal fossa bulge/convexity Dallas, Texas Privileged and Confidential 39 Unilateral Coronal Synostosis • Ipsilateral ear and zygoma anteriorly displaced • Anteriorly displaced ipsilateral glenoid fossa causes the chin to point to contralateral side • Recessed supraorbital and lateral rim; shallow orbit • Recessed zygoma and inferior rim • Root of nose constricted and deviated to ipsilateral side Dallas, Texas Privileged and Confidential 40 NORMAL LEFT CORONAL CRANIOSYNOSTOSIS Dallas, Texas Privileged and Confidential 41 NORMAL LEFT CORONAL CRANIOSYNOSTOSIS Dallas, Texas Privileged and Confidential 42 Dallas, Texas Privileged and Confidential 43 Dallas, Texas Privileged and Confidential 44 Treatment: Open Cranial Vault Remodeling Dallas, Texas Privileged and Confidential 45 Treatment: Open Cranial Vault Remodeling Dallas, Texas Privileged and Confidential 46 Bilateral Coronal Synostosis • Incidence: 10% of craniosynostosis • Morphology: Brachycephaly • Recession of supraorbital ridges with tall and broad forehead • Shallow orbits and hypertelorism • Increased vertical and transverse dimension of skull, but short in AP direction Dallas, Texas Privileged and Confidential 47 Dallas, Texas Privileged and Confidential 48 Dallas, Texas Privileged and Confidential 49 Syndromic Bicoronal Synostosis Dallas, Texas Privileged and Confidential 50 Treatment: Cranial Vault Distraction • 4-6months or older • Less blood loss • Shorter anesthesia time (3-4 hours) • Longer treatment time (2-3 months) • Same Hospitalization (4-7 days) • Same Scaring (Ear-to-ear Coronal sCar) • Much greater intracranial volume gains • Delay FOA to an older age Dallas, Texas Privileged and Confidential 51 Dallas, Texas Privileged and Confidential 52 Treatment: Posterior Vault Distraction + Fronto-orbital Advancement Dallas, Texas Privileged and Confidential 53 Lambdoid Synostosis • Incidence: Less than 3% of craniosynostosis • Morphology: Posterior plagiocephaly or occipital plagiocephaly • Ipsilateral occipital flattening and mastoid air cell prominence, downward cant of the posterior skull base to the affected side • Larger petrous ridge angle and middle cranial fossa on the unaffected side Dallas, Texas Privileged and Confidential 54 RIGHT LAMBDOID NORMAL CRANIOSYNOSTOSIS Dallas, Texas Privileged and Confidential 55 Lambdoid Synostosis Dallas, Texas Privileged and Confidential 56 Lambdoid Synostosis Dallas, Texas Privileged and Confidential 57 Positional plagiocephaly Dallas, Texas Privileged and Confidential 58 Positional Plagiocephaly Deformational Plagiocephaly • Incidence: 1:300 in the general population (Probably more now) • No functional sequelae- normal development, no risk of increased intracranial pressure due to head shape Types & Occasional Associations • Benign metopic ridge • Scaphocephaly: NICU stay, breech presentation • Plagiocephaly: Torticollis • Brachycephaly: low tone Dallas, Texas Privileged and Confidential 59 Deformational Scaphocephaly Dallas, Texas Privileged and Confidential 60 Positional Plagiocephaly • Head assumes parallelogram configuration • Ipsilateral