Funny Shaped Heads Ronald L. Young II, MD

Funny shaped heads Etiologies Congenital deformities Positional molding Cephalohematomas Mass lesions Congenital: craniosynostosis, intrauterine molding, encephalocele, Mass lesions: tumors, arachnoid cysts

Molded Heads Plagiocephaly Brachycephaly Scaphocephaly The most prevalent head shape of the American infant has gone from normocephalic to brachycephalic in one generation because of the back to sleep initiative.

Positional Plagiocephaly Occipital flattening Anterior displacement ear Bulge forehead Bulge malar eminence Parallelogram Local bald spot

Positional Plagiocephaly Associated with “torticollis” Short sternocleidomastoid on flat side Not stretched out Decreased rotation away from flat side Uterine constraint

Positional brachycephaly Symmetric flat occiput Increased bi-parietal diameter No ridging Focal bald spot Severe cases have ears pointing down

Positional brachycephaly Deep sleepers Don’t move after falling asleep Don’t sleep in bed Infant seat Bouncy seat Hypotonia Cause not effect

Positional Scaphocephaly “Premie head” Common in severe premies Can evolve into craniostenosis VP Shunt

Pathology Soft skull Weight of brain able to flatten skull Rapidly growing skull Self sustaining Flat tire Flat tire: the head tends to end up on the flat spot.

Time Course Flat spot noticeable 1 m/o Deformity peaks 4 m/o (Corrected) Head control Primary deformity Flat spot Secondary deformity Compensatory bulge

Time Course Improvement of the flat spot 6 m/o Dent in inion 6 m/o Posterior bulge Dent in inion 6 m/o Bulge takes 12-16 months 2 y/o Brain molds from inside

Time Course Ear asymmetry 3 y/o Rare residual deformity Not cosmetic problem Rare residual deformity

Why So Long? Head Growth

Treatment Position Therapy Keep off the flat spot Sleep <4 m/o Wedges and blankets Roll on side >4 m/o Fetal position Let baby fall asleep in usual position Then rotate onto side in fetal position Start at naps

Treatment Position Therapy Sit up in swing Get out of bouncy seat and infant seat Exer-saucer Walker “Tummy Time”

Treatment Molding Helmet Act like dental braces Slowly push in secondary deformity Keep off the primary deformity

Treatment Molding Helmet Patients with helmets look better faster Need to be worn to work Shoot for 23 hours / day Need to be custom fit Earlier the better Won’t work as well after 12 mo

Treatment Molding Helmet Cost Cranialtech $3000 Advanced Orthopro $1400 Complications Compliance Skin breakdown

Treatment Molding Helmet My indications Borderline parents Can’t get concept of keeping off flat spot Hypotonic children Won’t have head control by 4 months Hydrocephalus post shunt Will not have brain growth to mold skull Significant facial bulge Just not getting better Typically make this decision around 7-8 months of age. Babies that cant’t be repositioned, severe deformities.

Craniosynostosis

What is it? Premature fusion of the cranial sutures Sutures allow progressive enlargement of the skull with brain growth Normal sutural fusion is complete at 6 to 8 years of age Premature fusion produces progressive skull deformity

Epidemiology Occurs in 1 in 2100 children Primary non-syndromic is most common Lambdoid synostosis is very rare Multiple sutures involved in 8% of non-syndromic cases Calvarial bones grow perpendicular to the sutures. Growth: 50% at 2 months, 100% at 6 months, and 200% at 10 months.

Calvarial Development Intramembranous ossification Margins of calvarial bones form osteogenic front Sutures form at sites of near contact New bone is laid down by osteoblasts in spicules at the sutural margins Overall bone growth is driven by the expanding brain Calvarial vault develops in a fibrous membrane over the brain called the ectomeninx. Bone then divides ectomeninx into outer periosteum and inner dura. Osteogenic front: specialized proliferative cells Sutures form at areas of major dural reflections. Interaction between dura and osteogenic fronts maintains sutures Brain growth is exponential during the first year.

Calvarial Development

Known causes of craniosynostosis Pathobiology Known causes of craniosynostosis Teratogens Valproic acid, aminopterin, retinoic acid Malformation Microcephaly Encephalocele shunted hydrocephalus holoprosencephaly Metabolic disorders Rickets Hyerthyroidism Mucopolysaccharidoses Hurler’s, Morquio’s Hematologic disorders Thalassemias, sickle cell anemia, polycythemia vera Hyperthyroidism: premature osseous fusion, shunted hydrocephalus: true bony fusion does not occur 2-6% of isolated sagittal and 8-14% of cases of coronal synostosis are familial. Likely autosomal dominant with 0.6% penetrance by segregation analysis/

Pathobiology Syndromic Crouzon Apert’s Pfeiffer Jackson-Weiss Mutation in gene coding for fibroblast growth factor receptors

Pathobiology <10% inherited or syndromic 90% spontaneous Uterine constraint Early drop into cervical canal Multiple births Severe back pain last month of pregnancy

Pathobiology Premature closure of calvarial sutures also affects the skull base Coronal and metopic Effect extends to nasoethmoid complex, orbital roofs, and supraorbital ridges

Diagnosis Characteristic skull shape Lack of movement at suture Palpable ridging of fused suture CT scan Fusion, sclerosis, skull base deformities Deformational skull anomalies are much more common

Metopic 1 frontal keel 2 bitemporal narrowing 3 coronal suture displaced anteriorly 4 parieto-occipital bulging 5 bizygomatic narrowing 6 posterior displacement of supraorbital rim

Metopic

Unicoronal 1 fused suture 2 ipsilateral frontal flattening 3 bulging of temporal squama 4 contralateral frontal bulging 5 ipsilateral nasal root deviation 6 anterior displacement of ipsilateral ear 7 elevation of sphenoid wing 8 shortening of ipsilateral anterior cranial fossa 10 reduced mediolateral dimension of ipsilateral orbit

Bicoronal 1 fused suture 2 recessed supraorbital rim 3 prominent, elongated frontal bone 4 flattening of the occiput 5 bitemporal protrusion 6 anteriorly displace vertex 7 harlequin deformities

Sagittal

Lamdoid

Cloverleaf deformity

The case for surgery Aesthetic and functional Elevated ICP in 14% of single and 47% of children with multiple sutural synostosis Uncorrected children often socially isolated and stigmatized Low risk with modern craniofacial surgery

Surgery

Surgery

Complications Blood loss Infection CSF leak Post surgical trauma Poor cosmetic result Persistent cranial defects

Results

Results

Results

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