4Molded 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.
18Treatment Position Therapy Keep off the flat spotSleep<4 m/o Wedges and blanketsRoll on side>4 m/o Fetal positionLet baby fall asleep in usual positionThen rotate onto side in fetal positionStart at naps
24Treatment Molding Helmet My indicationsBorderline parentsCan’t get concept of keeping off flat spotHypotonic childrenWon’t have head control by 4 monthsHydrocephalus post shuntWill not have brain growth to mold skullSignificant facial bulgeJust not getting betterTypically make this decision around 7-8 months of age.Babies that cant’t be repositioned, severe deformities.
26What is it? Premature fusion of the cranial sutures Sutures allow progressive enlargement of the skull with brain growthNormal sutural fusion is complete at 6 to 8 years of agePremature fusion produces progressive skull deformity
29Epidemiology Occurs in 1 in 2100 children Primary non-syndromic is most commonLambdoid synostosis is very rareMultiple sutures involved in 8% of non-syndromic casesCalvarial bones grow perpendicular to the sutures. Growth: 50% at 2 months, 100% at 6 months, and 200% at 10 months.
30Calvarial Development Intramembranous ossificationMargins of calvarial bones form osteogenic frontSutures form at sites of near contactNew bone is laid down by osteoblasts in spicules at the sutural marginsOverall bone growth is driven by the expanding brainCalvarial 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 cellsSutures form at areas of major dural reflections.Interaction between dura and osteogenic fronts maintains sutures Brain growth is exponential during the first year.
32Known causes of craniosynostosis PathobiologyKnown causes of craniosynostosisTeratogensValproic acid, aminopterin, retinoic acidMalformationMicrocephalyEncephaloceleshunted hydrocephalusholoprosencephalyMetabolic disordersRicketsHyerthyroidismMucopolysaccharidosesHurler’s, Morquio’sHematologic disordersThalassemias, sickle cell anemia, polycythemia veraHyperthyroidism: premature osseous fusion, shunted hydrocephalus: true bony fusion does not occur2-6% of isolated sagittal and 8-14% of cases of coronal synostosis are familial. Likely autosomal dominant with 0.6% penetrance by segregation analysis/
33Pathobiology Syndromic Crouzon Apert’s Pfeiffer Jackson-Weiss Mutation in gene coding for fibroblast growth factor receptors
34Pathobiology <10% inherited or syndromic 90% spontaneous Uterine constraintEarly drop into cervical canalMultiple birthsSevere back pain last month of pregnancy
35PathobiologyPremature closure of calvarial sutures also affects the skull baseCoronal and metopicEffect extends to nasoethmoid complex, orbital roofs, and supraorbital ridges
36Diagnosis Characteristic skull shape Lack of movement at suture Palpable ridging of fused sutureCT scanFusion, sclerosis, skull base deformitiesDeformational skull anomalies are much more common
37Metopic1 frontal keel 2 bitemporal narrowing 3 coronal suture displaced anteriorly 4 parieto-occipital bulging 5 bizygomatic narrowing 6 posterior displacement of supraorbital rim
46The case for surgery Aesthetic and functional Elevated ICP in 14% of single and 47% of children with multiple sutural synostosisUncorrected children often socially isolated and stigmatizedLow risk with modern craniofacial surgery