Craniosynostosis and Plagiocephaly

Transcription

1 Craniosynostosis and Plagiocephaly Andrew Jea MD MHA FAAP Professor and Chief Section of Pediatric Neurosurgery Riley Hospital for Children Department of Neurosurgery Indiana University School of Medicine Goodman Campbell Brain & Spine Disclosures No financial disclosures but Thanks and gratitude for my colleagues in pediatric neurosurgery for these slides Dr. Lance Governale at Nationwide Children s Hospital Dr. Laurie Ackerman at Riley Hospital for Children Neurosurgery (2/55) Agenda Craniosynostosis vs positional plagiocephaly Anterior fontanel closure Microcephaly Benign macrocrania Neurosurgery (3/55)

2 Craniosynostosis Premature fusion of 1 cranial suture(s) Incidence 1:2000 to 1:2500 live births Some syndromic/familial, most not Fibroblast growth factor receptor pathways Skull cannot grow perpendicular to the suture so it grows parallel (Virchow s law) Differentiate from positional plagiocephaly Neurosurgery (4/55) Normal Sutures Neurosurgery (5/55) Positional Plagiocephaly Posterior flattening Anterior ear and forehead displacement on side of flattening Parallelogram shape Not craniosynostosis Neurosurgery (6/55)

3 Sagittal Craniosynostosis Long (AP) Narrow (lateral) Frontal bossing Occipital bossing Fontanel open or closed Scaphocephaly Neurosurgery (7/55) Sagittal Craniosynostosis Neurosurgery (8/55) Bilateral Coronal Craniosynostosis Short (AP) Wide (lateral) Syndromic? Symmetric positional flattening? Brachycephaly Neurosurgery (9/55)

4 Bilateral Coronal Craniosynostosis Neurosurgery (10/55) Unilateral Coronal Craniosynostosis Forehead flattening Orbit drawn up Fontanel displaced Nasal root deviation Neurosurgery (11/55) Harlequin sign Neurosurgery (12/55)

5 Unilateral Coronal Craniosynostosis mediclopedia.tumblr.com Neurosurgery (13/55) Metopic Craniosynostosis Pointed forehead Narrow forehead Triangle shaped Hypotelorism Ridging alone may be followed Trigonocephaly Neurosurgery (14/55) Metopic Craniosynostosis thememorymaker.wordpress.com 2012 shibaa.ivil.tripod.com 2012 Neurosurgery (15/55)

6 Lambdoid Craniosynostosis Posterior flattening Posterior ear and forehead displacement Trapezoid shape Ear forced down Rare Mark Proctor - Boston Children s Hospital Neurosurgery (16/55) Lambdoid Craniosynostosis Posterior flattening Posterior ear and forehead displacement Trapezoid shape Ear forced down Rare Mark Proctor - Boston Children s Hospital Neurosurgery (17/55) Lambdoid vs Positional Cannon - American Family Physician 2004 Cannon - American Family Physician 2004 Neurosurgery (18/55)

7 Diagnostic Uncertainty Skull x rays Phased out in favor of CT Head limited skull very low dose Same or less radiation than 4 view skull x rays Provides 3D skull images, brain not seen If shows open sutures, then no craniosynostosis (Epic orderable is RADCT400) Neurosurgery (19/55) 3D CT Neurosurgery (20/55) Natural History Left untreated, there is a risk for: Worsening head shape Overall head growth restriction leading to increased intracranial pressure (ICP) Neurosurgery (21/55)

8 Elevated ICP Neurosurgery (22/55) Treatment Goals Unlocking the bones Reshaping the skull Open vs Endoscopic vs Springs Neurosurgery (23/55) Craniosynostosis Treatment Open Endoscopic Spring-Assist >6 mo 2.5 to 3.5 mo 3 to 6 mo Incision ear to ear One or two 2cm incisions One 5cm incision for sagittal Eyes swollen shut No periorbital swelling Usually no periorbital swelling Transfusion always Transfusion rare Transfusion rare ICU 1d, Floor 3d Floor 1d Floor 1d No helmet Helmet required No helmet, but second surgery 3m Neurosurgery (24/55)

9 Open FOA Fronto orbital Advancement Posterior CVR Neurosurgery (25/55) Sagittal Synostosis Repair Options Strip Craniectomy Pi Procedure Craniosynostosis Treatment Open Endoscopic Spring-Assist >6 mo 2.5 to 3.5 mo 3 to 6 mo Incision ear to ear One or two 2cm incisions One 5cm incision for sagittal Eyes swollen shut No periorbital swelling Usually no periorbital swelling Transfusion always Transfusion rare Transfusion rare ICU 1d, Floor 3d Floor 1d Floor 1d No helmet Helmet required No helmet, but second surgery 3m Neurosurgery (27/55)

10 Endoscopic Neurosurgery (28/55) Endoscopic Strip Craniectomy with Helmet Helmet Hard shell Moldable foam inside Gaps where skull growth desired 23 hours per day Every day Managed by orthotist Neurosurgery (30/55)

11 Helmet Neurosurgery (31/55) Craniosynostosis Treatment Open Endoscopic Spring-Assist >6 mo 2.5 to 3.5 mo 3 to 6 mo Incision ear to ear One or two 2cm incisions One 5cm incision for sagittal Eyes swollen shut No periorbital swelling Usually no periorbital swelling Transfusion always Transfusion rare Transfusion rare ICU 1d, Floor 3d Floor 1d Floor 1d No helmet Helmet required No helmet, but second surgery 3m Neurosurgery (32/55) Springs Stainless steel Force is variable Patient age Bone thickness Deformity severity Second surgery for removal at 3m Greg Pearson - Nationwide Children s Hospital Neurosurgery (33/55)

12 Cranial Expansion with Normal Calvarial Shape Distraction Osteogenesis Neurosurgery (34/55) Cranial Expansion with Normal Calvarial Shape Bicoronal incision, craniotomy, leave bone on Implant distractors, wait 4-5 days Distract 1 mm/day x 30 days Remove posts, close skin (in office) Wait 3 months Remove distractors (in OR) Neurosurgery (35/55) Cranial Expansion with Normal Calvarial Shape Neurosurgery (36/55)

13 Cranial Expansion with Normal Calvarial Shape Preop: headache & papilledema Postop: both resolved Neurosurgery (37/55) Ilizarov and Distraction Osteogenesis: Applications in Syndromic Synostosis Plagiocephaly Treatment Cosmetic issue, does not affect brain devel Parents concerned? Alternate head position for sleep, holding Tummy time PT for torticollis Development of head control Helmet Neurosurgery (39/55)

14 Pumpkin s/p Helmet in Circleville Neurosurgery (40/55) Anterior fontanel closure Median time to closure 14 months Wide variation in closure times 1% closed at 3 months 38% closed at 1 year 96% closed at 2 years Neurosurgery (41/55) Early fontanel closure Assess for craniosynostosis Head growth along a curve? Signs of high ICP? HA, N/V, lethargy, upgaze palsy Developing normally? Neurosurgery (42/55)

15 Microcephaly Assess for craniosynostosis Brain growth drives skull growth Neurology referral Neurosurgery (43/55) Benign Macrocrania Head circumference initially crosses curves but then parallels a curve, often > 95% Familial large heads run in the family Neurosurgery (44/55) Benign Macrocrania Assess for craniosynostosis Head growth along a curve? Signs of high ICP? HA, N/V, lethargy, upgaze palsy Bulging, tense fontanel when upright Developing normally? Can be some delay in head control due to size Neurosurgery (45/55)

16 Diagnostic Uncertainty Head US No radiation Will definitively assess for hydrocephalus Large subarachnoid spaces are expected Limited MRI brain (at NCH) If fontanel closed No radiation and no sedation Neurosurgery (46/55) Thank you