Pediatric Neurosurgery

Craniofacial Surgery for Children

OBJECTIVES

1. Understand Skull Anatomy
2. Understand Intra-Operative Concerns with Craniofacial Reconstruction
3. Prepare for Potential Post-Operative Complications
4. Identify Risk Factors and Ways to Avoid Post-Operative Complications in Complex Craniofacial Patients

HEAD GROWTH

• Head circumference increases from 35cm to 47cm during the first year of life
• Brain volume doubles in the first year of life
• Head growth slows dramatically after the first 12-18 months of life
• During the first 12-18 months of life, most head growth is accomplished by expansion along suture lines
• Thereafter, the skull grows by internal resorption and external replacement

ABSORBABLE PLATING

• The importance of skull growth by bony resorption is that rigid fixation, e.g. metal, may migrate intracranially, piercing the dura and causing head-aches, seizures, or worse
• Absorbable plating systems made of poly-lactic acid polymers have been devised to last 6-24 months, depending on the polymer

METAL FIXATION COMPLICATION

ETIOLOGY OF CRANIOSYNOSTOSIS

• Unknown in sporadic cases
• All have a familial tendency
• Most felt to be secondary to a defect in fibroblast function

CRANIOSYNOSTOSIS

• Single suture synostosis is rarely associated with intracranial anomalies. It is a primarily cosmetic issue as brain volume is maintained by abnormal skull growth.
• However, there is a 10-15% incidence of elevated intracranial pressure in untreated craniosynostosis^*
• The sooner craniosynostosis is addressed, the better the cosmetic outcome.
• Conversely, the sooner craniosynostosis is addressed, the more likely the patient will require a second operation.
• TRUE SYNOSTOSIS OCCURS IN 5 / 10,000 LIVE BIRTHS

1. Childs Nerv Syst. 2005 Oct;21(10):913‐21. Epub 2005 May 3.
2. Intracranial pressure monitoring in children with single suture and complex craniosynostosis: a review. Tamburrini G, Caldarelli M, Massimi L, Santini P, Di Rocco C.
3. Childs Nerv Syst. 1995 May;11(5):269‐75.
4. Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management. Thompson DN, Harkness W, Jones B, Gonsalez S, Andar U, Hayward R.

INCIDENCE

• SAGITTAL 50%
• CORONAL 25%
• LAMBDOID 10-15%
• METOPIC 10-15%

SAGITTAL SYNOSTOSIS AKA SCAPHOCEPHALY

• INCIDENCE: 2/10,000 live births
• “Keel” deformity
• Rarely associated with skull base or facial abnormalities

CORONAL SYNOSTOSIS

• INCIDENCE: 1/10,000 live births
• “Harlequin” eye with unilateral coronal synostosis
• Invariably associated with facial and occasionally skull base anomalies
• Present in Apert's, Crouzon's, and Pfeiffer's syndromes

APERT'S SYNDROME

• Acrocephalosyndactyly
• Uncommon - usually sporadic but autosomal dominant transmission occurs
• Proptosis, high arched palate, complete symmetric syndactyly of fingers and toes, mental retardation
• Phenotypically similar to Crouzon's

CROUZON'S SYNDROME

• Most common of the craniofacial dysmorphic states
• Autosomal dominant transmission
• Proptosis, midface hypoplasia, usually cognitively intact
• Similar to Apert's but smarter and no syndactyly

PFEIFFER'S SYNDROME

• Autosomal dominant transmission
• Proptosis, mid-face hypoplasia, mild syndactyly
• Phenotypically midway between Apert's and Crouzon's

CARPENTER'S SYNDROME

• Autosomal recessive transmission
• Syndactyly, mental retardation, congenital heart defects
• Most common of the autosomal recessive craniofacial dysmorphic states

METOPIC SYNOSTOSISaka TRIGONOCEPHALY

• INCIDENCE: 0.5-1/10,000 live births
• “Beaked” forehead
• First suture to close physiologically (3-5 months of age radiographically)

LAMBDOID SYNOSTOSIS AKA PLAGIOCEPHALY

• INCIDENCE: 0.5-1/10,000 live births
• Clinical and radiographic distinction from occipital molding

IMPORTANT HISTORY DATA

• Were they premature?
• Medications?
• Seizures?
• Other Medical Conditions?

IMPORTANT INTRA-OPERATIVE DATA

• EBL?
• Blood given/available?
• Last H&H
• Complications, e.g. sinus/dural injury
• What lines are in, e.g. Central line, A-line, or drain?
• Is there a bony defect? If so, where?