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

OBJECTIVES

NEWBORN CRANIAL ANATOMY

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

HEAD GROWTH

• 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

Pre-Op Lateral Skull X-Ray

METAL FIXATION COMPLICATION

Pre-op CT

METAL FIXATION COMPLICATION

Intra-op Exposure

METAL FIXATION COMPLICATION

Internal Skull

ABSORBABLE PLATING

Intra-op

ABSORBABLE PLATING

Post-op

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*

_{*Childs Nerv Syst. 2005 Oct;21(10):913-21. Epub 2005 May 3.
Intracranial pressure monitoring in children with single suture and complex craniosynostosis: a review.}

_{Tamburrini G, Caldarelli M, Massimi L, Santini P, Di Rocco C.}

 _{Childs Nerv Syst. 1995 May;11(5):269-75.}

_{Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management.}

_{Thompson DN, Harkness W, Jones B, Gonsalez S, Andar U, Hayward R.}

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.

CRANIOSYNOSTOSIS

• TRUE SYNOSTOSIS OCCURS IN 5  /  10,000   LIVE BIRTHS

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

Siblings with
 SAGITTAL SYNOSTOSIS

SAGITTAL SYNOSTOSIS 
LATERAL SKULL RADIOGRAPH

SAGITTAL SYNOSTOSIS
AP SKULL RADIOGRAPH

SAGITTAL SYNOSTOSIS
HEAD CT WITH 3-D RECONSTRUCTION

PRE-OP REPAIR OF
SAGITTAL SYNOSTOSIS

SURGICAL REPAIR OF
SAGITTAL SYNOSTOSIS

SURGICAL REPAIR OF
SAGITTAL SYNOSTOSIS

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

CORONAL SYNOSTOSIS
HEAD CT WITH 3-D RECONSTRUCTION

POST-OP
CORONAL SYNOSTOSIS

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 SYNOSTOSIS
 aka TRIGONOCEPHALY

INCIDENCE:  0.5-1/10,000 live births

• “Beaked” forehead
• First suture to close physiologically (3-5 months of age radiographically)

TRIGONOCEPHALY

METOPIC SYNOSTOSIS

LAMBDOID SYNOSTOSIS 
aka PLAGIOCEPHALY

INCIDENCE:  0.5-1/10,000 live births

Clinical and radiographic distinction from occipital molding

LAMBDOID SYNOSTOSIS

OCCIPITAL FLATNESS

aka BENIGN POSITIONAL MOLDING

aka NON-SYNOSTOTIC POSTERIOR POSITIONAL PLAGIOCEPHALY

• Treatment of choice is an occipital molding helmet, the price of which has dropped from >$5000 5 years ago to $2000 today
• Difficulty with education of payors
• Improves cosmetic outcome from 40% acceptable to 80% good

POSITIONAL PLAGIOCEPHALY

LAMBDOID SYNOSTOSIS

OR POSITIONING

READY FOR PREP

PREPPED

BURR HOLE OPENING

PROTECTING THE DURA

CUTTING THE BONE

CASE CLOSED

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?

PACU CONSIDERATIONS

As always ABCs

AIRWAY

• Concomitant Disease Process
• History of Intubation
• Neurological Status
• Plan for Further Images
• Other Midline Anomalies

BREATHING

• What medications were used?
• What medications has the child been exposed to in the past?
• What is their baseline respiratory rate and oxygen saturation?

CIRCULATION!!!!!!!!!!!

• EBL can be significant in these cases
• 50-100% transfusion incidence in open craniofacial repair
• 25-75% transfusion incidence in endoscopic craniofacial repair
• The skull is a bilaminar bone, with marrow in the middle, the child will continue to ooze for days afterward, either into the scalp or a drain
• #1 Cause of Mortality in Craniofacial Surgery is Blood Loss

• Need to Closely Monitor BP, P, H&H
• Most Patients will be admitted to the ICU
• Assess Quality of Blood Oozing from Incision and Size of Incisions
• What Blood or Volume Expanders Remain Available?

What is an “abnormal head shape”?