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Pediatric Craniofacial Surgery

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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 withSAGITTAL 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

 

 

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 SYNOSTOSISaka 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”?