DESCRIPTION

INTRODUCTION  

CASE

DISCUSSION

CONCLUSION

Craniosynostosis is a common congenital defect requiring surgical intervention by a trained team in order to maximize cosmetic outcome and minimize risk of neurological injury. Recent technological improvements in surgical fixation devices have allowed earlier repair of craniosynostosis, leading to better cosmetic outcomes. The incidence of neurological compromise in non-syndromic craniosynostosis diagnosed early approaches 0%. In this article, we discuss a recent case of bilateral coronal synostosis diagnosed immediately after birth. Radiographic imaging studies (axial and three-dimensional reconstruction) are reviewed pre- and post-operatively. Literature is then reviewed and justification for reducing the tine necessary to wait pre-operativey to maximize normal outcome in patients with congenital skull deformities is discussed. Ultra-early repair of bilateral coronal synostosis can be safely performed on infants within the first week of life.  

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Craniosynostosis is premature closure of the cranial sutures and may affect any of the sutures. Coronal synostosis affects 1/10,000 live births. Untreated, coronal synostosis leads to progressive skull and facial abnormalities, classically ipsilateral frontal bone and orbital flattening, harlequin eye deformity, and a tendency for a towering configuration of the skull. The only treatment for true craniosynostosis is surgical reconstruction. The earlier the surgical reconstruction is performed, the less the long-term cosmetic deformity. Additionally, intracranial hypertension has been described in as many as 55% of patients with single suture synostosis.  With each successive suture closure, the incidence of normal intracranial pressure is essentially halved. Elevated intracranial pressure has been shown to reduce intelligence over a prolonged period.

Previously, the ability of the surgeon to advance the orbit and minimize the fronto-orbital flattening has been limited by the need to rigidly fixate the lateral margins of the bandeau. Non-absorbable plates or wires have been used in the past but migrate inward, occasionally causing dural injury as the child ages. The use of non-absorbable fixation devices has been largely abandoned in children and infants. In 1996, the FDA approved an absorbable plating system that maintains its strength for 6-12 months before completely resorbing. This system, and its competitors, have allowed "temporary" rigid fixation to maximize bandeau advancement for expansion of the frontal fossa, as first described by Tessier. We have used this technique for the last several years at Florida Hospital and describe a case of a 3 day-old infant in whom this technique was used.  

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A term infant (T.G.) was born without difficulty at Florida Hospital. On review in the well-baby nursery, he was noted to have significant bilateral coronal ridging (See figure 1).  

Figure 1: Pre-operative picture revealing towering (black arrow), coronal sutural ridging (blue arrow), and frontal bone and orbital flattening (red arrow)

The coronal ridging led to a pediatric neurosurgical consultation. A head CT with 3-D reconstructions was recommended. It was performed on day of life (DOL) #2. Bilateral coronal synostosis was confirmed in this newborn male. His anterior fontanelle was soft and flat. He was otherwise healthy, tolerating his feedings, without evidence of elevated intracranial pressure. Extensive discussion was had with the family and surgical repair was recommended. On DOL #4,T.G. underwent bicoronal craniotomy and craniofacial reconstruction ~or his craniosynostosis. A wavy Suttar incision was used in order to minimize incisional visibility. Bilateral coronal sutures were noted to have significant synostosis and were removed in their entirety. Bilateral bandeau advancement was performed, advancing each side approximately 1.5cm. The bandeau was hinged nasally although barrel staving was performed below the bandeau to the foramen cecum. The Lactosorb® plating system was then used to rigidly fixate the bandeau advancement. A pi reconstruction of the forehead was performed to create a normal contour to the forehead.  Horizontal barrel staving was then performed allow further bitemporal widening. The incision was closed with absorbable sutures (Vicryl Rapide®). The patient tolerated the procedure well and was sent to the neonatal intensive care unit post-operatively. Although not part of our routine, a head CT with 3-D reconstruction was performed just prior to discharge for illustrative purposes (See figures 2 and 3 for comparison of pre- and post-operative CTs). He was discharged to home on DOL#7, tolerating his feedings and with his incision healing well. He has continued to do well since then with close follow-up (See figure 4).

Figure 2: Pre-operative axial head CT (bone window) revealing coronal synostosis (white arrow) of frontal bone and orbital flattening compared to post-operative axial head CT revealing removal of coronal suture ana extent of fronto-orbital advancement (gray arrows)

Figure 3: Pre-operative head CT with 3-D reconstruction revealing coronal ridging (white arrow) with frontal bone and orbital flattening (gray arrow) compared to post-operative head CT with  3-D reconstruction revealing extent of advancement and pi (white arrow) reconstruction (absorbable plates are holding bone plates in place but are lower density than bone and therefore not visualized on the  3-D reconstruction)

FIGURE 4: Post-operative picture taken 2 weeks post-op revealing extent of improvement and healing of wavy Suttar incision (white arrow)

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Craniosynostosis remains a frustrating pathology to treat for several reasons. Early treatment improves both neurological and long-term cosmetic outcome but increases the risk of recurrent synostosis, requiring repeat operations. Secondary operations and syndromic patients are two factors previously shown to increase the morbidity and mortality of the surgical treatment of craniosynostosis. Thus far, early operation with absorbable plating systems have been successful in reducing the previously reported incidence of the need for repeat operation without increasing the morbidity or mortality. We have now used this system in greater than 30 patients with no mortality. The only complication to date has been a wound infection in a repeat operation done for pan-synostosis in a patient: born with unilateral coronal and sagittal synostosis who developed pan-synostosis. The absorbable plating system was not used in his first operation.

Complex craniosynostosis cases require aggressive treatment. Bicoronal synostosis, aka plagiocephaly or turribrachycephaly, is a specific anatomic diagnosis, easily made by CT although occasionally mistaken by the casual observer for lambdoid synostosis or even benign positional molding. It is important to have a trained examiner evaluate these patients to assess the need for further imaging studies or potential surgical intervention. The cosmetic appearance, while similar, is distinct. Lambdoid synostosis causes contralateral frontal bossing without a harlequin eye and the ipsilateral ear pulled posteriorly whereas benign positional molding, which is the most common cause of abnormal skull shape in infants, causes ipsilateral frontal bossing and the ipsilateral ear pushed anteriorly for a parallelogram effect. As mentioned previously, coronal synostosis usually reveals ipsilateral frontal bone and orbital flattening, harlequin eye deformity, and a tendency for a towering configuration of the skull. That being said, they all have a variable amount of frontal and occipital flatness. 

Craniosynostosis requires surgical intervention whereas benign positional molding may be left alone or treated with an occipital molding helmet. Our hope is that with rigid fixation of a temporary nature, we will be able to reap the benefits of early operation, i.e. improved neurological and cosmetic outcome, without the complications, i.e. recurrent synostosis/lack of growth.  

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1. Craniosynostosis is a potentially life-altering condition that requires early intervention and monitoring to minimize the risk of neurological injury and worsening cosmetic deformity in affected children.

2. Craniosynostosis may be safely treated ultra-early using current surgical techniques and tools.

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