Frontoethmoidal cephalocele: our experience of eleven cases managed surgically.

INTRODUCTION: Frontoethmoidal or sincipital cephaloceles are congenital malformations characterized by externalization of the meninges and/or brain tissue through a congenital bone defect between the frontal and ethmoid bones. These sincipital cephaloceles are very infrequent. While in developed countries the measures taken have brought about virtually zero frequency of this pathology, in our work environment these measures are virtually nonexistent, so that frontoethmoidal cephaloceles remain one of our concerns.

AIM: To describe the particularity of the management of frontoethmoidal cephaloceles in our country.

METHODS: This was a retrospective study carried out between January 1, 2007 and June 30, 2013. It concerned all cases of frontoethmoidal cephaloceles managed in the Neurosurgery Department of the University Hospital of Ouagadougou.

RESULTS: A total of 11 patients (6 females and 5 males) were attended to in 6.5 years. The age of the patients ranged from 1 day to 12 years, and 8 patients (72.72%) were less than 4 months old; 1 case was diagnosed at antenatal ultrasound. Clinically, all cases consisted of congenital cephalic swelling of gradual increase. A CT scan was performed in all cases and an ultrasound of the associated mass was carried out in 2 cases. Transcranial approach was done for all patients with 3 patients having had an incision of the mass to reduce redundant skin. The immediate postoperative course was favorable in 10 cases; 1 death was observed by cerebrospinal meningitis. We noted 1 case of recurrence.

CONCLUSION: The surgical treatment of frontoethmoidal cephaloceles is complex, sometimes requiring a multidisciplinary team. However, early closing of the malformation before the appearance of important bone deformities can prevent maxillofacial surgery. The results of the surgery are satisfactory as regards the vital prognosis. However, the fear of a more or less long-term occurrence of psychomotor retardation and the complications of early surgery make the recommendation and emphasis of prevention our main concern.