Basal encephalocele: surgical strategy and functional outcomes in the Tokyo experience

OBJECTIVEThe basal encephalocele (BEC) is the rarest form of encephalocele, with an incidence of about 1/35,000 live births. The incidence of its subtype, sphenoidal BEC, is even lower at about 1/700,000 live births. The aim of this study was to propose the optimal surgical approach to repairing BEC, with special attention to the reconstruction of the skull base bone defect.METHODSFourteen consecutive pediatric patients with BEC who underwent surgical repair between March 2004 and March 2020 (10 boys and 4 girls, age 25 days to 7 years, median age 4 months) were enrolled. The follow-up period of the surviving patients ranged from 53 to192 months (mean 119.8 months). The patient demographics, BEC subtypes, preoperative clinical condition, radiographic findings, surgical procedures, and postoperative course were retrospectively analyzed.RESULTSThere were 4, 8, and 2 cases of sphenoidal BEC, sphenoethmoidal BEC, and ethmoidal BEC, respectively. The size of the bone defect was small in 3 patients, medium in 7, and large in 4 patients. All the patients with sphenoethmoidal and ethmoidal BEC showed associated congenital anomalies other than cleft palate. In total, 25 operations were performed. Two patients underwent multiple operations, whereas the remaining 9 patients received only 1 operation. The transoral transpalatal approach was the initial procedure used in all 14 patients. The transfrontobasal approach was applied as an additional procedure in 2 patients and as part of a 1-stage combined operation in 2 patients. Autograft bone alone was used for skull base reconstruction in 17 early operations. A titanium mesh/plate was used in the remaining 8 operations without any perioperative complications. All BECs were successfully repaired. Three patients died during the clinical course due to causes unrelated to their surgery. All but one of the surviving patients started growth hormone replacement therapy before school age.CONCLUSIONSBased on the authors’ limited experience, the key to successful BEC repair involves circumferential dissection of the BEC and a firm reconstruction of the skull base bone defect with a titanium plate/mesh. The transoral transpalatal approach is a promising, reliable procedure that may be used in the initial operation. When a cleft palate is absent, transnasal endoscopic repair is recommended. The transfrontobasal approach should be reserved for cases with a huge BEC and other anomalies. Long-term prognosis is apparently favorable in survivors.

Morning Glory Syndrome

Morning Glory Syndrome is a very rare condition. It is characterized by an enlarged and funnel-shaped optic disc excavated and is associated with other craniofacial anomalies such as hypertelorism, cleft lip, cleft palate, and basal encephalocele. CaseReport. A case of a 30 year-old man who came for neurosurgical evaluation after a magnetic resonance imaging (MRI) scan that showed spheno-ethmoidal encephalocele is reported. Conclusions. There is a high association between basal encephalocele,midface abnormalities and Morning Glory Syndrome. A neurosurgical, ophtalmological and endocrinological evaluation must always be done. The patients present with ophtalmic signs and/or strabismus or poor vision. An image study should always be performed to delineate the extent of the lesion and a complete hormone screening should be carried out to exclude pituitary deficiency.

Basal Encephalocele-an Uncommon Cause of Respiratory Distress in Newborn

Basal encephalocele is one of the important causes of respiratory distress in newborn, although uncommon and hence under emphasized. It is a form of neural tube defect that usually presents with symptoms of nasal obstruction. Delayed presentation with variable symptoms have been noted. Collaborative surgical intervention is the treatment modality with different approaches. However, intervention early in neonatal period is a challenge in our region with resource limitations. Prognosis is worse with associated malformations and chromosomal anomalies. We report two cases of newborn with basal encephalocele presenting as respiratory distress at birth.Nepalese Journal of ENT Head and Neck Surgery, Vol. 5, No. 2, 2014, Page: 18-20