Anterior encephaloceles in children of Assamese tea workers

Abstract

Object: Anterior encephaloceles are rare congenital malformations. Most of the cases in the literature are reported from Southeast Asia. In India it is seen more frequently among manual laborers in the tea gardens of Assam. A brief background of the patients, clinical presentation, operative treatment, and outcome are discussed, with a review of the relevant literature. The causes and pathogenesis of anterior encephaloceles are discussed. The authors' surgical approach to repair of the defect, postoperative complications, and results are described.

Methods: Twenty-eight patients (mean age 38 months, range 1 month-12 years) with anterior encephaloceles who presented between 1998 and 2007 are included in the study. Patients were assessed for physical and psychological growth and development; any associated anomalies were noted. A detailed history of the patient's family, including the prenatal history, was obtained. Lesions were classified with the help of neuroimaging studies (skull x-ray, CT, ultrasonography, and MR imaging studies) and confirmed at surgery.

Results: Sixteen patients had nasofrontal, 9 had nasoethmoidal, and 3 had nasoorbital encephaloceles, and 12 patients had associated hydrocephalus. The modes of presentation were nasofrontal swelling, watering from the eyes, CSF leakage, fever, and vomiting. Parents of all the patients were ethnic tea garden workers. The average parental age at the time of marriage was 24 years for men and 18 years for women. Consanguinity was present in 6 patients. Alcohol consumption and tobacco chewing were a common practice in both parents. A total of 42 surgical procedures were done in 16 patients, one of whom died of postoperative meningitis. The mean follow-up duration was 38 months (1-92 months).

Conclusions: Anterior encephaloceles are rare in Western countries and other states in India, but this defect is more commonly seen among the ethnic tea garden workers in Assam. Transcranial repair is the treatment of choice. Unlike encephaloceles in other locations, anterior encephaloceles have a better clinical outcome after surgery.