title: Massive spontaneous hemothorax, giant intrathoracic meningocele, and kyphoscoliosis in neurofibromatosis type 1.
abstract: Neurofibromatosis type 1 (NF-1) is a heterogeneous autosomal dominant disease with an incidence ranging from 1 in 2500 to 1 in 3000. Rare intrathoracic vascular disorders resulting in massive spontaneous hemothorax with fatal consequences may occur in these patients, so also are various types of skeletal dysplasia which may result in dramatic presentations, posing management challenges to the attending physicians. We report the case of a 43-year-old woman with NF-1 who developed spontaneous massive hemothorax and was discovered to have a concurrent giant intrathoracic meningocele and thoracic kyphoscoliosis with severe vertebral dysplastic changes. Surgical treatment via a right thoracotomy with primary repair of the meningocele and spinal fusion with fibula graft resulted in good outcome. This case represents an extreme manifestation of this otherwise indolent disease in clinical practice.
authors.affiliation: Department of Surgery, Neurosurgery Unit, University of Ilorin and University of Ilorin Teaching Hospital, Ilorin, Nigeria.
authors.forename: Ayodeji Salman
authors.initials: AS
authors.lastname: Yusuf
authors.affiliation: Department of Neurosurgery, Amrita Institute of Medical Sciences and Research Centre, Cochin, Kerala, India.
authors.forename: Ashok
authors.initials: A
authors.lastname: Pillai
authors.affiliation: Department of Neurosurgery, Amrita Institute of Medical Sciences and Research Centre, Cochin, Kerala, India.
authors.forename: Sajesh K
authors.initials: SK
authors.lastname: Menon
authors.affiliation: Department of Neurosurgery, Amrita Institute of Medical Sciences and Research Centre, Cochin, Kerala, India.
authors.forename: Dilip
authors.initials: D
authors.lastname: Panikar
keywords: Hemothorax; kyphoscoliosis; meningocele; spinal fusion