Massive spontaneous hemothorax, giant intrathoracic meningocele, and kyphoscoliosis in neurofibromatosis type 1

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 ske...

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Bibliographic Details
Published in:Journal of surgical technique and case report 2014-01, Vol.6 (1), p.33-36
Main Authors: Yusuf, Ayodeji Salman, Pillai, Ashok, Menon, Sajesh K, Panikar, Dilip
Format: Article
Language:English
Subjects:
Abnormalities
Case Report
Complications and side effects
Neural tube
Neurofibromatosis
Thoracotomy
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Summary: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.
ISSN:2006-8808
0976-2825
DOI:10.4103/2006-8808.135151