Back to Search
Start Over
Management of Gorham Stout disease with skull-base defects: Case series of six children and literature review.
- Source :
-
International journal of pediatric otorhinolaryngology [Int J Pediatr Otorhinolaryngol] 2019 Sep; Vol. 124, pp. 152-156. Date of Electronic Publication: 2019 Jun 05. - Publication Year :
- 2019
-
Abstract
- Background: Gorham-Stout disease (GSD) is a rare lymphatic disorder which results in bone destruction. Defects of the skull base are difficult to manage, we describe cases to better understand the disease and discuss treatment.<br />Methods: Retrospective study including all patients treated for GSD skull-base defects. Medical records, clinical, imaging and treatment data were studied. A systematic review of the literature included case reports of the diseases for further analysis.<br />Results: 6 patients (5 males, 1 female) were included. Mean age at diagnosis was 3.5 years (range 0-10). Follow-up was of 5.2 years. Patients were divided into Naso-temporal (NT) and Vertebro-temporal (VT) groups following anatomical location. NT patients (4 patients) all had petrous defects extending anteriorly, including sphenoid, ethmoidal and mandibular defects. They all had cerebro-spinal fluid leak (CSF) and recurrent meningitis (range from 3 to 7). Two of those patients had sequelae including deafness, paralysis and epilepsy. VT patients (2 patients) all had temporal, occipital bone and cervical vertebrae defects. None had CSF leaks but both died from medullar compression (preceded by tetraparesis in one case). Overall, five out of six patients had type I Chiari malformation. Interferon seemed to be the most efficient medical treatment. Surgery included petrectomy, endonasal surgery for CSF leak management and neurosurgery for medullar management but could not guarantee long-term effects.<br />Conclusion: Main issues in skull base defects are CSF leaks and medullar compressions. Surgical treatment is necessary in both cases but can only be satisfactory if general medical treatment can stabilise the disease.<br /> (Copyright © 2019 Elsevier B.V. All rights reserved.)
- Subjects :
- Arnold-Chiari Malformation complications
Cerebrospinal Fluid Leak diagnosis
Cerebrospinal Fluid Leak etiology
Cerebrospinal Fluid Leak surgery
Child
Child, Preschool
Endoscopy
Female
Humans
Infant
Infant, Newborn
Male
Neurosurgical Procedures
Osteolysis, Essential complications
Osteolysis, Essential diagnostic imaging
Retrospective Studies
Osteolysis, Essential surgery
Skull Base
Subjects
Details
- Language :
- English
- ISSN :
- 1872-8464
- Volume :
- 124
- Database :
- MEDLINE
- Journal :
- International journal of pediatric otorhinolaryngology
- Publication Type :
- Academic Journal
- Accession number :
- 31195309
- Full Text :
- https://doi.org/10.1016/j.ijporl.2019.06.002