Skull Base 2010; 20(2): 119-123
DOI: 10.1055/s-0029-1238216
CASE REPORT

© Thieme Medical Publishers

Median Facial Cleft with a Frontoethmoidal Encephalocele Treated with Craniofacial Bipartition and Free Radial Forearm Flap: A Case Report

Marton König1 , 4 , Bernt Due-Tønnessen1 , 4 , Terje Osnes2 , 4 , Jan-Ragnar Haugstvedt3 , 4 , Torstein R. Meling1 , 4
  • 1Department of Neurosurgery, The National Hospital, Oslo, Norway
  • 2Department of ENT, The National Hospital, Oslo, Norway
  • 3Department of Orthopedics, The National Hospital, Oslo, Norway
  • 4Rikshospitalet HF University Hospital, Oslo, Norway, The National Hospital, Oslo, Norway
Further Information

Publication History

Publication Date:
29 October 2009 (online)

ABSTRACT

We describe a patient with a median facial cleft with a frontoethmoidal encephalocele, hypertelorism, hydrocephalus, and cerebrospinal fluid (CSF) leakage referred to our department due to numerous complications after previous surgical treatments. An 8-year-old girl, born with median cleft syndrome, underwent neurosurgical repair of the encephalocele at another hospital and cleft lip/palate repair later in the same year. Her hydrocephalus was treated with a ventriculoperitoneal shunt, but she underwent numerous shunt revisions due to recurrent intracerebral infections. In 2008, she was rehospitalized due to a gram-negative meningitis and cerebral abscess. She underwent surgery where part of her frontal bone was removed due to osteomyelitis. She was referred to our department due to persistent CSF leakage, recurrent infections, and significant dura defect. In addition, she had hypertelorism and a strongly reduced vision. We performed a monobloc and facial bipartition osteotomy where 15 mm of her frontal and nasal bone was removed after facial bipartiton. The dura defect was closed using a free fasciocutanous flap. The patient had no CSF leakage or infections postoperatively, and her hypertelorism was reduced. The case represents the first monobloc and facial bipartition osteotomy performed in Norway as a part of the treatment of median cleft syndrome with a nasoethmoidal encephalocele.

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Torstein R MelingM.D. Ph.D. 

Department of Neurosurgery, The National Hospital

0027 Oslo, Norway

Email: torstein.meling@rikshospitalet.no