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

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.

KEYWORDS

Congenital deformity - encephalocele - median cleft syndrome - craniofacial surgery - facial bipartition

REFERENCES

• 1 French B N. Midline fusion defects and defects of formation. In: Youmans JR Neurological Surgery, 2nd ed. Philadelphia; WB Saunders 1982 3: 1236-1380
• 2 French B N. Midline fusion defects and defect of formation. In: Youmans JR Neurological Surgery, 3rd ed. Philadelphia; WB Saunders 1990 2: 1081-1235
• 3 David D J. Cephaloceles: classification, pathology, and management—a review.  J Craniofac Surg. 1993;  4 192-202
• 4 David D J, Sheffield L, Simpson D, White J. Fronto-ethmoidal meningoencephaloceles: morphology and treatment.  Br J Plast Surg. 1984;  37 271-284
• 5 David D J, Simpson D A. Frontoethmoidal meningoencephaloceles.  Clin Plast Surg. 1987;  14 83-89
• 6 Mahapatra A K. Anterior encephaloceles.  Indian J Pediatr. 1997;  64 699-704
• 7 Mahapatra A K, Suri A. Anterior encephaloceles: a study of 92 cases.  Pediatr Neurosurg. 2002;  36 113-118
• 8 Mahapatra A K, Tandon P N, Dhawan I K, Khazanchi R K. Anterior encephaloceles: a report of 30 cases.  Childs Nerv Syst. 1994;  10 501-504
• 9 Mahatumarat C, Rojvachiranonda N, Taecholarn C. Frontoethmoidal encephalomeningocele: surgical correction by the Chula technique.  Plast Reconstr Surg. 2003;  111 556-565 discussion 566-567
• 10 Simpson D A, David D J, White J. Cephaloceles: treatment, outcome, and antenatal diagnosis.  Neurosurgery. 1984;  15 14-21
• 11 Smit C S, Zeeman B J, Smith R M, de V Cluver P F. Frontoethmoidal meningoencephaloceles: a review of 14 consecutive patients.  J Craniofac Surg. 1993;  4 210-214
• 12 Suwanwela C. Geographical distribution of fronto-ethmoidal encephalomeningocele.  Br J Prev Soc Med. 1972;  26 193-198
• 13 Suwanwela C, Suwanwela N. A morphological classification of sincipital encephalomeningoceles.  J Neurosurg. 1972;  36 201-211
• 14 Tessier P. Orbital hypertelorism. I. Successive surgical attempts. Material and methods. Causes and mechanisms.  Scand J Plast Reconstr Surg. 1972;  6 135-155
• 15 Aung T, Hta K. Epidemiology of frontoethmoidal encephalomeningocoele in Burma.  J Epidemiol Community Health. 1984;  38 89-98
• 16 Mahatumarat C, Taecholarn C, Charoonsmith T. One-stage extracranial repair and reconstruction for frontoethmoidal encephalomeningocele: a new simple technique.  J Craniofac Surg. 1991;  2 127-133 discussion 134
• 17 Suwanwela C, Suwanwela N. A morphological classification of sincipital encephalomeningoceles.  J Neurosurg. 1972;  36 201-211
• 18 Rapport II R L, Dunn Jr R C, Alhady F. Anterior encephalocele.  J Neurosurg. 1981;  54 213-219
• 19 van der Meulen J C, Vaandrager J M, Meulen van der J C. Surgery related to the correction of hypertelorism.  Plast Reconstr Surg. 1983;  71 6-19
• 20 Marchac D, Arnaud E. Midface surgery from Tessier to distraction.  Childs Nerv Syst. 1999;  15 681-694
• 21 Ortiz-Monasterio F, del Campo A F, Carrillo A. Advancement of the orbits and the midface in one piece, combined with frontal repositioning, for the correction of Crouzon's deformities.  Plast Reconstr Surg. 1978;  61 507-516
• 22 Posnick J C. Monobloc and facial bipartition osteotomies: a step-by-step description of the surgical technique.  J Craniofac Surg. 1996;  7 229-250 discussion 251