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Cent Eur Neurosurg 2009; 70(1): 43-47
DOI: 10.1055/s-0028-1082321
Case Report

© Georg Thieme Verlag KG Stuttgart · New York

A Non-midline Spheno-orbital Encephalocele in a Newborn

Eine laterale spheno-orbitale Encephalocele bei einem NeugeborenenU. Knopp 1 , A. Knopp 2 , F. Stellmacher 3 , E. Reusche 3 , M. Löning 4 , S. R. Kantelhardt 6 , H. von Domarus 5 , H. Arnold 5 , A. Giese 6
  • 1Department of Neurosurgery, University Hospital Lübeck, Lübeck, Germany
  • 2Department of Pediatrics, University Hospital Lübeck, Lübeck, Germany
  • 3Department of Pathology, University Hospital Lübeck, Lübeck, Germany
  • 4Department of Obstetrics and Gynecology, MHH, Hannover, Germany
  • 5Department of Craniofacial and Maxillary Surgery, University Hospital of Lübeck, Lübeck, Germany
  • 6Department of Neurosurgery, University of Goettingen, Goettingen, Germany
Further Information

Publication History

Publication Date:
03 February 2009 (online)

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Abstract

Basal encephaloceles in western countries occur in 1 of every 35 000–40 000 live births; with an incidence of less than 10% they are the least common of all encephaloceles. Certain subtypes such as transsphenoidal variants may be as rare as 1 in 700 000 live births. These rare encephaloceles are classified into five anatomic types: spheno-ethmodial, transsphenoidal, spheno-orbital, transethmoidal, and spheno-maxillary. Here we present an exceedingly rare variant of a non-midline basal encephalocele of the spheno-orbital type, which was treated by resection of the encephalocele, which contained dysplastic central nervous system tissue, on day four post partum. The patient had no neurological deficits and a six year follow-up showed a normal intellect and a good cosmetic result.

Zusammenfassung

Basale Encephalocelen treten in Europa oder Nordamerika bei einer von 35 000 bis 40 000 Geburten auf und stellen mit 10% die seltensten Encephalocelen dar. Bestimmte Typen, wie die transsphenoidalen Varianten, sind besonders selten und haben eine Häufigkeit von 1 auf 700 000 Lebendgeburten. Diese seltenen transsphenoidalen Varianten werden in fünf anatomische Typen klassifiziert, die spheno-ethmoidalen, transsphenoidalen, spheno-orbitalen, transethmoidalen und spheno-maxillären Encephalocelen. Hier stellen wir eine außerordentlich seltene Nicht-Mittellinien-Variante einer basalen Encephalocele des spheno-orbitalen Typs vor, die am vierten Tag post partum durch Resektion der Encephalocele und dysplastischen zentralnervösen Gewebes behandelt wurde. Bei exzellentem kosmetischem Ergebnis fanden sich keine neurologischen Defekte bei einer normalen kindlichen Entwicklung und normalem Intellekt über einen sechsjährigen Verlauf.

Key words

spheno-orbital encephalocele - basal encephalocele - skull base surgery - pre-natal diagnosis

Schlüsselwörter

Spheno-orbitale Encephalocele - Basale Encephalocele - Schädelbasischirurgie - pränatale Diagnose

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Correspondence

Prof. A. GieseMD 

Department of Neurosurgery

Georg-August-University

Robert-Koch-Str. 40

37075 Goettingen

Germany

Phone: +49/551/39 60 33

Fax: +49/551/39 87 94

Email: alf.giese@med.uni-goettingen.de