Background/Aims: Cephaloceles are common malformations of the central nervous system. However, the great majority of clinical experiences in large occipitocervical cephaloceles have not been reported previously. The purpose of this report is to investigate the pathogenetic factors involved in the development of cranial dysraphism and to analyze the clinical and pathological factors that influence the outcome in patients. Methods: Three hundred and twenty infants with craniospinal dysraphism and 12 with large occipitocervical cephaloceles were admitted to our institution in a 10-year interval between 1995 and 2005. Infants with cephaloceles, including newborns and 5 within the first year of life, were all operated by two authors in our institution, and they were analyzed retrospectively. Results: The sex predilection was limited to occipitocervical cephaloceles, where 8 of the 12 cases were females. Hydrocephalus was present in 25% of the patients at the time of diagnosis. Clinical presentation was most often consistent with hydrocephalus, focal neurological findings being a less prominent feature. Associated congenital anomalies were present in 50% of the children. Contemporary neuroimaging techniques including computed tomography and magnetic resonance imaging facilitated the diagnosis. Patients were initially managed by posterior fossa or cervical region exploration, followed by sac excision. Three cases died in the early postoperative period, and the surgical mortality in this series was 25%. They were the ones with the largest encephaloceles and microcephaly. The mean follow-up time was 3.4 years. The overall mortality rate for the whole series was 33.3% (4/12). The size of the cephaloceles and the presence or absence of neural tissue in the sac largely determines the outcome for patients with occipitocervical cephaloceles. Conclusion: Infants with lesions greater than 50 mm in diameter, containing a significant amount of neural tissue, have an extremely poor prognosis, especially if associated with microcephaly.

1.
Bozinov O, Tirakotai W, Sure U, Bertalanffy H: Surgical closure and reconstruction of a large occipital encephalocele without parenchymal excision. Childs Nerv Syst 2005;21:144–147.
2.
Erdincler P, Kaynar MY, Canbaz N, Kocer N, Kuday C, Ciplak N: Iniencephaly: neuroradiological and surgical features. Case report review of the literature. J Neurosurg 1998;89:317–320.
3.
Maelay J Jr, Dzenitis AJ, Hockey AA: The prognosis of encephaloceles. J Neurosurg 1970;32:209–218.
4.
Rosenfeld JV, Watters DAK: Hydrocephalus and congenital abnormalities; in Rosenfeld JV, Waters DAK (eds): Neurosurgery in Tropics. London, Macmillan, 2000, p 12.
5.
Salamo JF: Cervical meningocele in association with spinal abnormalities. Childs Nerv Syst 2005;21:4–5.
6.
Chapman PH, Swearingen B, Caviness VS: Subtorcular occipital encephaloceles. Anatomical considerations relevant to operative management. J Neurosurg 1989;71:375–381.
7.
Creighton RE, Relton JES, Meridy HW: Anaesthesia for occipital encephalocele. Can Anaesth Soc J 1974;21:403–406.
8.
Hoving E, Blaster S, Kelly E, Rutka JT: Anatomical and embryological considerations in the repair of the large vertex cephalocele. Case report. J Neurosurg 1999;90:537–541.
9.
Kojima T, Waga S, Shimizu T, Sakakura T: Dandy-Walker cyst associated with occipital meningocele. Surg Neurol 1982;17:52–56.
10.
Lorber J: The prognosis of occipital encephalocele. Dev Med Child Neurol 1967(suppl 13):75–86.
11.
Mahapatra AK, Gupta PK, Dev EJ: Posterior fontanelle giant encephhalocele. Pediatr Neurosurg 2002;36:40–43.
12.
Steinbok P, Cochrane DD: Cervical spinal dysraphism. J Neurosurg 1995;83:569–570.
13.
Simpson DA, David DJ, White J: Cephaloceles: treatment, outcome, and antenatal diagnosis. Neurosurgery 1984;15:14–21.
14.
Amato OA: Posterior encephalocele: a case of difficult tracheal intubation. West Afr J Med 1994;13:187–190.
15.
Caviness VS Jr, Evrard P: Occipital encephalocele. A pathological and anatomic analysis. Acta Neuropathol 1975;32:245–255.
16.
Yokota A, Kajiwara H, Kochi M, Fuwa I, Wada H: Parietal cephalocele: clinical importance of its atretic form and associated malformations. J Neurosurg 1988;69:545–551.
17.
Wong JH, Wong GK, Zhu XL, Chan YL, Fung E, Poon WS: Cervical meningocele with tethered cervical cord in a Chinese infant. Hong Kong Med J 2005;11:113–115.
18.
Walsh M, Graupman P: Meckel-Gruber syndrome in association with an occipital meningocele. Pediatr Neurosurg 2006;42:333–337.
19.
Sutton JB: The lateral recess of the fourth ventricle: their relation to certain cysts and tumors of the cerebellum, and to occipital meningocele. Brain 1887;9:352–361.
20.
Bindal AK, Storrs BB, McLone DG: Occipital meningoceles in patients with the Dandy-Walker syndrome. Neurosurgery 1991;28:844–847.
21.
Todo T, Usui M, Araki F: Dandy-Walker syndrome forming a giant occipital meningocele. Neurol Med Chir (Tokyo) 1993;33:845–850.
22.
Kojima T, Waga S, Shimizu T, Sakakura T: Dandy Walker cyst associated with occipital meningocele. Surg Neurol 1982;17:52–56.
23.
Tubbs RS, Wellons JC 3rd, Oakes WJ: Occipital encephalocele, lipomeningomyelocele, and Chiari I malformation: case report and review of the literature. Childs Nerv Syst 2003;19:50–53.
24.
Moorthy RK, Rajshekhar V: Management of hydrocephalus associated with occipital encephalocoele using endoscopic third ventriculostomy: report of two cases. Surg Neurol 2002;57:351–355.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.