Basal meningoencephalocele is frequently associated with midfacial anomaly, optic disc anomaly, brain anomaly, cerebrospinal fluid rhinorrhea, chiasma syndrome, and endocrinologic disturbance. The combination of basal meningoencephalocele and moyamoya disease is extremely rare. A 29-year-old man had basal meningoencephalocele (transsphenoidal type), anomaly of the optic disc (morning glory syndrome), panhypopituitarism and moyamoya disease. The patient was treated by hormone replacement, but surgical intervention was not required. Basal meningoencephalocele and moyamoya disease are a possible combination of the diseases.

Ingraham FD, Swan H: Spina bifida and cranium bifidum. I. A survey of five hundred and forty-six cases. N Engl J Med 1943;228:559–563.
Gisselsson L: Intranasal forms of encephalomeningocele. Acta Otolaryngol 1947;35:519–531.
Pollock JA, Newton TH, Hoyt WF: Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases. Radiology 1968;90:442–453.
Goldhammer Y, Smith JL: Optic nerve anomalies in basal encephalocele. Arch Ophthalmol 1975;93:115–118.
Lieblich JM, Rosen SW, Guyda H, Reardan J, Schaaf M: The syndrome of basal encephalocele and hypothalamic-pituitary dysfunction. Ann Intern Med 1978;89:910–916.
Ellyin F, Khatir AH, Singh SP: Hypothalamic-pituitary functions in patients with transsphenoidal encephalocele and midfacial anomalies. J Clin Endocrinol Metab 1980;51:854–856.
Bullard DE, Crockard HA, McDonald WI: Spontaneous cerebrospinal fluid rhinorrhea associated with dysplastic optic discs and a basal encephalocele. Case report. J Neurosurg 1981;54:807–810.
Koenig SB, Naidich TP, Lissner G: The morning glory syndrome associated with sphenoidal encephalocele. Ophthalmology 1982;89:1368–1373.
Caprioli J, Lesser RL: Basal encephalocele and morning glory syndrome. Br J Ophthalmol 1983;67:349–351.
Smith DE, Murphy MJ, Hitchon PW, Babin RW, Abu-Yousef MM: Transsphenoidal encephaloceles. Surg Neurol 1983;20:471–480.
Yokota A, Matsukado Y, Fuwa I, Moroki K, Nagahiro S: Anterior basal encephalocele of the neonatal and infantile period. Neurosurgery 1986;19:468–478.
Bakri SJ, Siker D, Masaryk T, Luciano MG, Traboulsi EI: Ocular malformations, moyamoya disease, and midline cranial defects: A distinct syndrome. Am J Ophthalmol 1999;127:356–357.
Suwanwela C, Suwanwela N: A morphological classification of sincipital encephalomeningoceles. J Neurosurg 1972;36:201–211.
Kindler P: Morning glory syndrome: Unusual congenital optic disk anomaly. Am J Ophthalmol 1970;69:376–384.
Massaro M, Thorarensen O, Liu GT, Maguire AM, Zimmerman RA, Brodsky MC: Morning glory disc anomaly and moyamoya vessels. Arch Ophthalmol 1998;116:253–254.
Hanson MR, Price RL, Rothner AD, Tomsak RL: Developmental anomalies of the optic disc and carotid circulation. A new association. J Clin Neuroophthalmol 1985;5:3–8.
Kobayashi S, Miyazaki M, Miyagi O, Yodonawa M, Shimizu S: A case of transsphenoidal meningoencephalocele. No Shinkei Geka 1990;18:1065–1070.
Sternberg H: Zur formalen Genese der vorderen Hirnbrüche (Encephalomeningocele anterior). Wien Med Wochenschr 1929;79:462–466.
Danoff D, Serbu J, French LA: Encephalocele extending into the sphenoid sinus. Report of a case. J Neurosurg 1966;24:684–686.
Wiese GM, Kempe LG, Hammon WM: Transsphenoidal meningohydroencephalocele. Case report. J Neurosurg 1972;37:475–478.
Ikeda H, Sasaki T, Yoshimoto T, Fukui M, Arinami T: Mapping of a familial moyamoya disease gene to chromosome 3p24.2–p26. Am J Hum Genet 1999;64:533–537.
Komiyama M, Nakajima H, Nishikawa M, Yasui T, Kitano S, Sakamoto H, Fu Y: High incidence of persistent primitive arteries in moyamoya and quasi-moyamoya diseases. Neurol Med Chir (Tokyo) 1999;39:163–167.
Rice JF, Eggers DM: Basal transsphenoidal encephalocele: MR findings. Am J Neuroradiol 1989;10:S79.
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.