Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com Images in Fetal Medicine Fetal Diagn Ther 2012;32:295–298 DOI: 10.1159/000341569 Encephalocystocele – Uncommon Diagnosis in Prenatal Medicine Robert Dankovcik b, c Vlasta Vyhnalkova d Stela Muranska c Eduard Kucera f Maria Korpova e Anna Plichtova a, e Maria Miklosova e Vladimir Ferianec a Jan Evangelista Jirasek g Marek Dudas e a Second Department of Obstetrics and Gynecology, Comenius University and University Hospital, Bratislava, b Second Department of Obstetrics and Gynecology, P. J. Safarik University and L. Pasteur University Hospital, c Center for Prenatal Diagnosis, s.r.o., d Department of Forensic Medicine and Pathology, Health Care Surveillance Authority of Slovak Republic, and e Fetal Medicine Program, Department of Cell Biology, P. J. Safarik University, Kosice, Slovakia; f Department of Gynecology and Obstetrics, Third Faculty of Medicine, Charles University in Prague, and g Institute for Care of Mother and Child, Prague, Czech Republic Clinical Case 1 Unilateral Encephalocystocele in an Otherwise Normal Brain A single live fetus [gestation week (GW) 18; healthy mother/ age 29, gravida 3, para 2] affected with prominent dilatation of the left lateral ventricle and atrophic brain tissue was identified by sonography ( fig. 1a, b). MRI in GW 24 revealed parieto-occip- ital encephalocystocele ( fig. 1 c, d). Cesarean section at GW 38 delivered a full-term newborn girl, eutrophic, 4,020 g, 51 cm, APGAR score 7/6, normal motorics, head circumference 44 cm. No malformations of the heart, kidney or other organs were found on sonography; small amounts of milk were ingested per os. The soft parieto-occipital protrusion ( fig. 1 e, f) showed no cerebrospinal fluid leakage or bleeding. Dyspnea and cyanosis developed 3.5 h postpartum; oxygen inhalation restored blood saturation to 99%, but inoperability was stated by a neurosur- geon. Sudden apnea and bradycardia appeared 5.5 h later, fol- lowed by asystolia after another hour. Autopsy revealed a largely normal brain with a normal left frontal lobe and a normal ante- rior portion of the left lateral ventricle. The expanded posterior part of the left lateral ventricle was covered in thinned differenti- ated brain tissue and herniated via the distended fontanella oc- cipitalis ( fig. 1g, h). Key Words Cephalocele  Encephalocystocele  Meningocele Abstract Encephalocystocele is a developmental malformation char- acterized by brain herniation accompanied with extracranial cystic protrusion of the ventricular system. This nosological unit is often overlooked and insufficiently classified merely as encephalocele. Herein, two exceptionally clear cases of the parieto-occipital cranioschisis with encephalocystocele and congenital hydrocephalus of the lateral ventricles are documented with 2-dimensional/3-dimensional sono- graphic images and the corresponding MRI findings. In both cases, prenatal diagnosis was confirmed by autopsy. Copyright © 2012 S. Karger AG, Basel Received: June 17, 2012 Accepted after revision: June 26, 2012 Published online: October 24, 2012 Assoc. Prof. Marek Dudas, MD, PhD Fetal Medicine Program, Department of Cell Biology P. J. Safarik University Moyzesova 11, SK–04001 Kosice (Slovakia) Tel. +421 90 551 3578, E-Mail marek.dudas.sk  @  gmail.com © 2012 S. Karger AG, Basel 1015–3837/12/0324–0295$38.00/0 Accessible online at: www.karger.com/fdt