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GYNAECOLOGY AND OBSTETRICS IMAGING Table of Contents   
Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 789-791
Antenatal ultrasound diagnosis of occipital meningoencephalocele-A case report


Department of Radio-diagnosis, Rural Medical College, Loni, Tal.Rahata, Dist.Ahmednagar. Pin-413736, India

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Date of Submission30-Sep-2006
Date of Acceptance10-Nov-2006
 

Keywords: Ultrasound, Antenatal diagnosis, Meningoencephalocele, Occipital

How to cite this article:
Chougule S R, Desai S M, Aironi V D. Antenatal ultrasound diagnosis of occipital meningoencephalocele-A case report. Indian J Radiol Imaging 2006;16:789-91

How to cite this URL:
Chougule S R, Desai S M, Aironi V D. Antenatal ultrasound diagnosis of occipital meningoencephalocele-A case report. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:789-91. Available from: http://www.ijri.org/text.asp?2006/16/4/789/32349

   Introduction Top


Antenatal ultrasonogrphy is a valuable diagnostic tool in detection of various fetal congenital anomalies. Early detection of anomalies helps in termination of pregnancy and prevents birth of defective fetus in the society. The congenital anomalies are common in the region of head, neck and spine. We describe one such illustrative case of a lethal anomaly.


   Case report Top


A twenty four year old woman patient came with five months amenorrhea for a routine antenatal check up without any previous ultrasonography examinations.

On ultrasonographic examination, there was a large skull defect postero-inferiorly with protrusion of the intracranial contents (meninges, CSF and brain parenchyma) in the occipital region. [Figure - 1],[Figure - 2].

The fetal spine was however normal. [Figure - 3].

The fetal movements, cardiac activity and rest of the fetal organs including kidneys were normal.

The above findings suggested the diagnosis of occipital meningoencephalocele.

The pregnancy was terminated immediately. The dead fetus showed findings described on the antenatal study and confirmed our diagnosis.

The plain radiograph (lateral view) of the abortus demonstrates no spinal abnormality. A defect is seen in the occipital region with overlying translucent soft tissue opacity. [Figure - 4].

The CT scan of the brain (limited axial sections) of the abortus also confirmed the bony defect and herniated brain parenchyma. [Figure - 5].

FIG.6 showing gross specimen of aborted fetus


   Discussion Top


These are uncommon neural tube defects occurring 1-4 times per 10,000 live births [1]. The cervical defects are much less common. Encephalocele is a bony defect in the skull, usually midline with protrusion of the intracranial contents. There is herniation of cerebral tissue and meninges. Approximately 75% are occipital, 13% frontal and 12% occur in parietal region [2] . Rare sites are protrusions through base of skull, orbits, nose or mouth [2].

Occipital encephaloceles have been diagnosed on ultrasound from about 9 weeks. After cranial ossification at 10 weeks, the skull defect and occipital sac may be demonstrated. In 30% cases there is associated spina bifida [1].

Sonographic diagnostic features include a juxtacranial mass that moves with head and in most instances a bony defect. If the mass appears cystic, the meningocele component predominates, while a solid mass indicates predominantly encephalocele [1]. In our case, both solid and cystic components were present indicating meningoencephalocele. When such abnormalities are found, the intracranial anatomy should be thoroughly evaluated, particularly for hydrocephalus, and spine should be examined for spina bifida. The fetal kidneys should be examined to exclude Meckel-Gruber syndrome (encephalocele, polycystic kidneys, polydactyly) [1].

The pathologic specimen in this case demonstrates a cranial defect and a large complex mass extending from occiput containing brain, cerebrospinal fluid and meninges.[Figure - 6] The alpha-fetoprotein was normal since the neural defect was 'closed', emphasizing the importance of ultrasound in establishing the diagnosis [1],[3].

Differential considerations with an anterior mass include teratoma (if the lesion is predominantly solid) and cystic hygroma (if it is predominantly cystic with internal septations). With a posterior mass, the possibilities include cystic hygroma, teratoma, iniencephaly, scalp edema, branchial cleft cyst, hemangioma [1],[2]. In cystic hygroma, the pattern of septa is usually characteristic [4],[5] .

The prognosis of encephalocele depends on location of lesion, amount of brain herniation and associated anomalies. Mortality can be upto 44%, and in survivors, intellectual impairment ranges from 40-91% [2]. Early detection offers prompt termination of pregnancy.


   Conclusion Top


Ultrasonography plays an exceedingly significant role in establishing fetal CNS anomalies as most of them are either lethal or disabling either due to the anomaly itself or due to commonly associated anomalies especially when screening laboratory tests like alpha-feto protein levels are within normal limits. A timely examination helps in early termination of such unwanted pregnancies.

 
   References Top

1.Sandler MA, Beute GH, Madrazo BL, Hudak SF, Walter R, Haggar AH, Maywood CM. Ultrasound case of the day. Occipital meningoencephalocele. Radiographics 1986; Nov.6(6):1096-9.  Back to cited text no. 1    
2.Ants Toi, The fetal head and brain, Rumack : Diagnostic ultrasound 3rd edition, 2005, Volume 2, ,37:1247.  Back to cited text no. 2    
3.Shaff MI, Blumenthal B, Coetzee M: Meningo-encephalocoele: prepartum ultrasonic and feto-amniographic findings. Br.J Radiol 1977 october 50:754-757.  Back to cited text no. 3    
4.Pearce JM, Griffin D, Campbell S: The differential prenatal diagnosis of cystic hygromata and encephalocele by ultrasound examination. J Clin.Ultrasound 1985 June (Medline).  Back to cited text no. 4    
5.Lieb W : Congenital meningoencephalocele and its differential diagnosis. Fortschr Ophthalmol 1987 (Medline).  Back to cited text no. 5    

Top
Correspondence Address:
S R Chougule
Department of Radio-diagnosis, Rural Medical College, A/P Loni, Tal-Rahata, Dist-Ahmednagar.Pin-413736
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32349

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]



 

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    Introduction
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    Discussion
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    References
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