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GYNAECOLOGICAL AND OBSTETRIC IMAGING Table of Contents   
Year : 2004  |  Volume : 14  |  Issue : 3  |  Page : 265-266
Prenatal diagnosis of iniencephaly on ultrasound


Department of Radiology and Obst and Gynecology, Lady Hardinge Medical College and Sucheta Kriplani Hospital, New Delhi, India

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Keywords: Iniencephaly, USG

How to cite this article:
Hemal U, Solanki R S, Varsheney A, Baliga S. Prenatal diagnosis of iniencephaly on ultrasound. Indian J Radiol Imaging 2004;14:265-6

How to cite this URL:
Hemal U, Solanki R S, Varsheney A, Baliga S. Prenatal diagnosis of iniencephaly on ultrasound. Indian J Radiol Imaging [serial online] 2004 [cited 2018 Nov 18];14:265-6. Available from: http://www.ijri.org/text.asp?2004/14/3/265/28599
Iniencephaly rarely has been reported prenatally. This is a fatal neural tube defect involving occiput and inions combined with rachischiasis of the cervical and thoracic spine with reteroflexion of the head.

A 27 year old, married for last seven years conceived and advised ultrasound at the end of 16 weeks of amenorrhoea. There was no history of consanguinity.

Ultrasonography was done using sonoline with 3.5 MHZ linear - transducer. The scan revealed short and rotated spine, with head fixed in retroflexion, facial structure seen clearly and showed grotesque appearance. This was constant observation even after repeat scan after an hour and on next day. The head was larger than trunk and abdomen, disproportionately. There were moderate dilatations of ventricle seen with thin rim of cerebral poarenchyma. The head was in fixed hyperextension and no neck region was visualized as immediately trunk was visualized, which appeared narrower. Femur length was 25 mm suggestive of 17 wks. Liquor amount was more for period of gestation.

Diagnosis of Iniencephaly was made and the pregnancy terminated. Foetus wt. Was 1.2 kg, pathologic specimen was examined, foetus was female showed fused occiput with upper part of trunk. Back showed black area in mid line with excessive presence of hair. Head was disproportionately larger.

No synonyms, prevalence 0.1 - 10:10,000, M:1 F:9, etiology is unknown, prognosis is universally fatal, recurrence risk - 1.4% differential diagnosis - klippel feil or anencephaly with spinal retroflexion. Associated anomalies includes - Agnathia, cephalocele, microstomia, palatal anteversion, spinabifida, omphalocele, gastroschisis, diaphragmatic hemia, pulmonary agenesis. The diagnosis of Inencephaly is established when occipital bone defect, cervical dysraphism and fixed retroflexion of fetal head seen[3]. Five rare case with long term survival have been reported and surgical correction of this deformity has also been described[4].



 
   References Top

1.NyBerg David A - Diagnostic ultrasound of fetal anomalies: Text of Atlas - The spine and neural tube defect, 160-161 - mosby year book.  Back to cited text no. 1    
2.Foderaro AE, A bu-Yousef MM, Bendi JA, et al: Antenatal ultrasound diagnosis of Intencephaly. J Clin Ultrosound 1987;15:550-554.  Back to cited text no. 2    
3.Solang Sahid, Waldo Seputueda, Vigor DeZerega, Jorge Gutierrez, Luis Rodriguez and Edgardo Corral. Iniencephaly: Prenatal diagnosis and management prenat. Dign. 2000:20-205.  Back to cited text no. 3    
4.Christine K. C. Loo. Brian Freeman and Dorotu standford - CNS Findings in Iniencephaly:Care report and literature review pathology 2001, 33. pp.112-115.   Back to cited text no. 4    

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Correspondence Address:
U Hemal
E-90, Ansari Nagar(East), AIIMS Campus, New Delhi-110 029
India
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Source of Support: None, Conflict of Interest: None


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



 

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