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http://20.193.157.4:9595/xmlui/handle/123456789/1357| Title: | Rare Case of Genital Malformation with Omphalocele, Exstrophy of Bladder, Imperforate Anus and Spinal Defect Complex-Autopsy Findings. |
| Authors: | Mamatha K, Yelikar BR, Varsha R Deshpande, BS Disha. |
| Keywords: | Extrophy of cloaca, Gestation, Placenta CASE REPORT A 22-year-old primigravida with 28 weeks of gestation visited in the second stage of labour. There was no significant antenatal history of exposure to teratogenic drugs, infection, diabetes, hypertension and exposure to radiation. Her married life was nine years and it was not consanguineous. There was no history of treatment for infertility. Her antenatal scan during 17 weeks revealed a single live foetus without congenital abnormalities. She delivered a live baby weighing 1.16 kg at 28 weeks of gestation with multiple congenital abnormalities. The baby died after 10 minutes. There was no history of similar malformations and death of a foetus with such similar malformations in the family. The dead foetus was sent for detailed autopsy examination. External examination showed the following: Foetus weighed 1.3 kg with attached placenta weighed 240 gm, crown-rump length – 19 cm, crown-heel length – 36 cm, head circumference – 29 cm, chest circumference – 19 cm. Placenta and umbilical artery showed normal histological features. Congenital malformations: • A large defect in the anterior abdominal wall (omphalocele) with widened umbilical ring with a circumference of 24 cm [Table/Fig-1] • Umbilical cord was inserted at the inferior border of the sac and splitting of the cord noted • The contents of the sac were liver, stomach, spleen, right kidney with attached bladder, left kidney, pancreas, loops of intestine with dilated distal loops filled with meconium, bilateral testis [Table/Fig-2] • External genitalia was not identified except two small ridges [Table/Fig-3] • Lumbosacral meningocele measuring 7 cm in diameter [Table/ Fig-3] • Imperforate anus with absence of genitelia – there was no anal opening in the perineal region [Table/Fig-4]. • Exstrophy of bladder - anterior wall of bladder was defective. Based on the presence of four cardinal anomalies diagnosis of OEIS complex was rendered. DISCUSSION OEIS is a rare complex in which clustering of many common |
| Issue Date: | 2017 |
| Publisher: | BLDE(Deemed to be University) |
| Abstract: | Omphalocele, Exstrophy of cloaca, Imperforate anus and Spinal defects (OEIS) is a severe manifestation of exstrophy-epispadias sequence with a combination of defects including OEIS. It results from improper closure of anterior abdominal wall and defective development of cloaca and urogenital septum due to defect in blastogenesis during the 4th week of gestation. Identification of this complex is important through foetal autopsy as this condition can recur in siblings. Prenatal diagnosis also helps to prevent foetal death with appropriate management in the less severe cases. In severe cases, termination of pregnancy is considered. A primigravida with 28 weeks of gestation had delivered a live baby with multiple congenital anomalies; baby died after 10 minutes. These anomalies were grouped under OEIS complex. Keywords |
| URI: | http://hdl.handle.net/123456789/1357 |
| Appears in Collections: | Faculty of Pathology |
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