Ryuta Saka1, Dan Yamamoto2, Seika Kuroda3, Souji Ibuka3, Tasuku Kodama3, Toshimichi Hasegawa3,4. 1. Department of Pediatric Surgery, National Hospital Organization Fukuyama Medical Center, 4-14-17 Okinogamicho, Fukuyama, Hiroshima, 720-8520, Japan. saka@pedsurg.med.osaka-u.ac.jp. 2. Department of Obstetrics and Gynecology, National Hospital Organization Fukuyama Medical Center, 4-14-17 Okinogamicho, Fukuyama, Hiroshima, 720-8520, Japan. 3. Department of Pediatric Surgery, National Hospital Organization Fukuyama Medical Center, 4-14-17 Okinogamicho, Fukuyama, Hiroshima, 720-8520, Japan. 4. Department of Pediatric Surgery, Tottori University, Tottori, Japan.
Abstract
BACKGROUND: Congenital pyloric atresia (CPA) is a rare gastrointestinal anomaly frequently associated with epidermolysis bullosa (EB). Although the complications of familial isolated CPA are minor, delays in diagnosis can increase the chances of morbidity. CASE PRESENTATION: Three female infants born to a Japanese mother presented with CPA at birth. There was no consanguinity between the parents, and the spacing between pregnancies was 2 years in each case. All 3 siblings had a prenatal diagnosis of CPA owing to polyhydramnios and a dilated stomach, without dilatation of the rest of the gastrointestinal tract. All patients underwent reconstructive surgeries for establishing bowel continuity (Case 1, pyloromyotomy; Case 2, gastroduodenostomy in a diamond fashion; and Case 3, gastroduodenostomy in a side-to-side fashion) soon after birth. Their postoperative courses were uneventful, and they grew up healthily, without any complications. CONCLUSION: Fetal ultrasonography is useful for diagnosing CPA prenatally. Successful prenatal diagnosis can lead to timely intervention after birth.
BACKGROUND:Congenital pyloric atresia (CPA) is a rare gastrointestinal anomaly frequently associated with epidermolysis bullosa (EB). Although the complications of familial isolated CPA are minor, delays in diagnosis can increase the chances of morbidity. CASE PRESENTATION: Three female infants born to a Japanese mother presented with CPA at birth. There was no consanguinity between the parents, and the spacing between pregnancies was 2 years in each case. All 3 siblings had a prenatal diagnosis of CPA owing to polyhydramnios and a dilated stomach, without dilatation of the rest of the gastrointestinal tract. All patients underwent reconstructive surgeries for establishing bowel continuity (Case 1, pyloromyotomy; Case 2, gastroduodenostomy in a diamond fashion; and Case 3, gastroduodenostomy in a side-to-side fashion) soon after birth. Their postoperative courses were uneventful, and they grew up healthily, without any complications. CONCLUSION: Fetal ultrasonography is useful for diagnosing CPA prenatally. Successful prenatal diagnosis can lead to timely intervention after birth.
Authors: T Hasegawa; A Kubota; K Imura; M Inoue; S Hata; T Kobayashi; H Kamiya; N Suehara Journal: J Clin Ultrasound Date: 1993-05 Impact factor: 0.910