TY - JOUR
T1 - Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula
T2 - A case report with prenatal diagnosis
AU - Miyano, Go
AU - Morita, Keiichi
AU - Kaneshiro, Masakatsu
AU - Miyake, Hiromu
AU - Koyama, Mariko
AU - Nouso, Hiroshi
AU - Yamoto, Masaya
AU - Nakano, Reiji
AU - Tanaka, Yasuhiko
AU - Nishiguchi, Tomizo
AU - Kawamura, Takakazu
AU - Fukumoto, Koji
AU - Urushihara, Naoto
N1 - Copyright:
Copyright 2015 Elsevier B.V., All rights reserved.
PY - 2015/2/1
Y1 - 2015/2/1
N2 - We describe herein a case of unilateral pulmonary agenesis (PA) with oesophageal atresia (EA)/tracheoesophageal fistula (TEF) that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.
AB - We describe herein a case of unilateral pulmonary agenesis (PA) with oesophageal atresia (EA)/tracheoesophageal fistula (TEF) that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.
KW - Oesophageal atresia
KW - tracheoesophageal fistula
KW - unilateral pulmonary agenesis
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U2 - 10.4103/0189-6725.151000
DO - 10.4103/0189-6725.151000
M3 - Article
C2 - 25659560
AN - SCOPUS:84922848223
VL - 12
SP - 86
EP - 88
JO - African Journal of Paediatric Surgery
JF - African Journal of Paediatric Surgery
SN - 0189-6725
IS - 1
ER -