title: Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis.
abstract: 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.
authors: Department of Pediatric Surgery, Shizuoka Children's Hospital, Shizuoka, Japan.
authors: Go
authors: G
authors: Miyano
authors: Keiichi
authors: K
authors: Morita
authors: Masakatsu
authors: M
authors: Kaneshiro
authors: Hiromu
authors: H
authors: Miyake
authors: Mariko
authors: M
authors: Koyama
authors: Hiroshi
authors: H
authors: Nouso
authors: Masaya
authors: M
authors: Yamoto
authors: Reiji
authors: R
authors: Nakano
authors: Yasuhiko
authors: Y
authors: Tanaka
authors: Tomizo
authors: T
authors: Nishiguchi
authors: Takakazu
authors: T
authors: Kawamura
authors: Koji
authors: K
authors: Fukumoto
authors: Naoto
authors: N
authors: Urushihara
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000601:surgery; D000328:Adult; D003937:Diagnosis, Differential; D004933:Esophageal Atresia / Q000175:diagnosis* / Q000601:surgery; D004946:Esophagostomy; D005260:Female; D005774:Gastrostomy; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities* / Q000601:surgery; D008171:Lung Diseases / Q000175:diagnosis* / Q000601:surgery; D011247:Pregnancy; D013902:Radiography, Thoracic; D013908:Thoracotomy; D014138:Tracheoesophageal Fistula / Q000175:diagnosis* / Q000601:surgery; D016216:Ultrasonography, Prenatal