Feeding Difficulties in a Newborn with a Cardiac Anomaly: More Than Just the Heart
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CLINICAL CONUNDRUM
Feeding Difficulties in a Newborn with a Cardiac Anomaly: More Than Just the Heart Anna M. Banc‑Husu1 · Peter T. Osgood1 · John E. Fortunato Jr.1 Received: 31 March 2020 / Accepted: 2 June 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Case Presentation Patient A was born at 39 weeks gestation (birth weight 3.2 kg) and diagnosed on day of life (DOL) #1 with double outlet right ventricle with Tetralogy of Fallot on echocardiogram following auscultation of murmur on newborn exam. She was discharged home on DOL#3 on breastmilk PO ab lib feedings with formula supplementation. She was tolerating oral feeds without emesis but was noted by parents to take up to 1 h to complete a feeding. On DOL#7, she developed cyanotic episodes while sleeping, which prompted urgent evaluation in the emergency room. She was admitted to pediatric cardiology for further evaluation and a cardiac computed tomography (CT) to assess for a cardiac cause of her cyanosis demonstrated an incidental finding of a patulous, distended esophagus with layering of fluid along the inferior and posterior esophagus (Fig. 1a). An esophagram performed on DOL#8 confirmed the presence of a dilated patulous esophagus with stasis and delayed transit through the gastroesophageal junction (GEJ) (Fig. 1b). Given these findings, an unsedated high-resolution esophageal manometry (HRM) study was performed on DOL #14 using a 6-French solid state 36-channel catheter via a transnasal approach. 20 swallows were analyzed using a bottle containing either Pedialyte or formula. Findings included impaired upper esophageal sphincter (UES) relaxation (median UES relaxation [UESR] 26 mmHg, normal ≤ 12 mmHg) [1], * John E. Fortunato Jr. [email protected] Anna M. Banc‑Husu [email protected] Peter T. Osgood [email protected] 1
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Ann & Robert H. Lurie Children’s Hospital of Chicago, 225 E. Chicago Ave, Box 65, Chicago, IL 60611, USA
failed esophageal body contraction with all 20 swallows, and absence of lower esophageal sphincter (LES) relaxation (mean integrated relaxation pressure over 4 s [IRP4] 26 mmHg, normal ≤ 15 mmHg) (Fig. 1c). The patient was made strictly nil per os (NPO) and started on supplemental feeds via nasogastric (NG) tube. What is the diagnosis?
Answer Based the Chicago Classification (v3.0, 2015), the patient met manometric criteria for type 1 achalasia with 100% failed peristalsis, impaired LES relaxation, and absence of pan-esophageal pressurization [2]. Given the patient’s age and co-morbidity of congenital heart disease, consideration of surgical myotomy for possible achalasia was deferred. She was followed closely as an outpatient and remained on NG feeds. She underwent surgical correction of her underlying congenital heart disease at 4 months of age, had a gastrostomy tube (GT) placed, and was progressively started on small volumes of pureed fee
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