Hypertrophic pyloric stenosis following persistent pulmonary hypertension of the newborn: a case report and literature review

• Published in: BMC pediatrics Vol. 18; no. 1; p. 290
• Main Authors: Iijima, Shigeo, Ueno, Daizo, Baba, Toru, Ohishi, Akira
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 03.09.2018; BioMed Central; BMC
• Subjects: Abdomen; Age; Antihypertensive Agents - therapeutic use; Arginine; Atropine - therapeutic use; Bosentan - therapeutic use; Bronchodilator Agents - therapeutic use; Case Report; Case reports; Complications and side effects; Diagnosis; Female; High-Frequency Ventilation; Humans; Hypertrophic pyloric stenosis; Infant, Newborn; Literature reviews; Meconium Aspiration Syndrome - complications; Meconium Aspiration Syndrome - therapy; Muscarinic Antagonists - therapeutic use; Neonate; Newborn babies; Nitric oxide; Nitric Oxide - therapeutic use; Nitric Oxide Donors - therapeutic use; Nitroglycerin - therapeutic use; Pathogenesis; Pediatric diseases; Persistent Fetal Circulation Syndrome - complications; Persistent Fetal Circulation Syndrome - etiology; Persistent Fetal Circulation Syndrome - therapy; Persistent pulmonary hypertension of the newborn; Pulmonary hypertension; Pyloric stenosis; Pyloric Stenosis, Hypertrophic - drug therapy; Pyloric Stenosis, Hypertrophic - etiology; Retina; Risk factors; Sildenafil Citrate - therapeutic use; Vasodilator Agents - therapeutic use; Vomiting; United States > US; Hypertrophic pyloric stenosis; Neonate; Arginine; Persistent pulmonary hypertension of the newborn; Nitric oxide; Pulmonary hypertension
• ISSN: 1471-2431; 1471-2431
• DOI: 10.1186/s12887-018-1270-0

Although persistent pulmonary hypertension of the newborn (PPHN) and infantile hypertrophic pyloric stenosis (HPS) are both well-known diseases that occur in early infancy, PPHN complicated by HPS is rare. As nitric oxide (NO) is an important mediator of biological functions, on both the vascular endothelium and smooth muscle cells, the decreased production of NO might play a role in the pathogenesis of both PPHN and HPS. We present the case of a neonate who developed HPS following PPHN, including a detailed review on research published to date, and we discuss the pathogenesis of PPHN and HPS. A female neonate born at 38 weeks of gestation, weighing 3140 g, developed PPHN due to meconium aspiration syndrome. Intensive treatment with high frequency oscillations and inhaled NO were initiated, and sildenafil and bosentan were added. She gradually recovered. At 15 days of age, the patient developed recurrent vomiting after feeding and the diagnosis of HPS was made. Intravenous atropine therapy was started at 20 days of age, but the efficacy was clinically unsatisfactory. The coadministration with transdermal nitroglycerin improved the symptoms, and oral feeding was successfully re-introduced. Our patient recovered from both PPHN and HPS using NO-related medications. A decrease in NO synthesis is likely to be a common pathway for PPHN and HPS.