Document Type : case report
Authors
1 MD, FIAE, Professor, Deptt. Of Pediatrics, Department of Pediatrics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Sawangi Meghe, Wardha, Maharashtra, India
2 MBBS, Resident, Deptt. Of Pediatrics, Department of Pediatrics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Sawangi Meghe, Wardha, Maharashtra, India
3 MD, Head of Department, Deptt. Of Pediatrics, Department of Pediatrics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Sawangi Meghe, Wardha, Maharashtra, India
4 MD, Associate Professor, Deptt. Of Pediatrics, Department of Pediatrics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Sawangi Meghe, Wardha, Maharashtra, India
Abstract
Unilateral Pulmonary aplasia with absent right pulmonary artery is a rare disorder, caused by the developmental arrest of primitive lung during embryonic life. It’s a complete absence of lung parenchyma vasculature and its main bronchus. The most common variant consists of carina and main stump bronchial stump with the absence of distal lung. It is frequently associated with many other congenital disorders like VACTERL syndrome (V-vertebral anomalies, A-anal atresia, C- cardiovascular anomaly, T- tracheosophageal fistula, E- esophageal atresia R-renal anomalies L- limb defects) and cardiac anomaly. These patients usually present with respiratory distress. Here we are presenting a case of a 2-month-old male infant suffering from unilateral lung aplasia with absent right pulmonary artery.
Keywords
- Boyden EA. Developmental anomalies of the lungs. The American Journal of Surgery. 1955 Jan 1; 89(1):79-89.
- Mohamed KM, William LN. Agenesis of the lung: report of four patients with unusual anomalies. Chest. 1985 Apr 1; 87(4):522-7.
- Pool PE, Vogel JH, Blount Jr SG. Congenital unilateral absence of a pulmonary artery: the importance of flow in pulmonary hypertension. The American journal of cardiology. 1962 Nov 1; 10(5):706-32.
- De Pozze S, Brescia MA, Amermon EE, Sharma KK. Agenesis of the left lung. Arch Pediatr. 1960; 77:485-90.
- Krammoh EK, Bigras JL, Prsa M, Lapierre C, Miró J, Dahdah NS. Therapeutic strategies in children with an isolated unilateral absent proximal pulmonary artery. Pediatric cardiology. 2010 Jul; 31(5):607-10.
- Finda JD, Michelson PH. Congenital disorders of the lung. In: Behrman RE, Kliegman RM, JensonHB editors. Nelson Textbook of Pediatrics. 18th Ed.Philadelphia: Saunders; 2008.
- Deutsch MA, Thieme SF, Hinterseer M, Johnson TR, Pfosser A, Reichart B, Nikolaou K, Behr J. Adult presentation of combined unilateral atresia of the right proximal pulmonary artery and left patent ductus arteriosus: case report and embryological considerations.
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