Alveolar Capillary Dysplasia Associated with Omphalocoele

Alveolar capillary dysplasia (ACD) is a rare and universally fatal form of persistent pulmonary hypertension of the newborn (PPHN). We report a case of a newborn male baby with a moderate sized omphalocoele who developed respiratory distress 10 hours after birth. He deteriorated and succumbed shortly afterwards despite maximal ventilatory support and inhaled nitric oxide. ACD was diagnosed at postmortem examination, and the association of ACD with omphalocoele and gut malrotation has not been reported. We suggest including ACD in the differential diagnosis of refractory PPHN in a newborn even with the presence of other risk factors for respiratory failure.

[1]  P. Stankiewicz,et al.  Alveolar capillary dysplasia. , 2011, American journal of respiratory and critical care medicine.

[2]  C. Langston,et al.  Profound hypoxemia and pulmonary hypertension in a 7-month-old infant: Late presentation of alveolar capillary dysplasia , 2008, Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.

[3]  R. Boldrini,et al.  ABCA3 mutation and pulmonary hypertension: a link with alveolar capillary dysplasia? , 2008, The Journal of pediatrics.

[4]  C. Chow,et al.  A neonate with coexisting congenital cystic adenomatoid malformation of the lung and alveolar capillary dysplasia: A case report with review of literature , 2008, Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.

[5]  A. Nicholson,et al.  Capillary apposition and density in the diagnosis of alveolar capillary dysplasia , 2006, Histopathology.

[6]  G. Kayser,et al.  A Rare Case of Aortic Coarctation and Ventricular Septal Defect Combined with Alveolar Capillary Dysplasia , 2007, Pediatric Cardiology.

[7]  A. Haque,et al.  Late presentation of alveolar capillary dysplasia in an infant , 2006, Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.

[8]  L. Spitz,et al.  CONGENITAL ALVEOLAR CAPILLARY DYSPLASIA AND ASSOCIATED GASTROINTESTINAL ANOMALIES , 2006, Fetal and pediatric pathology.

[9]  Thowfique Ibrahim,et al.  Persistent pulmonary hypertension of newborn due to congenital capillary alveolar dysplasia , 2005, Pediatric pulmonology.

[10]  D. Tibboel,et al.  Alveolar capillary dysplasia: a logical approach to a fatal disease. , 2005, Journal of pediatric surgery.

[11]  A. Sihoe,et al.  Alveolar Capillary Dysplasia with Congenital Misalignment of Pulmonary Vessels , 2005, Asian cardiovascular & thoracic annals.

[12]  B. Bejjani,et al.  Expanding the phenotype of alveolar capillary dysplasia (ACD). , 2004, The Journal of pediatrics.

[13]  L. Storme,et al.  Alveolar capillary dysplasia: a cause of persistent pulmonary hypertension of the newborn , 2002, European Journal of Pediatrics.

[14]  H. Rigatto,et al.  Alveolar capillary dysplasia. Report of a case of prolonged life without extracorporeal membrane oxygenation (ECMO) and review of the literature. , 2000, Early human development.

[15]  S. Hintz,et al.  Alveolar Capillary Dysplasia: Diagnostic Potential for Cardiac Catheterization , 1999, Journal of Perinatology.

[16]  R. Winter,et al.  Familial persistent pulmonary hypertension of the newborn resulting from misalignment of the pulmonary vessels (congenital alveolar capillary dysplasia). , 1998, Journal of medical genetics.

[17]  N. Finer,et al.  Inhaled nitric oxide enhances oxygenation but not survival in infants with alveolar capillary dysplasia. , 1997, The Journal of pediatrics.

[18]  K. To,et al.  Congenital misalignment of pulmonary vessels: an unusual syndrome associated with PPHN , 1995, Acta paediatrica.