Hydrops Fetalis and Congenital Pulmonary Capillary Haemangiomatosis in a Premature Infant - A Case Report and Literature Review

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Abstract

Pulmonary capillary haemangiomatosis (PCH) is a rare disorder of the lung, well described in adult literature. PCH is characterized by capillary proliferation, infiltrating the interstitium and alveolar walls. This leads to development of respiratory distress and to end-stage pulmonary hypertension. Mostly young adults are affected. The affection of newborn is described in less than ten cases in literature in the past forty years. PCH is a mostly deadly ending disease. We present a preterm born infant with antepartal diagnosed hydrops fetalis, who died 30 minutes after birth. Autopsy revealed PCH as lethal reason and not cardial disease as presumed before.

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  • 1. Wagenvoort CA Beetstra A Spijker J (1978) Capillary hemangiomatosis of the lungs. Histopathology 2: 401-406

  • 2. Eltorky MA Headley AS Winer-Muram H Garrett HE Jr Griffin JP. Pulmonary capillary haemangiomatosis: a clinicopathologic review. Ann Thorac Surg 1994; 57:772-776

  • 3. Oviedo A Abramson LP Worthington R et al (2003) Congenital pulmonary capillary hemangiomatosis: report of two cases and review of the literature. Pediatr Pulmonol 36: 253-256

  • 4. Enzensberger C Achterberg F Graupner O Wolter A Herrmann J Axt-Fliedner R Wall-motion tracking in fetal echocardiography-Influence of frame rate on longitudinal strain analysis assessed by two-dimensional speckle tracking. Echocardiography 2017 Jun; 34(6):898-905

  • 5. Enzensberger C Achterberg F Degenhardt J Wolter A Graupner O Herrmann J Axt-Fliedner R Feasibility and Reproducibility of Two-Dimensional Wall Motion Tracking (WMT) in Fetal Echocardiography. Ultrasound Int Open. 2017 Feb; 3(1): E26-E33

  • 6. Sposito Cavallo SL Macias Sobrino LA et al (2017) Congenital pulmonary capillary hemangiomatosis in a newborn Arch Agent Pediatr 115 (1): e17-e20

  • 7. Dello Russo P Franzoni A Baldan F et al (2015) A 16q deletion involving FOXF1 enhancer is associated to pulmonary capillary hemangiomatosis. BMC Medical Genetics 16:94

  • 8. Silva CJTA Massie J Mandelstam SA (2005)Pulmonary capillary haemangiomatosis in a premature infant. Pediatr Radiol 35: 635-640

  • 9. Mc Govern E McNally P O’Sullivan M et al (2016) Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension. Cardiology in the Young 26: 663-668

  • 10. Smrcek JM Baschat AA Germer U Gloeckner-Hofmann K Gembruch U Ultrasound Obstet Gynecol. 2001 May; 17 (5): 403-9

  • 11. Bartyik K Bede O Tiszlavicz L et al (2004) Pulmonary capillary haemangiomatosis in children and adolescents: report of a new case and review of the literature. Eur J Pediatr 163: 731-737

  • 12. Ginns LC Roberts DH Mark EJ et al. (2003) Pulmonary capillary hemangiomatosis with atypical endotheliomatosis: successful antiangiogenetic therapy with doxycycline. Chest 124: 2017-2022

  • 13. Almagro P Julia J Sanjaume M et al (2002) Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension. Report of 2 new cases and review of 35 cases from the literature. Medicine (Baltimore) 81: 417-424

  • 14. Best DH Sumner KL Austin ED et al EIF2AK4 mutations in pulmonary capillary haemangiomatosis. Chest 2014 145:231-236

  • 15. Langleben D Heneghan JM Batten AP et al. Familial pulmonary capillary hemagiomatosis resulting in primary pulmonary hypertension. Ann Intern Med 1988; 109: 106-109

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