A female infant with phacomatosis pigmentovascularis and congenital chylous ascites: A case report

Medicine
Shuai XuNing Sun

Abstract

Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by capillary malformation and pigmentary nevus. Congenital chylous ascites (CCA) is also a rare disease that results from maldevelopment of the lymphatic system. We report a case of a 5-month-old girl, who had both PPV and CCA. A 5-month-old girl is reported, who presented extensive nevus flammeus and an aberrant Mongolian spot with congenital chylous ascites. The expression of extensive nevus flammeus and an aberrant Mongolian spot with congenital chylous ascites, that was diagnosed as type IIb phacomatosis pigmentovascularis. Conservative treatment included administration of somatostatin, MCT-based diet or TPN with drainage of ascitic fluid. Surgery was taken into account after failed conservative treatments. Before surgery, it is necessary to locate the abnormal lymphatic vessels. Conservative treatment and surgery sometimes functioned limitedly on CCA. According to the classification system of ISSVA (the International Society for the Study of Vascular Anomalies), this case meet the classification of CLM included in combined vascular malformations. It is likely to there is a connection between these two congenital diseases.

References

Aug 23, 2001·Archives of Disease in Childhood·C BhatiaZ Slavik
Aug 23, 2002·The American Journal of Gastroenterology·Andrés Cárdenas, Sanjiv Chopra
Mar 23, 2005·Archives of Dermatology·Rudolf Happle
Jun 13, 2006·Journal of Vascular Surgery·Corradino CampisiFrancesco Boccardo
May 16, 2007·Journal of Pediatric Surgery·Minoru KuroiwaNorio Suzuki
Apr 10, 2010·Acta Paediatrica·Belma Saygili KaragolMehmet Sah Ipek
Sep 10, 2014·Journal of Indian Association of Pediatric Surgeons·Radheshyam PurkaitBirendranath Roy

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