Klippel-Trenaunay Syndrome Complicated by Ascites and Vaginal Lymphatic Drainage in Adolescence: A Case Report.

Oct 2012

Scribner DR Jr, Lara-Torre E, Heineck RJ, Weiss PM.

Source

Virginia Tech Carilion School of Medicine, Roanoke, VA.

Abstract

BACKGROUND:

 Klippel-Trenaunay syndrome is a rare disease characterized by capillary malformationsand soft tissue and bony hypertrophy and atypical varicosities. Management of this syndrome is focused primarily on treatment of the complications that arise from these malformations. Ascites and lymphedema are two of the more common complications in these patients.

CASE:

 A 15-year-old female with Klippel-Trenaunay syndrome presented with chylous ascites, vaginal drainage, and unilateral lower extremity lymphedema. Treatment included dilation, hysteroscopy and curettage, and laparoscopic evacuation of abdomino-pelvic ascites with resolution of symptoms for 32 months. Repeat laparoscopic drainage was successful and remains symptom free after 12 months.

CONCLUSION:

 Vaginal drainage of chylous ascites is a rare complication from Klippel-Trenaunay syndrome and can be successfully managed by techniques to remove abdomino-pelvic ascites.

Elsevier

Cystic lymphangiomatous hamartoma masquerading as massive ascites.

Cystic lymphangiomatous hamartoma masquerading as massive ascites.
Indian J Pediatr. 2009 May

Parakh A, Dubey AP, Garg A, Khurana N, Aggarwal SK.
Department of Pediatric, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India, ankitparakh102@rediffmail.com.

We report a 4-year-old boy presenting with a tense massive ascites and large hydrocele. History and physical examination were unremarkable. Routine laboratory studies were normal. Abdominal ultrasonography revealed massive ascites. Contrast CT was suggestive of a large cyst covering the entire peritoneal cavity. At laparotomy, a large cystic tumor was found extending into the scrotum through the left inguinal ring. Histopathologic examination diagnosed the tumor as a cystic lymphangiomatous hemartoma. Although abdominal lymphangiomas are seen in children, but presenting as massive ascites with hydrocele is very rare.

SpringerLink

A case of lymphangioleiomyomatosis found due to chylous ascites, pleural effusion and pelvic lymphadenopathy

A case of lymphangioleiomyomatosis found due to chylous ascites, pleural effusion and pelvic lymphadenopathy

Nihon Kokyuki Gakkai Zasshi. 2007

Yano T, Hasizume I, Kasamatsu N, Kato T, Shibata M, Ashinuma N, Kobayashi K, Yasuda T, Nakamura A.
Department of Respiratory Medicine, Hamamatsu Medical Center.

We report a case of lymphangioleiomyomatosis, complaining initially of abdominal distension due to massive chylous ascites. The patient was a 28-year-old woman in whom abdominal ultrasound had strongly suggested the existence of both pelvic lymphadenopathy and massive ascites, the latter subsequently turning out to be chylous. Pelvic lymph node biopsy yielded a diagnosis of lymphangioleiomyomatosis. High-resolution computed tomography (HRCT) of the chest showed no remarkable findings except for very few cystic changes in the lung parenchyma.

Pulmonary function had remained normal except for a temporary constrictive pattern when chylous pleural effusion developed. No airflow obstruction was detected on pulmonary function tests.

Although lymphangioleiomyomatosis is often associated with pulmonary symptoms, we should bear in mind the possibility of lymphangioleiomyomatosis even in the absence of such symptoms when facing any woman of child-bearing age with abdominal chylous ascites of unknown etiology.

PMID: 17554983 [PubMed - indexed for MEDLINE]