Journal of Vascular Surgery: Venous and Lymphatic Disorders
Case reports of the monthSuccessful treatment of large abdominal lymphatic malformations and chylous ascites with intra-abdominal lymphovenous anastomosis
Section snippets
Case report
A 10-year-old girl with a large abdominal LM associated with superficial chylous leak from the vagina and unilateral left lower limb lymphedema presented with progressive abdominal distention, exacerbation of vaginal leakage, and refractory lymphorrhea from trivial scratches on her leg.
One year earlier, we performed LVA on her left lower limb to reduce flow to the central lymphatic system, resulting in decreased lymphedema and vaginal chylous leakage. However, after menarche, she suddenly
Discussion
The observations and the biopsy result in our case suggested central conducting lymphatic anomaly (CCLA) or generalized lymphatic anomaly.4,11, 12, 13, 14, 15, 16, 17 At present, both diseases are characterized by a broad spectrum of anomalies involving the central lymphatic system, lumbar trunks, cisterna chyli, thoracic duct, and their major tributaries. Because of the rareness and similarity of these kinds of LMs, there is overlap in diagnostic criteria, making precise diagnosis difficult.
References (18)
- et al.
Diagnosis and management of primary chylous ascites
J Vasc Surg
(2006) - et al.
Complex lymphatic anomalies
Semin Pediatr Surg
(2014) - et al.
Life-threatening anomalies of the thoracic duct: anatomic delineation dictates management
J Pediatr Surg
(2001) - et al.
Medical and surgical management of chylothorax and associated outcomes
Am J Med Sci
(2010) - et al.
Lymphaticovenous bypass of the thoracic duct for the treatment of chylous leak in central conducting lymphatic anomalies
J Pediatr Surg
(2019) - et al.
Sclerotherapy for lymphangioma in children
Int J Pediatr Otorhinolaryngol
(2003) - et al.
Lymphatic malformations: diagnosis and management
Semin Pediatr Surg
(2014) - et al.
Pathogenic variant in EPHB4 results in central conducting lymphatic anomaly
Hum Mol Genet
(2018) - et al.
Clinical features and prognosis of generalized lymphatic anomaly, kaposiform lymphangiomatosis, and Gorham-Stout disease
Pediatr Blood Cancer
(2016)
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Author conflict of interest: none.
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The editors and reviewers of this article have no relevant financial relationships to disclose per the Journal policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest.