CLOVES SYNDROME: CLINICAL MANIFESTATIONS AND FEATURES OF SURGICAL TREATMENT
DOI:
https://doi.org/10.11603/1811-2471.2017.v0.i4.8375Keywords:
CLOVES syndrome, combined lymphatic malformations, children.Abstract
The aim of the study is to determine the frequency of CLOVES syndrome among patients with lymphatic malformations, to determine the features of its clinical course and to evaluate the results of treatment.
Methods. Among 126 patients with lymphatic malformations who received inpatient treatment during the period from December 2010 to March 2017 at the clinical base of Pediatric Surgery Department of the Bogomolets National Medical University NPSH "OKHMATDYT" CLOVES syndrome was diagnosed in 7 (5, 56%) children. Female patients were 5, male - 2.
Results. The diagnosis of CLOVES syndrome was made on the basis of a combination clinical features, in particular, constant symptoms in all children were lipomatous overgrows of the trunk, slow flow vascular malformations, lymphatic (n=6, 85.71%), capillary (n=5, 71.43%) and venous (n=4, 57.14%); lesion of the musculoskeletal system: scoliosis (n=7, 100%), "sandal gap" (n=3, 42,86%), macrodactyly (n=3, 42,86%), syndactyly (n=3, 42.86%), a triangular delta shape foot (n=1, 14.29%). Only some clinical signs of the syndrome identify at birth, with age primary symptoms progressed, and new congenital abnormalities diagnosed. In 7 children with CLOVES syndrome performed from 1 to 6 surgical interventions. The purpose of the surgery was to remove vascular malformations and fatty masses. Operative technics has its own features: intraoperative anticoagulant therapy (heparin 50 U/kg/h), selection and separate ligation of altered venous vessels, reduction of residual masses with argon coagulation. OK-432 sclerotherapy was performed in three patients. Conservative treatment for scoliosis with corsets Chenot used in three patients. Surgical management of fingers partial gigantism of the foot is carried out in one patient aged 11 years.
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