Primary Vulvar Lymphangioma Circumscriptum

Kökçü, Arif; Sari, Seher; Kefeli, Mehmet

doi:10.1097/lgt.0000000000000045

Cited by 20 publications

(17 citation statements)

References 19 publications

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“…However, the treatment of lymphangiectasia can be very challenging because of high recurrence rate after surgery, which is reported to be 23.1%. 3,9 The prognosis for patients with diffuse lymphangiomatosis is poor if the condition is resistant to standard therapies. 8 More conservative treatments such as skin care compression are difficult to apply considering the location of the lesions.…”

Section: Discussionmentioning

confidence: 99%

“…2 Twenty-two cases of primary lymphangiectasia (also referred to as lymphangioma circumscriptum) have been described and confirmed by histopathology. 3 Vulvar lymphangiectasia associated with NS is a rare phenomenon which, to our knowledge, has been described only once before. 4 For the treatment of lymphangiectasia, no differentiation has been made between acquired and congenital lymphangiectasia.…”

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confidence: 88%

“…1 Cutaneous lymphangiectasia, also called acquired lymphangioma, is a benign cutaneous disorder involving the pathologic dilatation of dermal and subcutaneous lymphatic channels. 2,3 The lesions formed by these malformed channels can have a “frogspawn”-like appearance. 2,3 As a result of these damaged lymph channels, lymph edema can develop in the affected body part.…”

mentioning

confidence: 99%

“…2,3 The lesions formed by these malformed channels can have a “frogspawn”-like appearance. 2,3 As a result of these damaged lymph channels, lymph edema can develop in the affected body part. 2 Twenty-two cases of primary lymphangiectasia (also referred to as lymphangioma circumscriptum) have been described and confirmed by histopathology.…”

mentioning

confidence: 99%

“…Observation and surgery seem to be the preferred strategy. 3 We present a patient with recurrent vulvar lymphangiectasia and NS who has been treated successfully with a 2-stage approach using lymphovenous anastomosis (LVA) followed by excision of the vulvar lesion and reconstruction.…”

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confidence: 99%

See 4 more Smart Citations

Lymphovenous Anastomosis and Secondary Resection for Noonan Syndrome with Vulvar Lymphangiectasia

Winters

Tielemans

Ulrich

2016

Plastic and Reconstructive Surgery - Global Open

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Summary:In this case report we describe the use of a 2-stage approach to treat severe recurrent vulvar lymphangiectasia in a patient with Noonan syndrome. First, 3 functional lymphatic vessels were identified and anastomosed to venules in an end-to-end fashion. Then, in a second surgical procedure, the vulvar lesions were resected as much as possible and the vulva was reconstructed. By the 12-month follow-up the patient had recovered well. Although there were still some small vesicles on the left labia there was no more ooze, itch, and pain. Lymphatic mapping using indocyanine green showed improvement of the edema of her vulva region and patent LVA. In addition to the demonstration of this 2-stage approach, this case report also demonstrates the benefits of preemptive LVA before performing surgery that may be at high risk for postoperative lymph edema.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 88%

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Lymphovenous Anastomosis and Secondary Resection for Noonan Syndrome with Vulvar Lymphangiectasia

Winters

Tielemans

Ulrich

2016

Plastic and Reconstructive Surgery - Global Open

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Genital Disease in Children

Fischer¹

2019

Harper's Textbook of Pediatric Dermatology

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Clinical outcomes of primary surgical treatment for acquired vulvar lymphangioma circumscriptum

Yoon

Kim

Lee

et al. 2015

Arch Gynecol Obstet

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ObjectiveTo assess the clinical outcomes of surgical treatment for acquired vulvar lymphangioma circumscriptum in patients who received radical surgery and/or adjuvant radiation therapy for cervical cancer.MethodsA retrospective chart review of eight patients was performed to assess the demographic information, chief complaints, treatment modality for cervical cancer, location, and primary treatment modality for vulvar LC, postoperative changes in symptoms, and/or signs, the development of local recurrence and the outcome of patients.ResultsAll eight patients were previously diagnosed with cervical cancer FIGO clinical stage IA to IIA and received surgery, radiation therapy, or concurrent chemoradiation therapy. Microscopic examination revealed multiple, dilated, D2-40-positive dermal vascular channels containing eosinophilic proteinaceous material, consistent with LC. Most chief complaints showed considerable improvements on assessment at the outpatient clinic after the primary surgery. No patient showed aggravation of symptoms. Two patients developed local recurrences. One patient developed recurrence on the opposite side 13 months after local excision. We performed a second wide local excision. Another patient developed recurrence 47 months after the primary surgery. Since the lesion was very small and localized, we decided to manage it conservatively, but monitor it very closely. The remaining six patients remained free of recurrence.ConclusionIt is not easy for gynecologists to have an initial clinical diagnosis of LC, because there are a number of diseases that exhibit similar clinical manifestation to that of vulvar LC. Even if it is diagnosed correctly, local recurrence often occurs. Relevant symptoms associated with LC are not only distressing, but also affect patients’ quality of life. Based on our data, we propose that surgical treatment could provide a more long-lasting answer compared to other treatment modalities, since it is beneficial in terms of clinical outcomes. In the future, a long-term follow-up investigation is required to assess the prognosis and to compare the efficacy and side effects of each modality.

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Primary Vulvar Lymphangioma Circumscriptum

Abstract: The treatment of primary vulvar lymphangioma circumscriptum is mainly surgery. Depending on the age of the patient and the extent of lesion, surgical treatment might be in the form of the local excision, labiectomy, or vulvectomy.

Cited by 20 publications

References 19 publications

Lymphovenous Anastomosis and Secondary Resection for Noonan Syndrome with Vulvar Lymphangiectasia

Lymphovenous Anastomosis and Secondary Resection for Noonan Syndrome with Vulvar Lymphangiectasia

Genital Disease in Children

Clinical outcomes of primary surgical treatment for acquired vulvar lymphangioma circumscriptum

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