Sildenafil for microcystic lymphatic malformations of the head and neck: A prospective study

Koshy, John C.; Eisemann, Bradley S.; Agrawal, Nikhil; Pimpalwar, Sheena; Edmonds, Joseph L.

doi:10.1016/j.ijporl.2015.03.034

Cited by 25 publications

(15 citation statements)

References 7 publications

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“…These data suggest that the effect of sildenafil we observed may be mediated by decompression of lymphatic vessels via indirect effects on adjacent smooth muscle rather than by direct changes on lymphatic vessel remodelling. A prospective study in five children with microcystic lymphatic malformations of the head and neck did not demonstrate any effect of sildenafil 13. In a series of 14 children with various vascular malformations, none of them reported any improvement and some side effects including bleeding and infections were observed in 4 of them 14.…”

Section: Discussionmentioning

confidence: 87%

Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil

et al. 2019

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General lymphatic anomaly (GLA) is a very rare disorder, characterised by multifocal lymphatic malformations into various tissues that is due to congenital abnormalities of lymphatic development. No treatment has ever proved its efficiency.We report a 22-year-old man with recurrent bronchial casts due to thoracic involvement of GLA. After a 6-month treatment with sildenafil (20 mg three times a day), a phosphodiesterase 5 inhibitor, chest CT scan showed a complete regression of ground-glass opacities and lung function test results improved substantially and remained stable for 1 year. The treatment was well tolerated.This observation suggests that sildenafil may be a therapeutic approach to be tested in thoracic involvement of GLA.

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Section: Discussionmentioning

confidence: 87%

Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil

et al. 2019

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“…In the literature, 19 pediatric patients with severe LMs have been treated with sildenafil, reported in six articles (Table 1) (Danial et al, 2014;Gandhi, Lin, & O'Hara, 2013;George et al, 2015;Koshy, Eisemann, Agrawal, Pimpalwar, & Edmonds, 2015;Singh, Singh, & Mundy, 2013;Swetman et al, 2012). All patients were children (8 days to 12 years old).…”

Section: Discussionmentioning

confidence: 99%

Oral sildenafil as a treatment option for lymphatic malformations in PIK3CA-related tissue overgrowth syndromes

Horbach

Jolink

Horst

2016

Dermatologic Therapy

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Patients with extensive lymphatic malformations associated with tissue overgrowth syndromes (such as Klippel-Trenaunay syndrome and CLOVES) often pose a therapeutic challenge for physicians. In recent years, it has been suggested that oral sildenafil therapy might be used to treat congenital lymphatic malformations. However, this possible new therapy has not yet been used in patients with lymphatic malformations associated with tissue overgrowth syndromes. A 30-year-old man with extensive capillary-lymphatic malformations of the right leg and thorax, and a tissue overgrowth syndrome caused by a somatic mutation in the PIK3CA gene, was treated with oral sildenafil due to symptoms of pain, dyspnea, and functional impairment. Several weeks after the start of the treatment, the patient reported softening of the lymphatic malformation and a significant improvement of his symptoms and physical condition. So far, sildenafil is still considered a last resort in the treatment of complex treatment-resistant lymphatic malformations. With this case report, we demonstrate that sildenafil could also be an alternative treatment option for lymphatic malformations in patients with syndromes belonging to the PIK3CA-related overgrowth spectrum.

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“…Sildenafil has been reported to decrease the size of lymphatic malformations in a small number of case reports; however, other studies have shown no effect of sildenafil. [53][54][55][56] Steroids, vincristine, and interferon have also been used sporadically to treat various malformations with mixed results. 57 The discovery of sirolimus as an effective treatment changed the management paradigm of patients with complicated vascular malformations.…”

Section: Vascular Malformationsmentioning

confidence: 99%

Advances in the Medical Management of Vascular Anomalies

Ricci

2017

Semin intervent Radiol

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Vascular anomalies comprise a spectrum of diseases that are broadly classified as tumors and malformations. Diagnosis is often challenging, given a wide range of clinical presentations with overlapping signs and symptoms. Accurate diagnosis is critical to determine prognosis and to generate a management plan, which frequently involves multiple subspecialists during different phases of treatment. An updated classification system provides structure and clear, consistent terminology, allowing for improved diagnosis, provider communication, and collaboration. Historically, treatment of vascular anomalies was primarily surgical and medical therapies were limited or ineffective. Recent discoveries of pharmacologic agents effective in treating vascular anomalies have broadened our medical therapeutic options, limiting the need for unnecessary or high-risk procedures and improving patients' quality of life.

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Sildenafil for microcystic lymphatic malformations of the head and neck: A prospective study

Cited by 25 publications

References 7 publications

Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil

Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil

Oral sildenafil as a treatment option for lymphatic malformations in PIK3CA-related tissue overgrowth syndromes

Advances in the Medical Management of Vascular Anomalies

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