Bleomycin A5 sclerotherapy for cervicofacial lymphatic malformations

Abstract Background Lymphatic malformations are rare benign cystic tumors that result from localized disordered embryologic development of the lymphatic system and can develop anywhere in the body, predominantly in the head and neck. These lesions are classified according to the diameter of the largest cystic cavity within the lesion into microcystic and macrocystic types. Historically, surgical excision has been considered the mainstay of treatment and still remains the first therapeutic option of choice for many surgeons particularly for giant macrocystic lesions. Several alternative therapeutic modalities emerged including intralesional sclerotherapy and laser therapy with encouraging results. The study is designed to assess the effectiveness and safety of surgical excision as an initial therapeutic option in the management of these malformations. Results Asymptomatic mass with parental cosmetic and functional concerns was the mode of presentation in six (66.7%) patients. Seven (77.8%) patients were presented before the age of 2 years. Six (66.7%) of the patients had their lesions in the neck. Complete surgical excision was achieved in eight (88.9%) patients without any evident significant injury to vital neurovascular structures. None of the patients had any difficulties with breathing, swallowing, or phonation and cosmetic results were satisfactory in the majority (88.9%) of them. Conclusion Radical surgical excision of giant macrocystic lymphatic malformations in children is possible in experienced hands. It is an effective and safe initial therapeutic option and gives satisfactory esthetic and functional results.

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Lymphatic Malformation Responsive to Sirolimus in Keratinocytic Epidermal Nevus Syndrome with KRAS Mutation: A Case and Brief Literature Discussion

Case Reports in Dermatology ◽

10.1159/000515247 ◽

2021 ◽

pp. 195-201

Author(s):

Emily Sideris ◽

Er Tsing Vivian Tng ◽

Paul Chee

Keyword(s):

Current Literature ◽

Kras Mutation ◽

Rare Case ◽

Mtor Inhibitor ◽

Vascular Malformations ◽

Lymphatic Malformation ◽

Literature Discussion ◽

Lymphatic Malformations ◽

Epidermal Nevus ◽

Overgrowth Syndromes

We present a rare case of KRAS keratinocytic epidermal nevus syndrome with lymphatic malformation, responsive to treatment with sirolimus, an mTOR inhibitor. A brief review of the current literature regarding sirolimus use in vascular malformations, lymphatic malformations, regional overgrowth syndromes, and RASopathies is discussed.

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Lymphatic malformations compromising the upper airway in children: ultrasound-guided intralesional focal sclerotherapy with bleomycin targeting culprit lesions

Pediatric Surgery International ◽

10.1007/s00383-020-04715-y ◽

2020 ◽

Vol 36 (9) ◽

pp. 1047-1054

Author(s):

Hidehito Usui ◽

Masato Shinkai ◽

Norihiko Kitagawa ◽

Kyoko Mochizuki ◽

Issei Kawakita ◽

...

Keyword(s):

Upper Airway ◽

Ultrasound Guided ◽

Lymphatic Malformations

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EXIT (Ex utero Intrapartum Treatment) in lymphatic malformations of the head and neck: Discussion of three cases and proposal of an EXIT-TTP (Team Time Procedure) list

International Journal of Pediatric Otorhinolaryngology ◽

10.1016/j.ijporl.2011.09.015 ◽

2012 ◽

Vol 76 (1) ◽

pp. 20-27 ◽

Cited By ~ 25

Author(s):

Stefania Stefini ◽

Tullia Bazzana ◽

Cinzia Smussi ◽

Michela Piccioni ◽

Tiziana Frusca ◽

...

Keyword(s):

Head And Neck ◽

Lymphatic Malformations ◽

Ex Utero Intrapartum Treatment

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Chylous Content May Determine the Optimal Surgical Approach for Mesenteric Lymphatic Malformations in Childhood

Journal of Vascular Surgery Venous and Lymphatic Disorders ◽

10.1016/j.jvsv.2021.06.008 ◽

2021 ◽

Author(s):

Paolo Gasparella ◽

Besiana P. Beqo ◽

Emir Q. Haxhija ◽

Castellani Christoph ◽

Christoph Arneitz ◽

...

Keyword(s):

Surgical Approach ◽

Lymphatic Malformations

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Spontaneously Resolved Macrocystic Lymphatic Malformations: Predictive Variables and Outcomes

Plastic Surgery ◽

10.1177/2292550317693815 ◽

2017 ◽

Vol 25 (1) ◽

pp. 27-31 ◽

Cited By ~ 1

Author(s):

Michael J. Phang ◽

Douglas J. Courtemanche ◽

Marija Bucevska ◽

Claudia Malic ◽

Jugpal S. Arneja

Keyword(s):

Respiratory Tract ◽

Median Time ◽

Vascular Malformations ◽

Neck Region ◽

Retrospective Chart Review ◽

Spontaneous Resolution ◽

Low Flow ◽

Upper Respiratory Tract ◽

Lymphatic Malformations ◽

Upper Respiratory

Introduction: Lymphatic malformations are benign, low-flow vascular malformations that typically present at or near birth. Due to morbidity associated with operative treatment, nonoperative treatment with injection of sclerosant has become the mainstay of therapy. Over the past 15 years, several patients at our centre with macrocystic (>2 cm cyst size) lymphatic malformations have seen their lesions resolve spontaneously while awaiting treatment. In this study, we review features of these patients that may contribute to spontaneous resolution. Method: A retrospective chart review was conducted from our Vascular Anomalies Clinic database (1999-2014) of all macrocystic lymphatic malformations; characteristics of patients with spontaneous resolution were reviewed. Results: Of 61 patients with macrocystic lymphatic malformations, 7 cases (11.5%; 4 females, 3 males) resolved spontaneously. Median age at malformation appearance was 2 years (range: 0-6.5 years), with median age at resolution of 4 years (range: 10 months-7 years). Median time from appearance to resolution was 24 months (range: 3-43 months), with a median follow-up time of 4 years (range: 1-15 years). All but 1 case was associated with local or upper respiratory tract infection antecedent to resolution. Six of the 7 lesions were located in the neck. Conclusion: Among the cases reviewed, there was a common theme of upper respiratory tract or local infection antecedent to spontaneous lesion resolution. Compared to the literature, our proportion of malformations presenting after birth and the proportion of malformations presenting in the neck region were higher than those of other series. Although side effects associated with treatment are generally mild and/or rare, risks related to sclerotherapy and the accompanying requirement for general anesthesia in pediatric populations nevertheless exist. As the median time from lesion appearance to resolution was 24 months, it may be reasonable to observe these malformations for up to 24 months before proceeding with treatment if the lesion does not impair function and disfigurement is not considerable, particularly if the lesion presents after birth and/or is located in the neck region.

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