scholarly journals Efficacy of Sirolimus in Patients Requiring Tracheostomy for Life-Threatening Lymphatic Malformation of the Head and Neck: A Report From the European Reference Network

2021 ◽  
Vol 9 ◽  
Author(s):  
Annegret Holm ◽  
Maroeska te Loo ◽  
Leo Schultze Kool ◽  
Päivi Salminen ◽  
Veronica Celis ◽  
...  

Extensive lymphatic malformations (LMs) of the head and neck region may require tracheostomy to secure the airway. Treatment of these life-threatening LMs is usually multimodal and includes sclerotherapy and surgery, among others. Recently, systemic therapy with sirolimus has been introduced as an effective treatment for venous and lymphatic malformations; its efficacy and safety profile in patients with extensive LM requiring tracheostomy are, however, as yet not fully known. We performed a retrospective, multicenter review and identified 13 patients with an extensive LM of the head and neck region, who previously underwent placement of tracheostomy and subsequently received sirolimus treatment with the aim to improve the local respiratory situation and remove the tracheostomy. Under sirolimus therapy, tracheostomy could be reversed in 8/13 (62%) patients, a further 2/13 (15%) patients improved markedly, and removal of the tracheostomy was planned at the time of writing, while 3/13 (23%) patients showed insufficient or absent response to sirolimus, rendering tracheostomy reversal not feasible. The median duration of sirolimus treatment until removal of tracheostomy was 18 months (range, 8 months to 5.6 years). Adverse events of sirolimus therapy were common [10/13 (77%) patients], yet the majority of these were mild [9/10 (90%) patients] and only one severe adverse event was recorded, with ulceration and necrosis at a catheter insertion site. In conclusion, sirolimus can be considered an effective and safe salvage treatment in patients with extensive LM even after placement of a tracheostomy, as closure of the latter was possible in the majority of patients (62%) of our retrospective cohort. A better understanding of when to start sirolimus therapy, of the duration of treatment, and of factors allowing the prediction of treatment response will require further investigation.

Author(s):  
I. M. Benzar

Background. Surgery has previously been the only treatment for lymphatic malformations (LMs), but in the head and neck region is challenging due to the risk of scarring, nerve damage, recurrence. Sclerotherapy may be a perfect alternative.Objective. The aim of the study is to determine the efficacy and safety of the OK-432 sclerotherapy in the children with craniofacial LMs.Methods. 81 children with head and neck LM between December 2010 and March 2017were involved into the study. The follow-up period was from 6 to 79 months. According to the size of cysts, LMs were classified into macrocystic, microcystic, and mixed. The result of the treatment of LMs was determined by the percentage of reduction in size as excellent (decrease by more than 90%), good (by 50%-89%), satisfactory (by 20%-49%) and none (by less than 20%).Results. The macrocystic LMs diagnosed in 41.97% of patients, microcystic - in 12.35%, and mixed – in 45.68% of children. OK-432 sclerotherapy only was performed for 83.9% of patients and in 12.3% in combination with surgery. The range of sclerotherapy sessions was from 1 to 11. An excellent result in 96.97% of cases was evidenced in the patients with macrocystic LM. Poor result was proved in the patients with microcystic LMs; the most of them (55.56%) had satisfactory result. In the patients with mixed LM, an excellent and good result was evidenced in 83.33%. After 198 sessions of OK-432 sclerotherapy, complications associated with the treatment occurred in 5 (2.52%) cases.Conclusions. OK-432 sclerotherapy is a safe and effective treatment of head and neck LMs in children. Macrocystic LMs proved the best response to OK-432 treatment.


2018 ◽  
Vol 132 (9) ◽  
pp. 827-831 ◽  
Author(s):  
K Mahawerawat ◽  
P Kasemsiri

AbstractBackgroundAlthough melioidosis in the head and neck region is uncommon, it is a potentially life-threatening infection. Thus, early diagnosis and proper management are very important.ObjectivesTo report the clinical presentation and management of melioidosis in the head and neck.MethodA retrospective study was conducted from 1 January 2013 to 31 October 2016 in Mukdahan Hospital, Thailand. Case records of patients who had presented with culture-positive melioidosis were analysed.ResultsMedical records of 49 patients (23 males and 26 females) were analysed. Patients ranged in age from 1 to 75 years. Clinical presentations included 22 parotid abscesses, 16 neck abscesses and 11 suppurative lymphadenitis cases. Only 35 patients (71 per cent) had high indirect haemagglutination assay titres of ≥ 1:160 (95 per cent confidence interval = 45.35–88.28). Almost half of the patients received intravenous ceftazidime and subsequently oral co-trimoxazole. Oral antibiotic regimens were prescribed for mild localised melioidosis. Overall, 95.65 per cent of patients were in remission and no relapses were observed (95 per cent confidence interval = 85.47–98.80).ConclusionCareful clinical correlation and proper investigation are required to establish an early diagnosis of melioidosis and to initiate appropriate treatment.


2021 ◽  
Vol 8 (4) ◽  
pp. 1234
Author(s):  
Manpreet Kaur ◽  
Parul Sachdeva ◽  
Rajan Syal ◽  
Savijot Singh

Background: Low flow vascular malformations are most common in the head and neck region. Only symptomatic malformations require treatment. Sclerotherapy followed by surgery was considered the gold standard treatment but in the head and neck region, it may produce cosmetic and physiological defects. In the present study, multiple injections of sclerotherapy with 3% sodium tetradecyl sulphate was used for the treatment of low flow vascular malformations.Methods: Twenty cases of low flow vascular malformations of the oral cavity who presented in the outpatient department of ESIC Model Hospital, Ludhiana from 2014-2016 were selected for the study. Only significantly sized (>4 cm) and easily accessible lesions were included. Staged sequential sclerotherapy with 3% STS under strict fluoroscopy control was used as the sole treatment.Results: A total of 20 patients were taken of which, 25% required three sessions, 65% five to six sessions each and 10% required eight sessions each. All patients showed good results with complete regression and no mucosal ulceration.Conclusions: Staged sequential sclerotherapy with 3% STS should be the treatment of choice in low flow vascular lesions involving mucosal and cutaneous structures of head and neck region especially anterior two-thirds of tongue, palate, gingiva, buccal mucosa and lips. Surgical removal may affect critical neurovascular structures and cause cosmetic deformity. So the removal is advisable in life-threatening conditions, lesions requiring general anaesthesia and single sitting removal.


2018 ◽  
Vol 10 (10) ◽  
pp. e25-e25
Author(s):  
Gerard Deib ◽  
Amgad El Mekabaty ◽  
Philippe Gailloud ◽  
Monica Smith Pearl

Life-threatening bleeding in the head and neck region requires urgent management. These hemorrhagic lesions, for example, a ruptured pseudoaneurysm, are often treated by transarterial embolization (TAE), but prior intervention or surgery, inflammation, anatomic variants, and vessel tortuosity may render an endovascular approach challenging, time-consuming, and sometimes impossible. We report two cases of severe head and neck hemorrhages successfully embolized with n-butyl cyanoacrylate via direct puncture, and propose this approach as a fast, safe, and effective alternative to TAE.


New Medicine ◽  
2019 ◽  
Vol 23 (4) ◽  
Author(s):  
Agata Wasilewska ◽  
Małgorzata Badełek-Izdebska ◽  
Lidia Zawadzka-Głos ◽  
Remigiusz Krysiak ◽  
Jarosław Żyłkowski

Introduction. Lymphatic malformations (LMs) are benign lesions thought to be caused by the abnormal development of the lymphatic system in utero. Most commonly, LMs affect the head and neck. Because of LM morphology and location close to important vascular and nervous structures, surgical treatment is difficult, associated with a high risk of complications, and often incomplete. Aim. Bleomycin sclerotherapy is a recognised minimally invasive technique used in the treatment of LMs. We present the outcomes of bleomycin therapy of LMs located in the head and neck area in children receiving therapy in our centre. Material and methods. Between September 2017 and October 2019, treatment with bleomycin was provided to a total of 6 patients with LMs of the head and neck, aged from 3 weeks to 10 years. The procedures were performed under ultrasound and/or fluoroscopy guidance. The aspects analysed included the number of procedures applied in patients, drug doses, treatment response and complications. Results. In 4 patients, the LM was located on the neck, in 1 patient ? on the neck and in the mediastinum, and in 1 patient in the cheek region. Three patients underwent 2 procedures, 1 patient ? 3 procedures, and 2 patients ? 1 procedure. The treatment outcome was excellent and good in 4 patients and 1 patient, respectively. However, in 1 patient, the therapeutic effect was unsatisfactory, and a decision was made to administer another course of treatment. The maximum single dose of bleomycin was 10,000 IU; the dose of 700 IU/kg BW was not exceeded. No complications were observed after the procedures. Conclusions. Preliminary results suggest that bleomycin sclerotherapy of LMs in the head and neck region in children is an effective and safe treatment modality.


1989 ◽  
Vol 103 (4) ◽  
pp. 379-382 ◽  
Author(s):  
N. D. Stafford ◽  
R. C. D. Herdman ◽  
S. Forster ◽  
A. J. Munrot

AbstractOver a 40 month period, 219 patients with AIDS were seen at St. Mary's Hospital, London. Kaposi's sarcoma, one of the criteria for diagnosing the syndrome, was the presenting feature in 62 patients. Eighty four patients developed Kaposi's sarcoma at some stage of their disease and the head and neck region was involved in 56 of them.Although Kaposi's sarcoma is rarely life threatening in AIDS, potential airway obstruction, pain or cosmetic disfigurement may justify treating the lesion. Whilst cutaneous tumours may be managed by using radiotherapy (16 Gray in four fractions over four days), this treatment produces a sever mucositis when used to treat mucosal disease; we, therefore use a combination of vincristine and bleomycin for this.


2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Ayşe Özlem Gündeşlioğlu ◽  
Emine Çiğdem Özen

Necrotizing fasciitis is a rapidly progressive soft tissue infection that can cause local tissue destruction, necrosis, and life threatening severe sepsis. Necrotizing fasciitis in the head and neck region caused by an extravasation injury is rare. This paper reports a patient with necrotizing fasciitis of the cervical region caused by an extravasation injury which required an early surgical debridement.


Author(s):  
N. N. Ivanova ◽  
A. M. Mitrofanova ◽  
D. V. Shevtsov ◽  
I. N. Vorozhtsov ◽  
N. S. Grachev

One of rare location of teratoma in children is head and neck region. Treatment options, including surgery, are determined by tumour location, its grade of differentiation, and patients age. This article presents case report of immature paratracheal neck teratoma in a 3-month old child, subjected for second-look surgery for residual tumour removal due to the risk of life-threatening complications.


2021 ◽  
Vol 100 (3) ◽  
pp. 141-146
Author(s):  
R.S. Oganesyan ◽  
◽  
N.S. Grachev ◽  
◽  

The variety of forms and localizations of lymphatic malformations (LMs) in the head and neck region determine the lack of unified approach to the treatment of this pathology. The surgical approach is divided into minimally invasive sclerotherapy technique, used mainly for macrocystic forms of LM, and classical surgery, which includes a wide range of surgical interventions on organs and structures of the head and neck. A combination of these two methods and planning of surgical staging are the key to success in the treatment of mixed forms of LMs and malformations of complex localizations with extensive distribution.


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