Surgical excision as primary treatment modality for extensive cervicofacial lymphatic malformations in children

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Abstract

Objective

There has been much recent focus on sclerotherapy treatment of lymphatic malformations with OK432. Surgical treatment however can have a number of advantages, including complete curative excision. The aim of this study was to evaluate the results of surgical excision as the primary (first) treatment for this condition. This group includes a number of children with very extensive disease as well as some with smaller lesions.

Methods

Prospectively collected database with additional information from medical records of children with cervicofacial lymphangiomas treated over 10 years at a tertiary paediatric referral centre. For this study only children who underwent surgical excision as the primary treatment modality were included.

Results

Total of 118 children with lymphatic malformations were treated under the care of the senior author over a 10 year period. Of these 53 patients, who underwent surgical excision as the primary treatment modality for cervicofacial lymphatic malformations were included in this study. Forty-one patients who underwent sclerotherapy as the initial treatment were excluded. Also excluded were 6 patients who underwent thoracic surgery and 18 who were treated conservatively. The majority of the patients (41, 77.3%) underwent only a single surgical procedure (36 – surgical excision, 5 – laser excision). At the first follow up after the primary surgery, the result was complete resolution of symptoms in 29 patients, near complete resolution in 13 patients (together 79.3%) and partial response in 11 (19.7%) patients. Twenty-three patients with disease localised only to the neck, all (100%) had a complete or near complete resolution of the disease after the primary surgery. Complete/near complete response was achieved in 98% cases with macrocystic disease, regardless of the location. Minor complications occurred in 11.3% patients. No permanent nerve weaknesses occurred.

Conclusion

Cervicofacial lymphatic malformations in children should be managed in a multidisciplinary setting. Surgery remains a very important treatment modality. The majority of patients (80%) in this study had complete or near complete resolution with one surgical procedure. Isolated neck lesions have the best outcomes (100% resolution in this study). Patients with macrocystic disease, achieved complete or near complete resolution in 97% of cases, regardless of the location. Some children with extensive disease will need multiple treatments. Surgical excision as the primary treatment modality in selected cases is safe and reliable technique and has good aesthetic and functional outcomes in experienced hands.

Introduction

Lymphatic malformations are low flow vascular anomalies, the incidence being 1:5000 at birth [1]. These comprise 6% of all paediatric soft tissue tumours [2]. Morphologically these consist of cyst like dilated vascular channels filled with lymph [3]. Lymphatic malformations are classified according to cyst size into macrocystic (few cysts which are at least 2 cm3 in size), microcystic (numerous small cysts which are less than 2 cm3 in size) or mixed [4], [5]. Lymphatic malformations usually present as slow growing soft swellings in the head neck region [6]. They can rapidly increase in size as a result of bleeding or infection, or may rarely decrease in size spontaneously [7]. Lymphatic malformations can be asymptomatic or may affect breathing or swallowing, depending on their size or location.

The treatment modalities for lymphatic malformations can be divided into active observation, sclerotherapy [8], [9], [10], surgical excision [11] or a combination of these. Surgical excision has the advantage of usually a single procedure to excise the disease. It offers potential complete eradication of the disease and a lifetime cure. Sclerotherapy will always leave residual malformation.

Review of the literature on this topic revealed many articles on surgical treatment as a part of multimodality treatment or as a salvage treatment for sclerotherapy failure, but only a few articles reporting on results of surgery as the only treatment. The aim of this study was to evaluate the results of a large series of cervicofacial lymphatic malformations in children treated by surgical excision as the primary (first) treatment at a tertiary paediatric referral centre.

Section snippets

Methods

This study was conducted at the department of paediatric otolaryngology at Great Ormond Street Hospital, London. All children with lymphatic malformations under the care of the senior author (BEJH) from January 2001 to December 2010 were evaluated. The data was collected from a prospectively collected database with supplemental information from medical records. These patients were managed in a multidisciplinary setting. The diagnosis was made on clinical basis and confirmed with MRI scan in all

Results

On reviewing the records, a total of 118 children with lymphatic malformations were treated at paediatric otolaryngology department at Great Ormond Street Hospital, under the care of the senior author over a 10 year period. Of these patients 53 underwent surgical excision as the primary treatment modality for cervicofacial lymphatic malformations (Fig. 1). Forty-one patients who underwent sclerotherapy as the initial treatment were excluded. Also excluded were 6 patients who underwent thoracic

Discussion

Cervicofacial lymphatic malformations in children present in the head and neck region in 75% cases [1]. Approximately 50–65% of these are present at birth, 90% are present by 2 years of age and 95% are present by adolescence [12], [13]. These can present with breathing or swallowing difficulties depending on the anatomical site and extent of the lesion. For large lesions in children the choice of treatment is between sclerotherapy and surgical excision. The advantages of surgery are, that the

Conclusion

Cervicofacial lymphatic malformations in children should be managed in a multidisciplinary setting. Surgery remains a very important treatment modality. The majority of patients (80%) in this study had complete or near complete resolution with one surgical procedure. Isolated neck lesions have the best outcomes (100% resolution in this study). Patients with macrocystic disease, achieved complete or near complete resolution in 97% of cases, regardless of the location. Some children with

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    Citation Excerpt :

    This solution causes scarring within the LM, which eventually leads to shrinking or collapse of the malformation (Fig. 1). Percutaneous sclerotherapy has replaced surgery in most cases of macrocystic malformations in the past 30 years (Acevedo et al., 2008; Poldervaart et al., 2009; Bajaj et al., 2011; Furuse et al., 2020; Burrows et al., 2008; Claesson and Kuylenstierna, 2002; Mitsukawa and Satoh, 2012). Macrocystic LMs of moderate size can be easily treated with sclerotherapy.

  • Surgical resection of macrocystic lymphatic malformations of the head and neck: Short and long-term outcomes

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    To date, a direct prospective comparison of these modalities has not been performed, and there are no guidelines to direct treatment [4–7]. This is particularly true in patients with purely macrocystic disease as most of the literature describes the treatment of a heterogeneous sample of patients with both macrocystic and microcystic components [4,8–10]. Surgical resection has been discussed as a mainstay of treatment for MLM at our institution due to historic success and experience.

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