Lymphatic malformations: A proposed management algorithm

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Abstract

Objective

The aim of this study was to develop a management algorithm for cervicofacial lymphatic malformations, based on the authors’ experience in managing these lesions as well as current literature on the subject.

Study design and methods

A retrospective medical record review of all the patients treated for lymphatic malformations at our institution during a 10-year period (1998–2008) was performed. Data collected: age at diagnosis, location and type of lesion, radiologic investigation performed, presenting symptoms, treatment modality used, complications and results achieved.

Results

14 patients were identified. Eight (57%) male and six (43%) female. There was an equal distribution between the left and right sides. The majority (71%) of cases were diagnosed within the first year of life. The majority of lesions were located in the suprahyoid region. The predominant reason for referral was an asymptomatic mass in 7 cases (50%) followed by airway compromise (36%) and dysphagia (14%). Management options employed included: observation, OK-432 injection, surgical excision and laser therapy. In 5 cases (36%) a combination of these were used.

Conclusion

Historically surgical excision has been the management option of choice for lymphatic malformations. However due to the morbidity and high complication rate associated this is increasingly being questioned. Recent advances in sclerotherapy e.g. OK-432 injection have also shown significant promise. Based on experience in managing these lesions as well as current literature the authors of this paper have developed an algorithm for the management of cervicofacial lymphatic malformations.

Introduction

Lymphatic malformations (LMs) are rare benign tumours that result from localized congenital malformations of the lymphatic system. These lesions are diagnosed during infancy in the overwhelming majority of cases and most often present as an asymptomatic mass in the cervicofacial region [1]. The management of LMs remains a challenge. Surgical excision has traditionally been the first line of treatment however due to the close proximity of vital structures this often leads to incomplete excision and recurrence, or damage to vital structures e.g. cranial nerves [2]. More recently the use of OK-432 has gained popularity especially in the management of macrocystic lesions [3]. The authors reviewed the records of all cases treated for LMs at our institution during a 10-year period. Whilst the majority of cases were managed surgically, promising results were obtained with observation alone as well as with OK-432 injection. Based on experience gained in the management of these lesions as well as current literature on the subject the authors of this paper have formulated a proposed management algorithm for LMs.

Section snippets

Patients and methods

After obtaining local ethics committee approval, all the patients diagnosed and treated for LMs at our institution between 1998 and 2008 were identified. A retrospective review of these patients medical records were performed focussing on age at diagnosis, location of lesion, type of LM, radiologic investigation performed, presenting symptoms, treatment modality used, complications encountered and results achieved.

Results

A total of 14 patients were identified (Table 3). The diagnosis was based on clinical examination and radiologic investigations. Eight (57%) patients were male and the remaining six (43%) female. Two cases (14%) were identified during the prenatal period, five (36%) at birth and three (21%) during the first year of life. The remainder of cases (29%) showed some variation regarding age of presentation with a range of 3–7 years of age. There was an equal distribution between the right and left

Discussion

LMs once referred to as either cystic hygroma or lymphangioma depending on cyst size is now more commonly divided into macrocystic, microcystic or combined disease [4]. The reported incidence of these tumours in the literature is quite variable, ranging between 4 per 10 000 births in one study [5] and 1 per 16 000 births in another [6]. The overwhelming majority of LMs occur in the cervical region with an increased incidence on the left side. There is no difference in distribution between the

Conclusion

LMs are essentially benign tumours and the management of these should reflect this. Even though surgery might have been the principal management option in the past, its complication rate is unacceptably high in the management of a benign condition. This combined with the prospect of new innovative management options that have been proven to be effective, have led the authors of this report to formulate a proposed algorithm (Fig. 1, Fig. 2) for the management of LMs.

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