Lymphatic malformations: A proposed management algorithm
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.
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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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