Lymphatic malformations of the head and neck: A retrospective review and asupport for staging

Background. Lymphatic malformations (LM) are rare benign congenital tumors appearing mainly in the head and neck with a considerably variable outcome. A need exists to validate a staging system, taking into account the progno sis of the malformation, including preoperative and postoperative complicat ions, long-term sequelae, and persistence of the disease to improve parenta l counseling and evaluate the outcome of a surgical treatment of such tumor s. Methods. Twenty-two patients treated for LM were selected from a series of 129 patients operated on for congenital malformations of the head and neck between 1986 and 1997 at St-Luc University Hospital, Brussels, Belgium. The ir charts were reviewed retrospectively, with a special focus on the anatom ic location of the lesions and all the complications reported. According to de Serres et al, LM up to now have been divided into six possible categori es according to their unilateral or bilateral infrahyoid and/or suprahyoid locations. Results. Stage I (unilateral infrahyoid): nine patients, 11% of complicatio ns (without mediastinal extension: 0%, with mediastinal extension: 50%); st age II (unilateral suprahyoid): three patients, 33% of complications; stage III (unilateral suprahyoid and infrahyoid): eight patients, 75% of complic ations; stage V (bilateral suprahyoid and infrahyoid): two patients, 100% o f complications. None of the children was initially seen with stage IV (bil ateral suprahyoid) or stage VI (bilateral infrahyoid) LM. Overall complicat ions, preoperative complications, postoperative complications, and long-ter m morbidity showed a significant increase from stage I to V (p < .01, p = . 002, p = .02, and p = .03, respectively). Conclusions. A staging system of cervicofacial LM based on the anatomic loc ation can be reliably used for prognostic purposes, allowing a more accurat e assessment of the global risk of complications and determination of surgi cal outcome. Mediastinal extension in stage I patients seems to be associat ed with a higher rats of complications. Such information can be used to inf orm parents more appropriately regarding the management and long-term progn osis of their children's malformation. (C) 2001 John Wiley & Sons, Inc.