Lymphatic malformations of the head and neck: A retrospective review and a support 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 prognosis of the malformation, including preoperative and postoperative complications, long‐term sequelae, and persistence of the disease to improve parental counseling and evaluate the outcome of a surgical treatment of such tumors. 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. Their charts were reviewed retrospectively, with a special focus on the anatomic 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 categories according to their unilateral or bilateral infrahyoid and/or suprahyoid locations. Results Stage I (unilateral infrahyoid): nine patients, 11% of complications (without mediastinal extension: 0%, with mediastinal extension: 50%); stage II (unilateral suprahyoid): three patients, 33% of complications; stage III (unilateral suprahyoid and infrahyoid): eight patients, 75% of complications; stage V (bilateral suprahyoid and infrahyoid): two patients, 100% of complications. None of the children was initially seen with stage IV (bilateral suprahyoid) or stage VI (bilateral infrahyoid) LM. Overall complications, preoperative complications, postoperative com‐ plications, and long‐term morbidity showed a significant in‐ crease 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 location can be reliably used for prognostic purposes, allowing a more accurate assessment of the global risk of complications and determination of surgical outcome. Mediastinal extension in stage I patients seems to be associated with a higher rate of complications. Such information can be used to inform parents more appropriately regarding the management and long‐term prognosis of their children's malformation. © 2001 John Wiley & Sons, Inc. Head Neck 23: 326–337, 2001.