Perioperative Considerations for a Patient with Juvenile Idiopathic Scoliosis and Kaposiform Lymphangiomatosis Undergoing Spinal Fusion: A Case Report

Abstract

Introduction: Kaposiform lymphangiomatosis is a lymphatic anomaly that, when symptomatic, presents in children at a median age of 6.5 years. Symptoms may include respiratory issues, palpable masses, and hemostatic abnormalities. Kaposiform lymphangiomatosis can be life-threatening due to diffuse tissue expansion and subsequent invasion of surrounding organs and tissues. Initial detection of this rare condition can be difficult due to the varied nature of its presenting symptoms and the overall lack of familiarity of clinicians with the condition. Consequently, misdiagnoses can occur, such as pneumonia, cancer, or other vascular anomalies. When considering operative treatment in patients with kaposiform lymphangiomatosis, meticulous pre-operative planning and multidisciplinary care are required due to the high risks of morbidity and death from blood loss. To the best of our knowledge, this is the first reported case of kaposiform lymphangiomatosis in a patient with juvenile idiopathic scoliosis.

Case report: We present the case of a 13-year-old boy diagnosed with kaposiform lymphangiomatosis at age 8 who, after being followed for several years in our orthopedic clinic for worsening juvenile idiopathic scoliosis despite brace wear, underwent posterior spinal fusion with minimal complications (i.e.., minor cerebrospinal fluid leak). The patient had also been followed for several years before his kaposiform lymphangiomatosis diagnosis for unexplained thrombocytopenia, fatigue, and joint pain. Interdisciplinary care involved multiple specialist teams to choose appropriate pre-operative medications, induction protocol, and bone graft.

Conclusion: Successful operative treatment in a patient with kaposiform lymphangiomatosis can be achieved with the involvement of an interdisciplinary team, anticipation and preparation for cardiac and pulmonary complications via chest tubes and pericardial windows, setting goal parameters to guide intraoperative monitoring, and ceasing medications such as sirolimus to prevent wound-related complications. Given the lack of a current standard of care for managing patients with kaposiform lymphangiomatosis who have spinal deformity, this report can serve as a guide that sets a benchmark for the management of similar cases.

Keywords: Juvenile idiopathic scoliosis; kaposiform lymphangiomatosis; pre-operative planning.

Figure 1

Radiographs of a 6-year-old boy with kaposiform lymphangiomatosis and juvenile idiopathic scoliosis. Anteroposterior images were taken before and after posterior spinal fusion from T2 to L2 and show (a) pre-operative scoliosis and (b) post-operative deformity correction. Lateral images show (c) pre-operative scoliosis and (d) post-operative deformity correction.