Life-threatening anomalies of the thoracic duct: anatomic delineation dictates management.
Congenital anomalies of the thoracic duct are rare, poorly characterized, and difficult to manage. The spectrum of pathophysiologic perturbations, presenting symptoms, radiographic findings, and interventions performed in 4 patients are shown. Accurate anatomic delineation of the malformation was only possible by direct injection contrast lymphangiography. Therapies tailored to address the anatomic aberrations included intralesional sclerotherapy, surgical excision and ligation, lymphovenous anastomosis, and omental interposition to interrupt dysfunctional collateral lymphatics to the lung. Accurate anatomic diagnosis of central lymphatic channel anomalies by contrast lymphangiography facilitates an individualized multidisciplinary approach to repair.
Fishman
S J
SJ
Department of Surgery, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Burrows
P E
PE
Upton
J
J
Hendren
W H
WH
D000293:Adolescent; D002648:Child; D002916:Chylothorax / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D016638:Critical Illness; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007813:Laparotomy / Q000379:methods; D008297:Male; D018570:Risk Assessment; D012720:Severity of Illness Index; D013897:Thoracic Duct / Q000002:abnormalities* / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome