Occult, life-threatening, cardial tumor in syndactylism in Gorlin Goltz syndrome.
Pediatric, orthopedic, plastic, and hand surgeons perform the surgical correction of syndactylism. It is sometimes forgotten, however, that syndactylism can be part of a syndrome. The components of such a syndrome can each be life threatening. A 7-month-old boy was hospitalized for correction of cutaneous syndactylism. The mother claimed that, with the exception of the syndactylism, her son was healthy. However, review of the admission documents found that the family physician suspected Gorlin Goltz syndrome. A preoperative echocardiography showed 3.9-cm intramural tumor in the wall of the left ventricle. Electrocardiography documented ventricular tachycardia. Because of the danger of life-threatening malignant ventricular tachycardia, the tumor was resected. Before the resection, cardiac transplantation was considered because of the size of the tumor. Histologic examination found a fibroma. When observing syndactylism, one must always be aware of the possibility of a syndromic disease. It is particularly important that screening investigations, including electrocardiography and echocardiography, are performed before surgical treatment.
Department of Pediatric Surgery, Universitätsklinikum Jena, Jena, Germany.
Th
T
Doede
J
J
Seidel
F-T
FT
Riede
L
L
Vogt
F-W
FW
Mohr
F
F
Schier
D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis* / Q000601:surgery; D004452:Echocardiography; D004562:Electrocardiography; D005350:Fibroma / Q000473:pathology / Q000601:surgery; D005385:Fingers / Q000002:abnormalities*; D006338:Heart Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D013576:Syndactyly / Q000175:diagnosis*