Cutaneous signs of spinal dysraphism. Report of a patient with a tail-like lipoma and review of 200 cases in the literature.

The term "spinal dysraphism" was coined in 1940 by Dr Lichtenstein to designate incomplete fusion or malformations of structures in the dorsal midline of the back, particularly congenital abnormalities of the vertebral column and spinal cord. Raphes develop on the face and head, brancheal arches, sternum, and spinal column. When dysraphism occurs in these sites, failure of closure of fontanelles, cleft lip and palate, brancheal cysts, and abnormalities of the ribs and spine result. A review of 200 cases of occult spinal dysraphism showed the condition to be more common in female patient and to be associated with cutaneous signs in more than 50% of instances. The age at which neurologic symptoms appeared in recorded cases is from birth to 76 years, the average being three years. A case of spinal dysraphism with a tail-like cutaneous structure is presented. The cutaneous manifestations accompanying spinal dysraphism that may lead to early recognition of this syndrome and early institution of treatment are discussed.

Management of the acute abdomen in children with leukemia.

With the increase in remission and survival in children with acute leukemia, the complication of acute abdomen has become increasingly important in the total care of these children. A total of 286 children with acute leukemia was treated at Memorial Hospital between 1966 and 1971. Sixteen or 5.6% of these children developed an acute abdomen during the course of their disease. During the same period, many other children developed symptoms and signs mimicking an acute abdomen. Of the 9 children treated surgically, 5 were long-term survivors. Two children treated conservatively died, and 5 patients were diagnosed at autopsy. Prognosis was better in those patients in remission at the time of surgery, and where remission was maintained by immediately restarting antileukemic chemotherapy. A more aggressive surgical approach to the acute abdomen, combined with careful supportive measures, is further adding to the numbers of long-term survivors in childhood leukemia.

Nevus lipomatosus cutaneus superficialis (Hoffmann-Zurhelle).

We are reporting seven cases with nevus lipomatosus cutaneus superficialis. A review of the literature indicates that this is a relatively rare type of connective tissue nevus with little more than 50 cases recorded. Localization of the cutaneous nodular lesions over the upper thigh and buttock and histologic findings of fat lobules within the substance of the nevoid malformation differentiate these lesions from other varieties of the connective tissue nevi.