Correction of severe blepharoptosis.

An analysis is made of 81 patients with severe blepharoptosis who underwent correction with autogenous fascia. In case of severe blepharoptosis, which means a levator function of less than 5 mm, correction by levator resection gives insufficient results. Better results are obtained by frontalis suspension, whereby the upper eyelid is connected to the frontalis muscle using fascia lata strips according to Crawford. The operative technique is described in detail. Particular interest has been paid to the width of the vertical lid fissure and symmetry between both upper eyelids after operation. The overall results were rated satisfactory to excellent with an average of 9 mm of postoperative vertical lid fissure. Comparison of the results of unilateral versus bilateral blepharoptosis correction revealed better results in the group of bilateral ptosis correction. In 76% of the bilateral patients, there was an asymmetry of less than 0.5 mm between both palpebral fissures, while in the unilateral group this result was achieved in only 35% of the cases. Symmetry is difficult to achieve in the unilateral group, especially when the vertical lid fissure of the nonptotic eye is 10 mm or more. In these cases, correction of both upper eyelids should be considered. The best time to operate on patients with severe congenital blepharoptosis is around the age of 4 to 5 years, because lagophthalmus after operation is better tolerated and the leg has developed sufficiently to provide an adequate amount of fascia. The correction of severe blepharoptosis by frontalis suspension with autogenous fascia shows a high rate of success and few complications.

Familial cutaneous cylindromas: investigations in five generations of a family.

BACKGROUND: Multiple cutaneous cylindromas are probably inherited in an autosomal dominant way. OBJECTIVE: Our purpose was to describe a large family with cutaneous cylindromas, trichoepitheliomas, and milia occurring in five generations and to elucidate further the mode of inheritance. METHODS: We examined 39 family members and obtained information on 31 other members from reports of relatives. RESULTS: The pedigree included 237 members, 118 male and 119 female, with 30 affected patients (11 male, 19 female). Between 33% and 100% of the children of affected family members had one or more of these skin lesions. Female-to-female, female-to-male, male-to-female, and male-to-male inheritance occurred. CONCLUSION: Multiple cutaneous cylindromas are inherited in an autosomal dominant way with variable clinical expression. Penetrance reaches 100% in adult life. This condition is associated with trichoepitheliomas and milia.

Cutaneous cylindroma with malignant transformation.

BACKGROUND: Malignant cutaneous cylindroma is a rare tumor. It has been described in 26 cases, both in the solitary form and in the autosomal dominant inherited multiple tumor form. The authors present two new cases that occurred in one family with a history of multiple cylindromas. METHODS: Clinical and histopathologic data of both tumors were compared with those of 26 other cases in the literature. Immunohistochemical examinations were performed. RESULTS: The malignant tumors were distinguished from the benign lesions by rapid growth, long-standing ulceration, or bleeding. Histopathologic examination showed a well-differentiated carcinoma in one patient and a poorly differentiated tumor in the other. In the latter, lymph node metastasis developed, and the patient died 2.5 years later. Histopathologic criteria of malignancy included cell pleomorphism, frequent mitoses and loss of jigsaw pattern, peripheral palisading, hyaline sheaths, and dual cell population. CONCLUSIONS: These observations are in accord with those in the literature. Malignant cutaneous cylindroma developed more often in the multiple tumor form than in the single tumor form. Malignant cylindroma is an aggressive carcinoma with a tendency to local destructive growth and metastases.

Synovial hand infection from Mycobacterium terrae.

A case of extensive synovial infection in the hand due to Mycobacterium terrae is described. The infection is resistant to drug therapy but appears to remain localised.