基底細胞母斑症候群8例および本邦における報告例の検討

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Eight cases of basal cell nevus syndrome (BCNS) are reported with a review of 95 cases in the Japanese literature. In contrast to equal sex distribution in the literature, the male to female ratio in our cases was 6 to 2. Five patients were in the 2nd decade and 3 in the 5th decade. Including our patients, the mean age of the patients in the literature was 24 years. Multiple jaw cysts were seen in 82.0% of them, which was followed in order of frequency by ocular hypertelorism (79.6%), frontal and parietal bossings (69.9%), calcification of the falx cerebri (66.0%), pits of hands and feet (63.1%) and so forth. The presence of jaw cysts which included 31 primary cysts and 10 recurrent cysts with or without multiple recurrences was also constantly found in our patients. All but one had multiple cysts. Of the 31 primary cysts, 21 occurred in the mandible and 10 in the maxilla.<BR>Radiographically, the cysts were unilocular in 32 cysts (78%), unilocular with marginal scalloping in 5 cysts (12%) and multilocular in 4 cysts (10%). The primary cysts were accompanied by unerupted teeth in 11 cysts (35%) and congenitally missing teeth were found in the affected area in 12 cysts (40%). The latter findings were characteristic of the cysts associated with BCNS. Excluding 3 untreated cysts, marsupialization alone, marsupialization followed by enucleation, and enucleation and primary closure or packing open were performed respectively for 9 (32%), 4 (14%) and 15 (54%) primary cysts. Of 27 primary cysts with at least 6 months follow-up, recurrences were noted in 7 cysts (26%) during the period of 10 months to 12 years and 10 months postoperatively. In all cases the recurrences were found in patients in the 2nd decade. The rate of recurrence was significantly higher in cysts with unerupted teeth. The failure to remove the cysts in one piece seemed to be the potential factor leading to recurrence. Histologically, the presence of epithelial islands and/or daughter cysts were observed in 13 cysts (34%) and mitotic figures seen in the basal layer of the lining epithelium was 1.2/5 HPF.
社団法人日本口腔外科学会の論文

基底細胞母斑症候群8例および本邦における報告例の検討

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