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The rare syndrome of the agnathia with microstomia, aglossia, synotia (the external ears approaching one another in the midline) and brain malformation (agnathia-holoprosencephaly) was reported by Pauli et al . (1983) as a developmental field defect. This syndrome has two subgroups. One is more severe with brain malformation (holoprosencephaly), and the other is less severe without brain malformation. This report presents a long surviving case of this syndrome without brain malformation.

Summary A giant h˦mangioma associated with a thrombocytopenia is a rare syndrome (eleven previously reported cases). This case was treated successfully by radical removal of the h˦mangioma. Removal of the h˦mangioma seemed to cure the thrombocytopenia.

Maffucci (1881) described a rare syndrome of mesenchymal dysplasia which consisted of multiple haemangiomata of the soft tissues and multiple enchondromata of the long bones. Lewis and Ketcham (1973) collected 105 cases in the literature and discussed the functional and neoplastic significance of the condition. Since then there have been isolated reports (kennedy, 1973; Loewingeret al., 1977) of this condition, including one which involved a Chinese female (Chenget al., 1981). We wish to present two additional cases and describe how we dealt with their main problems.

The rare syndrome which includes ptosis, blepharophimosis and epicanthus inversus is recorded in one family through five generations. The genetic significance of the syndrome is emphasised and the literature on the surgical management of the condition reviewed.

This rare syndrome was first reported by Trelat in 1869. Subsequently, about 30 cases have appeared in the literature (Smith 1970). The cardinal features are a cleft palate, with or without a cleft lip, salivary pits, popliteal webs and toe-nail dysplasia. Other anomalies may also appear, such as ankyloblepharon, syndactyly, skeletal defects of the lower limb, hypoplastic genitalia, abnormal scalp hair and intercrural pterygium.