Vanishing testes syndrome associated with diabetes mellitus
A 30-year-old man complaining of polyuria, polydipsia and asthenia was seen in consultation with a plasma glucose level of 3.97 g/l. He was discovered diabetic 1 year ago and treated with glibenclamide, which was stopped by the patient after 5 months because of lack of money. There was no family history of diabetes. Physical examination showed an eunuchoid status man with micropenis, hypoplastic scrotum with absence of testes confirmed by ultrasonographic exploration. Three months treatment with glibenclamide felt to normalise plasma glucose level and metformin was added to the treatment. Association of glibenclamide and metformine at maximal doses did not normalise the plasma glucose level which was well controlled by adding intermediate acting insulin two times a day.
This patient presented a type 2 diabetes with need of small dose of insulin to get tight glyceamic control. His clinical phenotype is compatible with vanishing testes syndrome. In our condition of practice, genetic investigation was not available. This case is with no doubt a case of type 2 diabetes associated with vanishing testes syndrome. Several genetic syndromes associated with diabetes mellitus has been described but vanishing testes syndrome rarely. The clinical particularity observed in this case of diabetes associated with a genetic syndrome is the need of insulin to achieve good glyceamic control like what is observed in some cases of MODY.