Endocrine Abstracts

Introduction: Solitary median maxillary central incisor syndrome (SMMCI) is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factors and an uncertain etiology. Its incidence is estimated at 1/50.000.

Material and Methods: We report 2 cases of patients with SMMCI associated with GH isolated deficiency in one case and multiple pituitary hormone deficiency in the other one.

Case 1: A 4-year-old girl with no significant medical history consulted for growth retardation at –3.5 S.D. She had a particular physionomy with blond hair, fair skin (contrasting with the skin tone of the parents), baby face and a single maxillary median central incisor. Psycho-motor development was normal. Hormonal investigations concluded to a complete GH deficiency, a central hypothyroidism and a central corticotropic deficit. Hypothalamic MRI showed a pituitary stalk interruption syndrome without any other cerebral anomaly. No other malformation (cardiac, renal, vertebral) was found. She was treated by growth hormone, L-thyroxine and hydrocortisone with satisfying heigt gain and improvement of general condition.

Case 2: The second observation is about a 7-year-old girl with a complete and isolated GH deficiency discovered at the age of 3. She was receiving GH regularly with good results. She had the same particular physionomy than the first girl : blond hair and baby face. This made us re-examine her, so we found also the same dentar anomaly : a solitary median maxillary central incisor.

Notice, the parents didn’t pay attention to this anomaly in the two cases.

Conclusion: The SMMCI is a rare condition that may be associated with midline anomalies, hypopituitarism, holoprosencephaly and other visceral malformations. It constitues also an eshtetic problem that requires interdisciplinary intervention, generally after the eruption of the permanent teeth.