[Hypoglossia-hypodactylia syndrome: apropos of a case with maxillo-mandibular synostosis, glossopalatine ankylosis and cleft palate].

BACKGROUND

The hypoglossia-hypodactylia syndrome, a combination of limb hypogenesis and micrognathia is exceptionally associated with glossopalatine ankylosis and cleft palate.

CASE REPORT: A twin girl from monochorionic pregnancy had hypoglossia and micrognathia with anterior maxillo-mandibular fusion, glossopalatine ankylosis and cleft palate. Her left foot was amputated distal to the talus and calcaneous; her right foot had only one phallange. The second phallange of the second finger was hypoplastic on her right hand. There was no abnormality on left hand. Her sister was unaffected. The maxillo-mandibular fusion was divided on second day. The infant was able to suckle on the tenth day. Episodes of airway obstruction occurred on cardiorespiratory recording and the patient was further monitored at home.

CONCLUSION

This case suggests that the hemodynamic disorders due to multiple vascular connections from monochorionic pregnancies may have induced ischemic lesions of hypoglossia-hypodactylia. Other malformations and signs of brain stem dysfunction should be detected.