Oromandibular-limb hypogenesis syndromes: a case of aglossia with an intraoral band.

Abstract

Oromandibular and limb syndromes feature primarily in sporadic case reports because of their low incidence. They include Moebius syndrome, aglossia-adactylia syndrome, Hanhart syndrome, glossopalatine ankylosis syndrome, limb deficiency-splenogonadal fusion syndrome and Charlie M. syndrome. There is confusion in the classification of these patients because of the associated anomalies and the frequency of overlapping features. This paper presents a patient with oromandibular malformations associated with major defects in the upper and lower limbs. Aglossia in the presence of an intraoral band is a peculiar association demanding classification. This case confirms that aglossia-adactylia syndrome and the glossopalatine ankylosis syndrome are variants along a spectrum.

• Authors: F R Grippaudo, D C Kennedy
• Journal: British journal of plastic surgery (Br J Plast Surg) Vol. 51 Issue 6 Pg. 480-3 (Sep 1998) ISSN: 0007-1226 [Print] England
• PMID: 9849371 (Publication Type: Case Reports, Journal Article)

Topics

• Abnormalities, Multiple (classification)
• Child
• Humans
• Limb Deformities, Congenital (classification)
• Male
• Mandible (abnormalities)
• Mouth Abnormalities (classification)
• Tongue (abnormalities)