IADR Abstract Archives
Full-mouth rehabilitation of a Triple A syndrome patient
Objectives: Triple A syndrome (ORPHA:869; OMIM:231550) is a rare autosomal recessive disorder caused by mutations on chromosome 12q13. The aim of this paper was to report a 21-year-old Tunisian male with Triple A syndrome and present, step by step, his successful full-mouth rehabilitation.
Methods: A 21-year-old female, born to clinically healthy and consanguineous parents, was referred to the removable partial denture department for a full mouth rehabilitation. The patient’s medical history included achalasia revealed after an X-ray examination after barium ingestion which showed a very slow barium passage through the esophagus and a severely constricted distal esophagus. Since then, he is being symptomatically treated and closely monitored. Few years later, other symtomps were frequently reported: poor concentration, severe fatigue, moodiness and anxiety. Cortisole hormone test revealed an adrenal insufficiency which is being treated with hydrocortisone. Eyes's dryness complaints were confirmed by a negative Schirmer's test and alacrimia is being treated with artificial tear.The physical examination revealed a low body mass index due to frequent vomiting, matication incapacity and consequent swallowing difficulties. Oral examination showed xerostomia, oral hyperpigmentation, hypoplasia and a decreased occlusion vertical dimension resulting from the absence of several permanent teeth.
Results: The maxillary rehabilitation consisted of an association between tooth-supported fixed partial prosthesis and distal-extension removable partial prosthesis with extracoronal resilient attachments in order to provide vertical resiliency, facilitate the replacement of worn attachments and remarkably enhance the esthetics.The mandibular rehabiliation consisted of a tooth-supported removable partial prosthesis in order to provide sensory feedback, preserve the alveolar ridge, improved the stability and enhance the retention thanks to customized ball attachments.
Conclusions: According to the systematic review of literature: "The therapeutic treatment and prosthetic rehabilitation of Triple A syndrome patients", conducted by Pr Mansour Lamia et al. in 2017, this is one of the very first reports presenting the successful full-mouth rehabilitation of a Triple A syndrome patient.
Methods: A 21-year-old female, born to clinically healthy and consanguineous parents, was referred to the removable partial denture department for a full mouth rehabilitation. The patient’s medical history included achalasia revealed after an X-ray examination after barium ingestion which showed a very slow barium passage through the esophagus and a severely constricted distal esophagus. Since then, he is being symptomatically treated and closely monitored. Few years later, other symtomps were frequently reported: poor concentration, severe fatigue, moodiness and anxiety. Cortisole hormone test revealed an adrenal insufficiency which is being treated with hydrocortisone. Eyes's dryness complaints were confirmed by a negative Schirmer's test and alacrimia is being treated with artificial tear.The physical examination revealed a low body mass index due to frequent vomiting, matication incapacity and consequent swallowing difficulties. Oral examination showed xerostomia, oral hyperpigmentation, hypoplasia and a decreased occlusion vertical dimension resulting from the absence of several permanent teeth.
Results: The maxillary rehabilitation consisted of an association between tooth-supported fixed partial prosthesis and distal-extension removable partial prosthesis with extracoronal resilient attachments in order to provide vertical resiliency, facilitate the replacement of worn attachments and remarkably enhance the esthetics.The mandibular rehabiliation consisted of a tooth-supported removable partial prosthesis in order to provide sensory feedback, preserve the alveolar ridge, improved the stability and enhance the retention thanks to customized ball attachments.
Conclusions: According to the systematic review of literature: "The therapeutic treatment and prosthetic rehabilitation of Triple A syndrome patients", conducted by Pr Mansour Lamia et al. in 2017, this is one of the very first reports presenting the successful full-mouth rehabilitation of a Triple A syndrome patient.