Region Jnkpings ln Odontologiska Institutionen

Prosthodontic rehabilitation of a young woman with Goltz Syndrome/Incontinentia pigmenti - A case report

Sondell K*, Bergendal B, Falahat B


The patient was seen by the paediatric dental care at the age of six. At birth the presence of streaked hypopigmentation of the skin and syndactyly of toes on the left foot was observed. Later impaired eyesight and hearing on the left was diagnosed. In the dentition hypoplasia of enamel and teeth, late eruption and irregular placement were noticed. At the age of twenty a genetic counselling was accomplished and the ectodermic disorders were considered consistent with Goltz Syndrome (1)(3) but with a considerable overlap of Incontinentia pigmenti (2).


This presentation describes the prosthodontic rehabilitation of a young woman with the tentative diagnosis Goltz Syndrome.


The patient has during childhood been treated with a multidisciplinary team approach. Minor clinical interventions were adapted to the patient's dental and psychological stage by paediatric dentistry, dentomaxillofacial radiology and orthodontics. The patient was considered for prosthodontic intervention at the age of twenty. Seven permanent teeth were congenital missing in the left jaws and three deciduous teeth remained. Several teeth in the left jaws were malformed with hypoplastic and hypomineralized enamel. The result of the prosthodontic treatment atthe three-year follow-up is presented. 


A functional and aesthetic habilitation was performed successfully in two stages resulting in implant and tooth supported prosthodontics.


This multidisciplinary team approach has had the advantages of continuity and sound therapy decisions. The overall treatment has been planned and performed over a long period. Thus early extensive irreversible prosthodontic treatment in a growing individual could be avoided.  

Uppdaterad: 2013-04-03
Anna Thofelt, Avdelningen för odontologisk radiologi Jönköping, Folktandvården