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Oral ulceration in Stüve-Wiedemann syndrome: a new presentation
  1. Kiran Singh,
  2. Khaled Borghol,
  3. Rhodri Williams and
  4. Kevin McMillan
  1. Oral and Maxillofacial Surgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
  1. Correspondence to Dr Kiran Singh; kirandeep.singh2{at}nhs.net

Abstract

Stüve-Wiedemann syndrome (SWS) is a rare, autosomal recessive disorder, causing dysautonomia and multisystem failure. Symptoms include skeletal malformations, restricted joint mobility and desensitisation to pain. Patients with SWS presenting with intraoral lesions are extremely rare and this is probably due to their shortened lifespan. We present a case of a 9-month-old patient who presented to our Oral and Maxillofacial Surgery (OMFS)Unit with a chronic inflamed ulcer affecting the tongue, secondary to trauma from erupting central incisors. We believe that depapillation in conjunction with an increased pain threshold contributed to its development. The patient was successfully treated by extraction of the lower central incisors and intralesional steroid injections under general anaesthetic. This case highlights that patients with SWS can present to the OMFS clinician with oral lesions and that they can be safely managed under general anaesthesia.

  • anaesthesia
  • dentistry and oral medicine
  • genetics
  • paediatrics
  • congenital disorders

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Footnotes

  • Contributors KS, KB, RW, KM were directly involved in the patient’s care. KS, KB, RW, KM contributed to writing the manuscript. KS, KB, RW, KM proofread the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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