Oculodentodigital dysplasia: plastic treatments and self-esteem estimation
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CASE REPORT
Oculodentodigital dysplasia: plastic treatments and self-esteem estimation Ning Chai 1 & Zhongliang Lang 1 & Minggang Wang 1 & Yanjun Chu 1 Received: 16 August 2019 / Accepted: 28 October 2019 # Springer-Verlag GmbH Germany, part of Springer Nature 2019
Abstract Oculodentodigital dysplasia is a rare autosomal dominant inherited syndrome which can influence the developments of multiple organs and tissues in embryo and adults. It can cause the abnormities in ophthalmological, dental, skeleton, and other systems like neurological system, etc. The manifestations of ODDD were in highly various; most of patients, however, were easily recognized by the typical facial characteristics since the eye–teeth–nose were involved. The facial appearances of patient were affected along with the development of selfesteem. In this report, a 23-year-old female was referred to our constitution with classical ODDD facial manifestations. Considering the age, complications, and personal facial condition, we performed epicanthoplasty, lateral canthotomy, and correcting procedures for both sides of 3rd and 4th fingers syndactyly simultaneously. In addition, we used Rosenberg self-esteem scale (RSES) to evaluate the selfesteem level before the surgery, 9 days after surgery (suture removed) and 1 month after surgery, respectively. The outcomes showed that self-esteem was higher in postoperative stage. The patient was satisfied with the cosmetic outcomes as well. Level of evidence: Level V, therapeutic study. Keywords Oculodentodigital dysplasia . Facial appearances . Plastic operation . Teenagers . RSES . Self-esteem
Oculodentodigital dysplasia: plastic treatments and self-esteem estimation Oculodentodigital dysplasia (ODDD) is an extremely rare developmental disorder which can affect multiple organs and systems of the body. Since, it was first reported by Lohmann in 1920 [1]. It was estimated that only 300 of such patients have been reported internationally so far [2]. The prevalence of this disorder was around 1 in 10 million [2, 3]. ODDD patients have a broad spectrum clinical presentations due to the mutation in the connexin 43 gene (Cx43), also referred to as gap junction alpha 1 gene [3]. Symptoms include microphthalmus, microcornea, congenital cataract, glaucoma and reduced vision, typical fourth–fifth fingers syndactyly (type III syndactyly), clinodactyly, and small finger middle phalanx absence and camptodactyly. As for oral region, the patients can present
* Yanjun Chu [email protected] 1
Department of Plastic and Burn Surgery, Anhui Provincial Hospital of Anhui Medical University, 17th Lujiang road Hefei Anhui China
multiple hypoplasia of the tooth enamel, microdontia, caries, and odontodysplasia [4–6]. Recently, some reports demonstrated that some specific neurological symptoms may be related with ODDD, such as lower limb spasticity, progressive leukencephalopathy, epilepsy, neurogenic bladder, and hemisensory loss [7, 8]. As for craniofacial features, narrow nose, hypoplastic alae with a long nasal bridge, cl
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