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Introduction: Chondroectodermal dysplasia (Ellis–van Creveld syndrome (EVC)) (OMIM: #225500) is a rare skeletal dysplasia with unknown exact prevalence. EVC patients may have abnormal skeletal and extra skeletal symptoms. We report a case of EVC patient and review previous cases reported in Iran.Materials and methods: The patient was a 30 years old woman referred to our hand clinic for an extra finger in both hands. She was born to a consanguineous marriage. The patient had a history of bilateral valgus deformity of the knees, for which she underwent proximal tibial and distal femoral osteotomy. Upon examination, the patient had bilateral polydactyly and brachymetacarpia with hypotrophic fingernails. She was about 120 cm tall and had acromesomelic type dwarfism. Oral examination revealed serrated incisal margins, dental transposition, a diastema, conical teeth and, enamel hypoplasia. In the radiographic examination of upper extremity, postaxial polydactyly and polymetacarpia, enlarged distal radius, and fusion of capitate and hamate were seen. We searched online databases (Pubmed, Scopus, Google Scholar) and found 14 Iranian papers with 21 reported patients (there was no time limit). We reviewed available clinical and genetic data and the geographic origins of patients.Results: 14 articles reporting 22 EVC patients (including our patient) from Iran have been published in Persian and English (7 in Persian and 7 in English). All patients presented with characteristic EVC symptoms but were diagnosed at a relatively late age, 18 patients were born to consanguineous marriages.Conclusions: It seems that based on these studies, it is possible to identify some families with this genetic mutation. We can warn such families of the dangers of consanguineous marriage through genetic counseling before marriage. In addition, by identifying families with such problems, we can detect such anomalies in the baby earlier with more careful prenatal care.

IntroductionLunate fracture is a rare injury. Most reports are associated with other wrist injuries such as perilunate dislocation and distal radius fracture. Isolated lunate fracture has been reported even more rarely. The choice of treatment and outcomes are consequently undetermined.Case PresentationIn this case report we will describe a lunate avulsion fracture as an isolated injury after a fall from nine meters treated operatively by excision of the comminuted avulsed fragment. After 33 months of follow-up radiographs showed no sign of degenerative joint disorder on simple X-ray, but slight Volar Intercalated Segment Instability (VISI) by a capitolunate angle of 26 degrees was noted. Clinically, the patient was pain free near full wrist and forearm range of motion and could perform his previous vocational and recreational tasks without any limitations.ConclusionsDespite apparently good short and mid-term clinical outcome, slight volar intercalated segment instability after 33 months of follow-up revealed that lunotriquetral ligament function was probably lost, which led to static instability. This ligament injury may be missed primarily. Excision of the avulsed osteochondral fragment should be the last option of treatment and most attempts should be tried to fix and/or restore the normal anatomy of ligamentous structure.