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Am. J. Biomed. Sci. 2020,12(2),91-98;doi:10.5099/aj200200091
Received:13 November 2019; | Revised:11 December 2019; | Accepted:03 June 2020

 

Arthrogrypotic Syndrome is the Usual Misnomer in Children with Du Pan Syndrome

 

Ali Al Kaissi MD, MSc1,2*, Vladimir Kenis MD3, Sami Bouchoucha MD4 , Mohammad Shboul PhD5, Franz Grill MD2, Hamza Al Kaissi6, Rudolf Ganger PhD2, Susanne Gerit  Kircher MD, MSc7

1 Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, First Medical Department, Hanusch Hospital, Vienna, Austria

2 Orthopaedic Hospital of Speising, Paediatric Department, Speisinger Str. 109, Vienna-1130, Austria

3 Pediatric Orthopedic Institute N.A. H. Turner, Department of Foot and Ankle Surgery, Neuroorthopaedics and Systemic Disorders, Parkovaya Str., 64-68, Pushkin, Saint-Petersburg, Russia

4 Department of Pediatric Orthopedic Surgery, Children Hospital, Tunis, Tunisia

5 Department of Medical Laboratory Sciences, Jordan University of Science and Technology, Irbid 22110, Jordan

6 Erasmus Medical University of Rome-  Tor Vergata Medicine and Surgery-Italy

7 Institute of Medical Chemistry, Medical University of Vienna, Austria

*Corresponding Author

Dr. Ali Al Kaissi

Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling

First Medical Department, Hanusch Hospital. Heinrich Collin Str. 30 A-1140. 2Orthopaedic Hospital of Speising

Speisinger strasse 109 A-1130, Vienna

Austria.

Tel: +43180182 - 1260

Fax: +43180182 - 1494

Email: ali.alkaissi@oss.at

 

Abstract

Objective: The term arthrogrypotic syndrome is of common usage by different medical disciplines.  The hallmarks of acromesomelic dysplasia Du Pan Syndrome are characteristic facial features, severe growth deficiency and multiple contractures.

Material and Methods: Seven children (four females and three males) age range of (1-10 years) were sought in our departments from the period of 1994-2018. All patients were given the diagnosis of arthrogryposis in other institutions. Though, all demonstrated the classical features of abnormalities which are consistent with the diagnosis of Du Pan syndrome.

Results: The distinctive clinical and radiological phenotype was consistent with Du Pan syndrome. Strikingly, multiple dislocations along the hips and knees, elbows and ankle joints were detected which gives the false impression of arthrogrypotic syndrome.

Conclusion: Unfortunately, on the one hand, the terms arthrogrypotic and or amyoplasia syndrome still prevails in many medical disciplines. The clinical phenotype is the corner stone in establishing diagnoses in the vast majority of children who are born with multiple contractures. On the other hand, it is mandatory to search for extra-skeletal malformations in patients with Du Pan syndrome.

 

Keywords: Arthrogryposis; Du Pan syndrome; Radiology

 

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