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Objective This project investigates two cases of skeletal dysplasia from archaeological excavations of the New Kingdom Period (c. 1400–1050 BCE) portion of the Tombos cemetery in Sudan. Materials: Fair to well-preserved skeletal remains of two individuals, one adult and one juvenile, are examined here. Methods All available skeletal elements were analyzed macroscopically. A differential diagnosis was conducted for each individual. Results The adult individual, U36.Sh2.B10, displays bilateral mesomelic dysplasia and Madelung’s deformity. The juvenile individual, U36.Sh2.B5, also displays bilateral mesomelic dysplasia and characteristics associated with Madelung’s deformity. Conclusions A differential diagnosis of Leri–Weill dyschondrosteosis (LWD) is suggested for the adult female individual (U36.Sh2.B10). The second case (U36.Sh2.B5) is an approximately three to five-year-old individual and is difficult to diagnose given the young age; however, LWD remains the most likely diagnosis. Significance There are few cases of LWD in the paleopathological literature, and fewer still of juveniles. The cases described are useful examples in expanding research demonstrating the variability in the expression of skeletal dysplasias in juveniles and adults. Limitations Taphonomic alterations and fragmentation of the crania and portions of the postcrania limited the observation of the full suite of characteristics associated with skeletal dysplasias. U36.Sh2.B5 is difficult to diagnose given the individual’s young age and the possibility that this individual had not yet developed the more observable characteristics associated with these conditions. Suggestions for further research Researchers are encouraged to continue examining the range of expression of skeletal dysplasias in juveniles and adults.

Purpose To establish whether Madelung's deformity demonstrates a radiographic continuum of severity and whether a forme fruste does exist. Methods Ulnar tilt, lunate subsidence, palmar carpal displacement, and lunate fossa angle were measured in 81 wrist radiographs with obvious or suspected Madelung's deformity. Statistical analyses based on these measurements were performed to ascertain if there is a deformity continuum. Results Ranges of 15° to 51° (mean, 28°) for ulnar tilt, –7 to +11 mm (mean, –0.8 mm) for lunate subsidence, 9 to 25 mm (mean, 15.3 mm) for palmar carpal displacement, and 20° to 56° (mean, 33°) for lunate fossa angle were obtained. Significant correlations were observed between all measurements. Conclusions Madelung's deformity encompasses a spectrum of radiographic abnormality. Type of study/level of evidence Diagnostic I.

Purpose To evaluate the hypotheses that all Madelung's deformity subjects have dyschondrosteosis (defined as short stature and mesomelia, in addition to Madelung's deformity) and to evaluate the concept that Madelung's deformity may affect the entire radius. Methods A radiographic and medical records review was performed for 26 subjects (46 extremities) with Madelung's deformity. The radiographs were assessed for radius and ulna length, sagittal radial bow, severity of the Madelung deformity, and radiocapitellar joint space. The sagittal radial bow and the radiocapitellar joint space were used to classify subjects according to whether the Madelung deformity was limited to the distal radius or involved the entire radius. Results Thirty-one extremities in 18 subjects were classified as having a distal radius Madelung deformity and 15 extremities in 8 subjects were classified as having an entire radius Madelung deformity. The radius and ulna length and subject height were significantly decreased compared with age- and height-matched normal values in both groups; the entire radius group was more severely affected. In addition, the entire radius group had more severe deformities with respect to lunate subsidence and ulnar tilt. All of the entire radius subjects and 9 of 14 of the distal radius subjects had dyschondrosteosis. Conclusions Madelung's deformity presents as a spectrum. It may affect the entire radius or it may affect only the distal radius. Extremities with involvement of the entire radius have a shorter radius and ulna, decreased height, and a more severe deformity than extremities with involvement of only the distal radius. Additionally, most subjects with Madelung's deformity have dyschondrosteosis. Type of study/level of evidence Diagnostic III.

Purpose Madelung's deformity is usually recognized near the completion of skeletal growth and corrective osteotomy of the radius is frequently performed to treat the deformity and reduce pain. This study reviewed the clinical and radiographic results of a volar approach, ligament release, and dome osteotomy technique for treatment of this deformity. Methods Between 1990 and 2000, 26 wrists in 18 patients were treated with a volar ligament release and dome osteotomy of the distal radius. The average age of the patients at the time of surgery was 13 years. All patients were available for review at an average of 23 months after surgery. Radiographs before surgery and at final follow-up evaluation were analyzed for the extent of correction. Results All patients treated with this corrective osteotomy reported a reduction in pain and improved appearance. Patients showed improvements in forearm supination and wrist extension, with no loss of pronation or flexion. Improvements in the radiographic parameters of radial inclination and lunate subsidence also were observed. Four wrists required concurrent ulnar-sided surgery, and 3 additional wrists required staged ulnar shortening. One patient required a Darrach resection 3 years after the index procedure. Conclusions The results of volar dome osteotomy provide improved range of motion, improved appearance, radiographic correction, and pain relief while preserving soft-tissue support for radial rotation around the distal ulna. Long-term follow-up evaluation is ongoing. Level of Evidence Therapeutic, Level IV.

This article describes a modification of the double-osteotomy radial technique that has been described previously and summarizes our clinical experience with this method in 4 female patients. All patients had improvement in functional motion and were satisfied with the improved appearance of their wrist after surgery.

Madelung's deformity is an uncommon congenital condition of the wrist usually seen in adolescent girls. It first was documented in the 1800s and is characterized by a shortened radius that curves ulnarly and volarly, a prominent ulna head that projects dorsally from the wrist, and a triangular arrangement of the carpal bones. It is mostly an aesthetic deformity although functional problems and pain may prompt surgeons to undertake a variety of corrective surgical procedures with varying degrees of success. This challenging condition is encountered rarely in a hand surgeon's practice. Even more obscure than the condition is the physician it is named after: Otto Wilhelm Madelung, a distinguished and successful German surgeon who lived at the turn of the century. This article provides a historical perspective on the person and the condition that is still an enigma a century later.

A resurgence of interest in Madelung's deformity has developed recently because of improved operations for correction of the deformity, identification of the genetic loci for the condition in certain syndromal variants, identification of an anterior ligamentous structure tethering the carpus, and preventive treatments in growing children. The process is reviewed in this article and a new surgical technique is presented. The procedure is performed by way of an anterior incision that is more cosmetically appealing. The release of an anterior ligamentous structure described by Vickers is performed simultaneously with a dome shaped osteotomy of the radius. The fragments, once alignment is corrected, are stabilized with temporary pin fixation and a long arm cast until the bone has healed.

Resume La maladic de Madelung symptomatique ne repond pas bien au traitement conservateur et les objectifs du traitement chirurgical sont essentiellement d'obtenir un interligne articulaire radio-carpien congruent et bien oriente afin de conserver des amplitudes articulaires maximales et indolores. Les differentes modalites du traitement chirurgical proposees jusqu'ici ont un caractere essentiellement esthetique et ne sont pas suffisantes pour aboutir aux objectifs cites. Les auteurs proposent une nouvelle approche therapeutique de cette deformation : il s'agit d'une arthrodese radio-lunaire et radio-cubitale inferieure avec l'artifice de Kapandji associee a une osteotomie de reorientation du bloc radiocarpien a la demande. Les resultats obtenus dans les quatre premiers cas sont evalues avec un recul clinique de un an et demi a cinq ans.

Dyschondrosteosis is a dominant hereditary bone dysplasia resulting in wrist deformity and mesomelic dwarfism. No previous cases of this condition have been described from North America. This report also includes the first documented case in a male. European authors have distinguished between dyschondrosteosis and Madelung's disease. Clinical and radiologic findings in 5 patients in 2 families presented here and a review of the literature strongly suggest that these two conditons are in fact identical. Dyschondrosteosis would appear to be the preferred designation since, historically, it has included all of the features of the condition.

A 22-year-old woman with Madelung's deformity was treated by radial osteotomy and a Lauenstein procedure. This technique allows near normal restoration of the distal radius architecture, support of the ulnar side of the carpus, maintenance of forearm pronation and supination, and a satisfactory cosmetic result.

Madelung's deformity may be a manifestation of a hereditable autosomal dominant mesomelia known as dyschondrosteosis. All male patients with Madelung's deformity have been noted to have the complete syndrome. We present a case of Madelung's deformity, dyschondrosteosis, and ligamentous laxity in a Negro male, and stress the importance of the complete evaluation and genetic counseling of such patients.

The writers have presented a case of bilateral dyschondroplasia of the distal radial epiphysis (Madelung's deformity) with fusion of the semilunar and triangular bones.