Familial Tarsal and Carpal Synostosis with Radial-Head Subluxation (Nievergeltʼs Syndrome)

Pearlman, Hubert S.; Edkin, Robert E.; Warren, Robert F.

doi:10.2106/00004623-196446030-00015

Cited by 37 publications

(11 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Most have described synostosis of the navicular with one cuneiform bone [Lusby, 1959;Gregersen, 1977;Miki et al, 1985;Wiles et al, 1988;Sato and Sugiura, 19901. Mesomelic dysplasia, Nievergelt type is the only previously described type which has carpal and tarsal synostoses as consistent findings [Pearlman et al, 1964;Dubois, 1970;Hess et al, 19781. However, the other features appear to be distinctly different from those of our cases.…”

Section: Normalsupporting

confidence: 52%

Dominant mesomelic dysplasia, ankle, carpal, and tarsal synostosis type: A new autosomal dominant bone disorder

1992

View full text Add to dashboard Cite

A new type of mesomelic dysplasia was in 3 generations of a large Thai family. It is characterized by bilateral symmetrical marked shortening of the ulnae and shortening and bowing of the radii. The proximal fibula is usually short and synostoses are present between the tibia and fibula and the small malformed calcaneus and talus. The prominent calcanei on the ventral surfaces of the distal fibulae are a characteristic feature of the new type. Carpal and tarsal synostoses are present in some affected people. All affected individuals walk on the tips of their toes with the dorsal foot deviated laterally. The deformities of the radius and ulna somewhat resemble those of mesomelic dysplasia, Langer type, but otherwise the condition is distinctly different. This new mesomelic dysplasia is an autosomal dominant trait with complete penetrance and variable expressivity over 3 generations.

show abstract

Section: Normalsupporting

confidence: 52%

Dominant mesomelic dysplasia, ankle, carpal, and tarsal synostosis type: A new autosomal dominant bone disorder

1992

View full text Add to dashboard Cite

show abstract

“…Kassner et aL (1976) described three patients with proximal symphalangism, metacarpophalangeat synostoses, tarsal and carpal fusions and humeroradial synostoses in three generations of a family. Moreover, the authors pointed out the fact that two members of this family were previously reported by Pearlman et al (1964) as examples of the Nievergelt syndrome. But, because these patients did not have the mesomelic dysplasia that is an integral part of the Nievergelt syndrome, they proposed that the term "Nievergelt-Pearlman syndrome" should be dropped.…”

Section: Discussionmentioning

confidence: 78%

A case of multiple synostoses syndrome

Tsuruta

Yamazaki

1980

Jap J Human Genet

View full text Add to dashboard Cite

“…Although it often has an autosomal dominant inheritance, sporadic inheritance has also been documented. Classic features include limited motion of the elbow and forearm with associated carpal coalitions 44 : Similar manifestations may also be seen in the feet including clubfeet and metatarsal synostosis. 44 …”

Section: Nievergelt's Syndromementioning

confidence: 90%

Carpal Coalitions and Metacarpal Synostoses

Gottschalk

Danilevich

Gottschalk³

2016

Hand (New York, N,Y.)

View full text Add to dashboard Cite

Background: Carpal coalition and metacarpal synostosis are uncommon congenital anomalies of the carpus and hand. Methods: A comprehensive review of the literature was performed to help guide surgical and non-surgical treatment of carpal coalition and metacarpal synostosis. Results: The embryology, epidemiology, medical and surgical management, and associated outcomes are detailed. Conclusions: Most patients with these disorders will likely benefit from conservative measures. Surgery should be considered in patients with pain and limitations in wrist and hand function.

show abstract

Familial Tarsal and Carpal Synostosis with Radial-Head Subluxation (Nievergeltʼs Syndrome)

Cited by 37 publications

References 0 publications

Dominant mesomelic dysplasia, ankle, carpal, and tarsal synostosis type: A new autosomal dominant bone disorder

Dominant mesomelic dysplasia, ankle, carpal, and tarsal synostosis type: A new autosomal dominant bone disorder

A case of multiple synostoses syndrome

Carpal Coalitions and Metacarpal Synostoses

Contact Info

Product

Resources

About