Abstract
Children with ulnar longitudinal deficiency present with a rare spectrum of malformations that involve the digits, partial to complete absence of the ulna, carpals, and hypoplasia of related joints, muscles, tendons, nerves, and blood vessels. Ulnar longitudinal deficiency appears to be non-inherited, but it does have a close association with other musculoskeletal anomalies, such as proximal femoral focal deficiency, fibular and tibial ray deficiency, phocomelia, scoliosis, clubfeet, absent patellae, and hip abnormalities. Clinically, the child’s entire upper limb is hypoplastic, and the elbow may be abnormal or fused. Patients most commonly have partial absence of the ulna, but usually with a stable wrist. Patients with ulnar longitudinal deficiency commonly have finger and thumb involvement, and most of their surgical treatment is focused on the function of their hand.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Bauer AS, Bednar MS, James MA. Disruption of the radial/ulnar axis-congenital longitudinal deficiencies. J Hand Surg Am. 2013;38(11):2293–302.
Koskimies E, Lindfors N, Gissler M, Peltonen J, Nietosvaara Y. Congenital upper limb deficiencies and associated malformations in Finland: a population-based study. J Hand Surg Am. 2011;36A:1058–65.
Oberg KC, Feenstra JM, Manske PR, Tonkin MA. Developmental biology and classification of congenital anomalies of the hand and upper extremity. J Hand Surg Am. 2010;35A:2066–76.
Tickle C, Summerbell D, Wolpert L. Positional signaling and specification of digits in chick limb morphogenesis. Nature. 1975;254:199–202.
Tickle C. Experimental embryology as applied to the upper limb. J Hand Surg Am. 1987;12B:294–300.
Yang Y, Kozin SH. Cell signaling regulation of vertebrate limb growth and patterning. J Bone Joint Surg. 2009;91A(Suppl 4):76–80.
Bayne LG. Ulnar club hand (ulnar deficiencies). In: Green DP, Hotchkiss RN, Pederson WC, editors. Operative hand surgery. 3rd ed. New York: Churchill Livingstone; 1993. p. 288–303.
Havenhill TG, Manske PR, Patel A, Goldfarb CA. Type 0 ulnar longitudinal deficiency. J Hand Surg Am. 2005;30A:1288–93.
Kummel W. Die missbildungen der extremitaeten durch defect, verwachsung und ueberzahl. Bibliotheca Medica (Casse). 1895;Heft 3:1–83.
Ogden JA, Watson HK, Bohne W. Ulnar dysmelia. J Bone Joint Surg. 1976;58A:467–75.
Swanson AB, Tada K, Yonenobu K. Ulnar ray deficiency: its various manifestations. J Hand Surg Am. 1984;9A:658–64.
Ogino T, Kato H. Clinical and experimental studies on ulnar ray deficiency. J Pediatr Orthop. 1983;3:37–40.
Cole RJ, Manske PR. Classification of ulnar deficiency according to the thumb and first web. J Hand Surg Am. 1997;22A:479–88.
Goldfarb CA, Manske PR, Busa R, Mills J, Carter P, Ezaki M. Upper-extremity phocomelia reexamined: a longitudinal dysplasia. J Bone Joint Surg. 2005;87A:2639–48.
Buck-Gramcko D. Congenital malformations of the hand and forearm. Chir Main. 2002;21:70–101.
Carter PR, Ezaki M, Oishi S, Herring JA. Disorders of the upper extremity. In: Herring JA, editor. Tachdjian’s pediatric orthopaedics, vol. 4. 1st ed. Philadelphia: Saunders; 2008. p. 536–9.
El Hassan B, Biafora S, Light T. Clinical manifestations of type IV ulna longitudinal dysplasia. J Hand Surg Am. 2007;32A:1024–30.
Miller JK, Wenner SM, Kruger LM. Ulnar deficiency. J Hand Surg Am. 1986;11A:822–9.
Carroll RE, Bowers WH. Congenital deficiency of the ulna. J Hand Surg Am. 1977;2:169–74.
Straub LR. Congenital absence of the ulna. Am J Surg. 1965;109:300–5.
Broudy AS, Smith RJ. Deformities of the hand and wrist with ulnar deficiency. J Hand Surg Am. 1979;4A:304.
Flatt AE. The care of congenital hand anomalies. St Louis: Quality Medical Publishers; 1994.
James MA, Bednar M. Deformities of the wrist and forearm. In: Green DP, Hotchkiss RN, Pederson WC, Wolfe SW, editors. Green’s operative hand surgery. 5th ed. New York: Churchill Livingstone; 2005. p. 1479–83.
Vitale CC. Reconstructive surgery for defects in the shaft of the ulna in children. J Bone Joint Surg. 1952;34A:804–10.
Smith AA, Greene TL. Preliminary soft tissue distraction in congenital forearm deficiency. J Hand Surg Am. 1995;20A:420–4.
Chen GX, Zhou ZA, Yang L. Ulnar lengthening using a half ring sulcated external fixator for ulnar longitudinal deficiency: a case report. Cell Biochem Biophys. 2013;67(2):809–12.
Laurin CA, Farmer AW. Congenital absence of the ulna. Can J Surg. 1959;2:204–7.
Blair WF, Shurr DG, Buckwalter JA. Functional status in ulnar deficiency. J Pediatr Orthop. 1983;3:37–40.
Schachinger F, Girsch W, Farr S. Soft tissue distraction prior to single bone forearm surgery in ulnar longitudinal deficiency: a report of two cases. J Hand Surg Asian Pac. 2018;23(1):153–7.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2021 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Gottschalk, H.P., Bednar, M.S. (2021). Ulnar Longitudinal Deficiency. In: Laub Jr., D.R. (eds) Congenital Anomalies of the Upper Extremity. Springer, Cham. https://doi.org/10.1007/978-3-030-64159-7_11
Download citation
DOI: https://doi.org/10.1007/978-3-030-64159-7_11
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-64158-0
Online ISBN: 978-3-030-64159-7
eBook Packages: MedicineMedicine (R0)