APSICON 2024 poster final (1)

AN UNUSUAL PRESENTATION OF A CONGENITAL LONGITUDINAL DEFICIENCY OF UPPER LIMB
Dr Lakshmi Narayanan P - Mch Plastic Surgery Postgraduate

Thanjavur Medical College
Introduction Discussion
Congenital longitudinal deficiencies of the upper Radial longitudinal deficiency are categorized as a malformation of the radioulnar axis in the OMT
limb are divided into radial, central, ulnar, and classification involving a spectrum in which the radial structures of the upper extremity are
intersegmental forms. Radial and ulnar underdeveloped or completely absent.It is an uncommon condition, in nearly 1:5000 live births. According
longitudinal deficiencies are the most common to modified bayne and klug classification type 0 represents deficiency of thumb or carpus in presence of
types, of which radial deficiency is 3 to 4 times normal length radius.It is frequently bilateral and asymmetric(syndromic) but also may be sporadic and
more common. Radial and ulnar deficiencies each isolated. Patients with syndromic association have abnormalities of both radius and thumb.There is a new
demonstrate a spectrum of malformations from modification of blauth classification of thumb hypoplasia Type 6 (absent thumb and index). In our case,
minor digital involvement to complete absence of there is also a type B brachydactyly of middle finger with absent distal phalanx. There is also a possibility
a forearm bone, carpal bones, and digits with
that this case can be a type 0 ulnar longitudinal deficiency with type C hand difference, but the absent
hypoplasia of joints, muscles, tendons, ligaments,
radial artery and underdeveloped thenar muscles made us diagnose this case as a radial longitudinal
nerves, and blood vessels. Radial longitudinal
deficiency.
deficiency is associated with a number of
syndromes while ulnar longitudinal deficiency is
associated with other musculoskeletal anomalies.
Both conditions have a high incidence of ipsilateral
thumb abnormalities. Here we present a case of
unusual presentation of sporadic and isolated type
0 radial longitudinal deficiency with type 6 thumb
hypoplasia and absent radial artery.
Case Description
A 17 year old female presented with deformity of
left hand with absence of 2 fingers and difficulty in
daily activities. Patient was operated for
syndactyly of ulnar side two fingers in childhood.
Patient had no other congenital anomalies. There
is no family history of any congenital skeletal
abnormalities. Physical examination of the right
upper extremity revealed restricted movements of
the fingers, web creep between two fingers on the
ulnar side with fixed flexion deformity of the ulnar
2 fingers. Thenar muscles could not be made out
and hypothenar muscles were well developed.
Ulnar pulse was palpable and radial pulse was not
palpable. X-ray of the right upper extremity
demonstrated proximal radioulnar synostosis,
carpal coalition, metacarpal synostosis, and
absence of 2 fingers and radial side finger having
only two phalanges. CT angiogram of left upper
limb was taken which showed absence of radial
artery and presence of persistent median artery
which ended before the wrist. Patient was
evaluated for syndromic association and
syndromes ruled out. Patient was diagnosed as
type 0 radial longitudinal deficiency with type 6
thumb hypoplasia and type B brachydactyly of
middle finger and clinodactyly of ring and little
fingers.
Conclusion References
Congenital longitudinal deficiencies (both radial and ulnar), its 1) Bayne LG, Klug MS. Long-term review of the surgical treatment of radial deficiencies. J Hand Surg Am. 1987;12(2):169–179.
2) James MA, McCarroll Jr HR, Manske PR. The spectrum of radial longitudinal deficiency: a modified classification. J Hand Surg Am.
multiple types and treatment remains a challenging topic of 1999;24(6):1145–1155.
discussion among plastic and hand surgeons. An uncorrected 3)Chang PS, Goldfarb CA, Bae DS, et al. Defining features of hand anomalies in severe thumb hypoplasia: a classification modification. J
longitudinal deficiency leads to the loss of hand function. Hand Surg Am. 46(5):422. 2021;e1–422:e5.
4)Bell J. The Treasury of Human Inheritance: On Hereditary Digital Anomalies. London: Cambridge University Press; 1951
Therefore, thorough examination and evaluation is needed for
5) Bayne LG. Ulnar club hand (ulnar deficiencies). In: Green DP, ed. Operative Hand Surgery. Vol 1. New York, NY: Churchill Livingstone;
early diagnosis and prompt treatment which results in better 1993:251–548.
outcome. 6) Cole RJ, Manske PR. Classification of ulnar deficiency according to the thumb and first web. J Hand Surg Am. 1997;22(3):479–488

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AN UNUSUAL PRESENTATION OF A CONGENITAL LONGITUDINAL DEFICIENCY OF UPPER LIMB

Dr Lakshmi Narayanan P - Mch Plastic Surgery Postgraduate

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