it discusses the ulnar deficiency and its Prosthosis management

1. Congenital longitudinal Ulnar
Deficiency and its Management
Abhishek Tripathi, Lecturer, Prosthetics, NILD, KOLKATA
Ref:
1. AAOS, Atlas of Amputations and Limb Deficiencies, 3rd edition
2. https://musculoskeletalkey.com/ulnar-deficiency-ulnar-clubhand/

2. Objective
1. What is Ulnar deficiency & its clinical
presentation/ morphology ?
2. What are its probable causes/etiology?
3. What are the different classification system for
Ulnar Deficiency?
4. What problem is faced (deficits) by individual
due to Ulnar deficiency?
5. What are the conservative and surgical methods
of management in Congenital Ulnar Deficiency?

3. Congenital Ulnar deficiency
• Partial or total absence of the
Ulna: This deficiency mainly
affects the Ulnar border the
upper limb. But upper arm and
radial side of the hand may also
be affected.
• Also known as
– Longitudinal deficiency of ulnar
partial or total
– Ulnar hemimelia
– Ulnar dysplasia
– Ulnar aplasia/ hypoplasia
– Post-axial deficiency
– Ulnar clubhand
• May be unilateral or bilateral and
often asymmetric.

4. Morphology
• Characteristic anomalies in congenital
Ulnar deficiency:
– Shortening of forearm
– Ulnar bowing of Radius
– Shoulder may be externally rotated
– Elbow may be in flexion or extension
contracture with synostosis
– Radius head may dislocated
– Elbow complex may be unstable
– Hand may have web-space contracture
with hypoplastic, absent or mal-rotated
thumb or digits with ectro or
syndactyly.
– Unlike Radial deficiency, the hand is
usually reasonably aligned with the
wrist level.
• With associated common
abnormalities of shoulder, elbow,
wrist and hand
Radio-humeral synostosis

5. Morphology
• Plain X-ray including shoulder,
elbow, wrist and hand may be
necessary for assessing
condition.
• An AP Xray will help in clear
Ulna view for better
classification.
• AP and Lateral radiograph of
elbow helps to determine the
radial dislocation and elbow
instability
• AP and Lateral radiograph of the
hand determines the thumb
deficiency and Syndactyly
components.
• However these finding may not be quite visible in
case of child.

6. Etiology
• The etiology of forearm or hand deficiency
remain unknown most often.
• Also ratio of radial to ulnar longitudinal deficient
was found to be 1:4.5 .
• As per study, incident rate approx. 1 in 100,000
live birth of the children
• Genetic defect is also found to be one the
reason.
• Apart from these, approx. 11 different sysdrome
were found to be cause of the Ulnar deficiency

7. Classification System for longitudinal
Ulnar deficiency
• There are various types of classification exist, due to
involvement of shoulder, elbow, forearm, wrist and
hand.
• These classification are based on
– Involved forearm and elbow abnormalities
– Involved hand abnormalities
• These classification reveals the possibility of
asymmetrical presentation of the different conditions.
• If all the system are kept and studied together, they
provide detailed information, as shown in next slide.

8. Classification System for longitudinal Ulnar deficiency
classes Kummel Ogden Bayne Cole and Manske
I Hypoplasia of otherwise normal Ulna
with distal epiphysis
Hypoplasia; distal epiphysis
present
II Partial aplasia (absence of distal part of
Ulna, including the distal epiphysis
Partial aplasia; distal
epiphysis absent
III Total aplasia of ulna Total aplasia
IV Radiohumeral Synostosis
with total aplasia of Ulna
A Normal radio-humer
joint
Normal thumb and first web
space
B Radio-humeral
Synostosis
Mild first web and thumb
deficiency
C Dislocation of radio-
humeral joint
Moderate to severe first
web and thumb deficiency
(loss of opposition, mal-
rotation, thumb index
synductyly, absent extrinsic
tendon
D Thumb absent

9. Classification System for longitudinal Ulnar deficiency

10. Recent changes in longitudinal Ulnar
deficiency classification
• Recently, the forearm and elbow classification
of the longitudinal deficiency of the Ulna
included Bayne type 0, which meant normal
length of the Ulna but this Ulnar side hand
deficiency.
• Further included Type V, which meant
proximal Ulnar Longitudinal deficiency where
distal forearm bone remain bifurcated.

11. Typical Deficits/problems due to Ulnar
deficiency
• In case of unilateral involvement, minimum functional
deficiency occurs
• Patient with bilateral involvement specially with elbow
flexion contracture will have difficulty in most of ADL
like perineal hygiene, toileting, dressing.
• Patient with absent thumb will have difficulty in pinch
and grasp of large/spherical object
• With internal rotation of shoulder and forearm
pronation, hand to mouth and hand to head motion,
perineal care and bi-manual activity get affected.
• Most of the difficulty occurs in case of Radio-humeral
Synostosis and those with absent or deformed digits.

12. Treatment and functional
consideration
• Functional improvement is the main goal for any surgical
intervention
• External rotation Corrective osteotomy of humerus is performed for
correcting complex deformity, where shoulder is in internal
rotation, forearm in pronation
• In bilateral rotational differences of forearm, a rotational osteotomy
of forearm is performed.
• One-bone forearm is performed in case of elbow instability with
poor forearm rotation.
• Patient with radio-humeral synostosis with Pterygium Cubitale
Syndrome is rarely operated with osteotomy to re-align the elbow
for functional improvement, as there is no increase in Range of
Motion due to the procedure.
• Elbow disarticulation procedure is also one of the rarely used
procedure in such condition.

13. Treatment and functional
consideration
• Hand surgery are more frequent then other surgical
procedure in Ulnar deficiency.
• Syndactyly release, rotational osteotomies,
pollicization, opponensplasty, contracture release and
web-space deepening are the procedure for improving
hand functions such as grasp, pinch or
accommodation.
• Distraction lengthening for forearm and humerus are
also done by Illizarov’s technique, but are more
complex.
• Surgical removal of the dislocated radial head or Ulnar
anlage is not recommended.

14. Conservative management
• Splinting is used initially for
stretching wrist but there
are clinical evidence
suggesting lack of deformity
prevention in the splint.
• Static Splint can control
alignment of the joint it
covers and should be used
at night time only.
• Post elbow disarticulation,
through elbow or above
elbow style of prosthesis
may also be used for
functional improvement.

15. Summary
• Ulnar club-hand/Ulnar deficiency is relatively less
frequent than radial club hand
• It is associated with other Musculo-skeletal
abnormalities but not with systemic abnormalities.
• Functional improvement is achieved mainly by
rotational osteotomies or rare cases elbow
disarticulation is also performed.
• Hand reconstruction surgery are common.
• Splinting has less role while post elbow disarticulation
Prosthetic fitment is done to improve function.