Ulnar Deficiency and Management.pptx

it discusses the ulnar deficiency and its Prosthosis management

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

2. Objective What is Ulnar deficiency & its clinical presentation/ morphology ? What are its probable causes/etiology? What are the different classification system for Ulnar Deficiency? What problem is faced (deficits) by individual due to Ulnar deficiency? 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.