Elsevier

The Journal of Hand Surgery

Volume 49, Issue 2, February 2024, Pages 180.e1-180.e9
The Journal of Hand Surgery

Scientific Article
An Analysis of Associated Conditions and the Relationship Between the Severity of Hand Manifestations With That of the Forearm in Ulnar Longitudinal Deficiency

https://doi.org/10.1016/j.jhsa.2022.05.021Get rights and content

Purpose

A deeper investigation of medical and musculoskeletal conditions in patients with ulnar longitudinal deficiency (ULD) is needed. The association between the severity of the manifestations of ULD in the hands and forearms has not been firmly established. The purpose of this study was to describe the medical and musculoskeletal conditions associated with ULD and examine the relationship between hand and forearm anomalies.

Methods

The Congenital Upper Limb Differences registry was queried for all patients with a diagnosis of ULD, as defined by the Oberg-Manske-Tonkin classification system, between 2014 and 2020. The patients’ demographic information, medical and musculoskeletal comorbidities, radiographs, and clinical images were reviewed. The participants were classified using the Bayne, Cole and Manske, and Ogino classification systems.

Results

Of 2,821 patients from the Congenital Upper Limb Differences registry, 75 patients (2.7%) with ULD (14 bilateral), with 89 affected extremities, were included. Hand anomalies were present in 93% of the patients. Approximately 19% of the patients had an associated medical comorbidity, and 20% of the patients had an associated musculoskeletal condition. Cardiac anomalies were present in 8.0% of the patients, and 12% of the patients had a lower extremity abnormality. Radial head dislocation was observed in 13 of 18 patients with Bayne type II or III ULD compared with 8 of 43 patients with other types of unilateral ULD. There was a significant positive association among the Bayne and Ogino, Bayne and Cole/Manske, and Ogino and Cole/Manske classification systems in patients with unilateral ULD.

Conclusions

Associated medical and musculoskeletal conditions are common in patients with ULD, of which cardiac and lower extremity abnormalities are most frequently observed. There is a significant positive association between the severity of forearm anomalies and that of hand anomalies in patients with unilateral ULD. All patients with ULD should undergo a thorough cardiac evaluation by their pediatrician or a pediatric cardiologist.

Type of study/level of evidence

Symptom prevalence study III.

Section snippets

Materials and Methods

Before study initiation, institutional review board approval was obtained. The Congenital Upper Limb Differences (CoULD) registry, a multicenter database of patients with congenital upper limb anomalies, was used.29,30 Patients aged less than 18 years who are receiving care for a congenital upper limb difference from a participating institution and have undergone no previous surgical intervention for their congenital upper limb difference are eligible for inclusion in the registry. At the time

Results

A total of 89 extremities were identified in 75 patients (61 unilateral and 14 bilateral). The mean age at enrollment was 3.4 years (median, 1.9 years; range, 0–15.5 years). In this study, 70 of the 75 patients (93%) with ULD had an anomaly of the hand. A total of 14 of the 75 patients (19%) had an associated medical condition (Table 2), and 15 of the 75 patients (20%) had an associated musculoskeletal abnormality (Table 3).

Discussion

Ulnar longitudinal deficiency is an uncommon congenital malformation of the upper extremities, with a highly variable clinical presentation, that potentially affects the entire length of the upper extremities, from the shoulder girdle to the hand.14,15,24,27,28 Unlike RLD, ULD is believed to occur sporadically and has less commonly been associated with concomitant medical conditions. Goldfarb et al17 examined the medical and musculoskeletal conditions in 165 patients with RLD and found that

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  • No benefits in any form have been received or will be received related directly or indirectly to the subject of this article.

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