OBJECTIVE: Classification systems and treatment for children with radial longitudinal deficiency are classically focused on the hand and wrist. However, the elbow can affect the function of these patients secondary to stiffness or instability. The objective of this study was to determine if a correlation exists between severity of radial longitudinal deficiency (RLD) and degree of proximal ulnar hypoplasia.
METHODS: A single-institution retrospective review was performed at a pediatric hospital. One hundred elbows were identified in 72 patients who met the inclusion criteria. Measurements such as the coronoid height, olecranon coronoid angle (OCA), and the anterior coverage index were obtained from lateral radiographs of children with RLD. Ten elbows had posterior subluxation of the ulnohumeral joint radiographically. Eleven patients had thrombocytopenia absent radius (TAR) syndrome.
RESULTS: There was a significant mean difference for OCA between RLD types. Type 4 RLD (mean [M] = 17.9; standard deviation [SD] = 11.3) had a significantly lower OCA than type 0 (M = 26.4; SD = 5.9) and type 1 (M = 31.0; SD = 5.0). A subanalysis of the 58 elbows with type 4 RLD demonstrated that those with TAR (n = 11) had significantly higher anterior coverage index total scores (M = 1.7; SD = 0.3) than those with type 4 without TAR (M = 1.5; SD = 0.2).
CONCLUSIONS: The degree of proximal ulnar hypoplasia is correlated with increasing severity of RLD, with significantly lower OCA and coronoid height observed in the elbow radiographs of patients with type 4 RLD compared with type 0 and type 1. Children with TAR have less proximal ulnar dysplasia and higher anterior coverage index than children with type 4 RLD without associated TAR.
METHODS: Prognostic IV.

OBJECTIVE: To determine whether preservation of blood supply to the index metacarpophalangeal joint decreases the rate of physeal arrest.
METHODS: A retrospective review of 41 pollicized digits in 35 patients with 2-year minimum radiographic follow-up was conducted at a single institution. Other complications evaluated included nonunion at the pollicized digit base and clinical instability at the new carpometacarpal joint. Findings were compared to historical controls, which were performed by our group prior to routine identification and sparing of the metacarpophalangeal joint blood supply. No other modifications to surgical technique were made between the previous and current patient cohorts.
RESULTS: Two pollicized digits in two different patients had radiographic evidence of physeal arrest, one of which was partial and the other complete, for an arrest rate of 4.9%. This was significantly less than the arrest rate in our historical cohort of 24.7% (21 of 85 patients). Five patients did not have radiographic bony union at the base of the index metacarpal, but only one patient had clinical instability at the new carpometacarpal joint.
CONCLUSIONS: Significantly fewer patients who underwent index finger pollicization with preservation of the metacarpophalangeal joint blood supply went on to develop physeal arrest when compared to patients who underwent pollicization prior to adoption of this technique. This finding suggests that sparing of the physeal blood supply is preventative against proximal phalanx physeal arrest.
METHODS: Therapeutic IV.

OBJECTIVE: To assess the effect of radial longitudinal deficiency on the function of pollicized digits as determined by the Thumb Grasp and Pinch (T-GAP) assessment.
METHODS: We retrospectively evaluated 25 hands with thumb hypoplasia that underwent index finger pollicization. Patients were followed for an average of 10.4 years. Hands were divided by severity into two groups: no or mild radial longitudinal deficiency (RLD) (Group 1 = 16) and moderate to severe RLD (Group 2 = 9). We collected demographic information and completed physical examination measures, including hand strength, elbow, wrist, and hand range of motion, the Kapandji opposition score, active grasp span, and T-GAP total score.
RESULTS: Patients with moderate to severe forms of RLD had stiffer long fingers, lower Kapandji opposition scores, and limited active and passive range of motion for elbow flexion, wrist ulnar deviation, and pollicized thumb interphalangeal flexion. They had shorter forearms, decreased active grasp span, and fewer thumb creases at the interphalangeal thumb joint. In addition, the T-GAP total score was significantly lower when comparing the two groups. Children with mild dysplasia were able to achieve 32% of age-matched normal grasp strength. Patients with more severe radial dysplasia averaged 17% less grasp strength compared with children with mild dysplasia. Patients with moderate to severe RLD also had lower T-GAP total scores and strength measurements if they had limited wrist ulnar deviation.
CONCLUSIONS: Individuals with moderate to severe RLD have unique anatomical factors that affect outcomes after pollicization. These individuals use their thumbs for fewer activities, have weaker grasp, and retain more primitive grasp patterns compared with those who have milder forms of RLD.
METHODS: Prognostic IV.

Addressing the deforming stresses arising from soft tissue and continued skeletal development is crucial to obtain long-term correction of forearm carpal alignment in Radial longitudinal deficiency. The aim of the present study was to report the medium-term follow-up results of radialization with ulnar cuff osteotomy in children. A total of 17 patients (21 involved limbs) with a mean follow-up of 66 months (range 50 to 96) were reviewed. Mean correction of the hand forearm angle at the final follow-up was 51°. Mean hand forearm position preoperatively and at the final follow-up were -1.1 cm (SD 0.9) and +1.3 cm (SD 0.8), respectively. This metaphyseal osteotomy relaxed the radial structures throughout the original phase of deformity correction. The mean ulnar growth was 62% of the contralateral side at the final follow-up. Our technique may provide a feasible solution to the correction and prevent recurrence of deformity while maintaining ulnar growth in the medium to longer term.Level of evidence: III.

BACKGROUND: Phenotypic differences and functional limitations in children with congenital radial and ulnar longitudinal deficiencies (RLD/ULD) are well understood for the forearm and hand. However, anatomical features of shoulder elements in these pathologies have only been scarcely reported. Moreover, shoulder function has not been assessed in this patient population. Therefore, we aimed to define radiologic features and shoulder function of these patients at a large tertiary referral center.
METHODS: We prospectively enrolled all patients with RLD and ULD (minimum age: 7 years) for this study. Eighteen patients (12 RLD, 6 ULD) with a mean age of 17.9 years (range, 8.5-32.5) were evaluated using clinical examination (shoulder motion and stability), patient-reported outcome measures (Visual Analog Scale, Pediatric/Adolescent Shoulder Survey, Pediatric Outcomes Data Collection Instrument), and radiologic grading of shoulder dysplasia (including length and width discrepancy of the humerus, glenoid dysplasia in the anteroposterior and axial view [Waters classification], and scapular and acromioclavicular dysplasia assessment). Descriptive statistics and Spearman correlation analyses were performed.
RESULTS: Despite five (28%) cases having anterioposterior shoulder instability and five (28%) cases with decreased motion, outcome scores indicated an overall excellent function of the shoulder girdle, with mean Visual Analog Scale of 0.3 (range, 0-5), mean Pediatric/Adolescent Shoulder Survey of 97 (range, 75-100), and mean Pediatric Outcomes Data Collection Instrument Global Functioning Scale of 93 (range, 76-100). The humerus was, on average, 15 mm shorter (range, 0-75), and metaphyseal and diaphyseal diameters both reached 94% of the contralateral side. Glenoid dysplasia was detected in nine (50%) cases, with increased retroversion evident in 10 (56%) cases. However, scapular (n = 2) and acromioclavicular (n = 1) dysplasia were rare. Based on radiographic findings, a radiologic classification system for dysplasia types IA, IB, and II was developed.
CONCLUSIONS: Adolescent and adult patients with longitudinal deficiencies exhibit various mild-to-severe radiologic abnormalities around the shoulder girdle. Nevertheless, these findings did not seem to negatively affect shoulder function as the overall outcome scores were excellent.

Ulnar longitudinal deficiency (ULD) is a rare skeletal condition marked by the partial or complete failure of the formation of the ulna. This rare condition is often associated with fixed flexion deformity, radial head subluxation, complex carpal, metacarpal, and digital abnormalities. Most presentations are male-preponderant and right-sided. Different classifications have described ULD. Usually, the condition is not associated with systemic findings; however, detailed physical examination and radiologic evaluations are crucial for assessing and managing affected patients. We report a rare case of ULD in an 11-month-old female infant with congenital absence of the left ulna, four digits, and a postaxial hypoplastic finger.

Congenital radial longitudinal dysplasia remains an \'unsolved problem\' in hand surgery. The challenges presented by the skeletal deficiency of the distal radius and soft tissue dysplasia of the severe radial longitudinal deficiency have been addressed by a number of techniques that aim to stabilize the position of the hand relative to the forearm and optimize forearm growth and hand function. Analysis of hand function and position in these children is difficult because of the abnormal \'wrist\' mechanics, and the published results of the techniques used to date often lack a standardized approach and importantly the perception of function from the patient\'s perspective. The existing data is reviewed and compared with the results of cohorts from two major congenital upper limb centres. Soft tissue distraction prior to radialization or centralization may offer benefit in ulnar growth and forearm length but there is a need for further research into the long-term functional outcomes of the various techniques available to determine the optimal choice for these children.Level of evidence: V.

One concern in the surgical treatment of radial longitudinal deficiency (RLD) is certainly the skin incision. Over the years many different types have been proposed and used. We propose a new skin incision technique: a double Y sliding flap with the main body along the dorsal wrist crease, followed by raising a proximal and a distal flap providing wide access to the dorsal surface of the wrist joint. After correction of the wrist deformity, skin triangles are resected on the radial and ulnar sides of the incision. Then the skin of the distal flap is slid radially and proximally, filling the defect left by the resected radial triangle, while the proximal flap is slid in the opposite direction, enabling transverse closure along the ulnar side of the incision. The final scar comprises a central body along the dorsal wrist crease, and a radial branch. The aim of this study was to analyze the clinical results of this new double Y sliding flap approach for the surgical treatment of type III and IV RLD. We retrospectively reviewed medical records of surgical correction of RLD using our new incision, between January 2016 and December 2018 in our department of hand surgery. Endpoints comprised correction of redundant skin, scar appearance, and complications. Twelve limbs in 9 patients treated with this double Y sliding flap approach were reviewed: correction of redundant skin was systematic, only 2 limbs showed postoperative complications (1 case of notable edema and 1 of delayed wound healing), and scar aspect was graded good in 11 of the 12 cases. The double Y sliding flap was safe, with minimal complications, adequate skin restoration, wide exposure of the wrist, and esthetically good scar. LEVEL OF EVIDENCE: IV.

Background: In resource challenged nations, the true magnitude of the congenital hand differences (CHD) remain unknown due to a lack of nation-wide surveillance system. We created a hospital-based registry system with the aim to determine the prevalence and distribution of CHD and compare the Swanson/IFSSH (SI) and Oberg, Manske and Tonkin (OMT) classifications. Methods: Data of children aged 0 to 18 years with CHD was entered into the online registry and classified based on the SI and OMT classifications from January 2018 to December 2021. The prevalence and distribution of CHD and risk factors like family history were summarised using descriptive analysis. Syndromic and heredity associations were further analysed using Fischer exact test and odds ratio. Clustered columns were used to compare the two classification systems. Results: A total of 307 patients with CHD presented during the study with a prevalence of 2.4/1,000 patients. Among them, 164 were unilateral, 21 were born of consanguineous marriages (7.6%) and 10 (3.6%) had a family history of CHD. Although not statistically significant, bilateral involvement was twice as likely to be hereditary compared to unilateral involvement. A total of 10 different syndromes were identified among 27 patients of which Holt-Oram syndrome was most common. The most common CHD was Radial longitudinal defificiency (111). A total of 82 (27%) children had other associated anomalies that could not be grouped as a syndrome. Two patients could not be classified using either SI or OMT classifications. Conclusions: The online registry established an efficient way to store and analyse data related to CHD. It provides new information on its prevalence in South India, which is similar to the existing literature. Most of the CHD can be grouped in both the SI and OMT classifications. However, there still remains some conditions that are unclassifiable. There is a need for a national registry of CDH for effective management, funding and research. Level of Evidence: Level IV (Epidemiological).

Pollicization has been a very successful procedure in the treatment of specific types of hypoplastic thumb. Although much has remained the same since early descriptions of the procedure in the 1800s and 1900s, refinements over the years have made it safer and more predictable. Over the years at our institution we have studied, modified and refined our incisions to produce a new thumb that is aesthetically pleasing with excellent function. We present our technique for pollicization along with pearls and pitfalls we have discovered.