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A strong association has been reported between keloid formation after syndactyly reconstruction and primary digital enlargement, and methotrexate (MTX) treatment has been shown to produce promising results in a few reported cases. However, detailed surgical technique for revision of keloid formation after syndactyly division has not been well described, and there is still no standard treatment protocol regarding use of MTX in such cases. In this paper, we describe the technical details of keloid excision and full thickness skin grafting followed by MTX medication for the treatment of massive keloid formation after syndactyly division. We also describe a case of unsuccessful use of MTX, and our experience of its prophylactic use for a patient with syndactyly with primary digital enlargement.

Macrodystrophia lipomatosa is a rare, non-hereditary form of congenital local gigantism characterised by enlargement and hypertrophy of all mesenchymal tissue components with a disproportionate increase in adipose tissue. This form of macrodactyly has been reported in association with other anomalies including polydactyly, brachydactyly, syndactyly, and symphalangism. We describe a previously unreported case of bilateral upper extremity macrodystrophia lipomatosa with syndactyly in a 23-month-old boy. In this report, we emphasise the importance of establishing a diagnosis with imaging and review the described surgical approaches to treating this difficult condition.

Background: Different techniques are used to release simple and complex congenital syndactyly in order to create an adequate web space, and to separate the fingers to allow independent function.

Methods: This article is a systematic review of the literature, aiming to evaluate the evidence for the different techniques and outcome measures utilised.

Results: The studies consisted mainly of retrospective, non-controlled descriptive series and a few retrospective cohort studies. The level of evidence is predominantly poor.

Conclusions: Although recommendations in favour of any particular surgical technique cannot be given based on evidence, a number of conclusions can be drawn out of the existing literature with regards to the design of the incisions for finger separation, use of pulp flaps and grafts.

Background: In a small series, it has been postulated that delayed release of complex syndactyly of the 3rd web in Apert syndrome patients causes compression on epiphyses, with early epiphyseal closure, leading to symphalangism and reduced capitate ossification. We wished to see whether this remains true in a larger series.

Methods: We reviewed radiographs of 48 patients (86 hands) with Apert syndrome seen in the department of Plastic and Reconstructive Surgery, Great Ormond Street Hospital, between the years 2001–2012. Patients underwent surgical release of syndactyly in a staged fashion with the 3rd web release left until last. We measured the size of the capitate ossification center relative to that of the hamate and determined the relative position of the middle finger metacarpal relative to the ring finger metacarpal.

Results: We found agreement with many findings, however we weren’t able to demonstrate the catch-up growth of the capitate after release of the third web. The failure of normal distal migration of the 3rd metacarpal appeared to occur until the 3rd web release is performed.

Conclusions: Consistent findings of delayed ossification of the capitate and failure of normal distal migration of the third metacarpal add support to the initial hypothesis, however, we cannot fully conclude that an earlier release of the third web is recommended, further research is still needed.

Background: Syndactyly is one of the commonly encountered congenital hand anomalies. However, there are no strict guidelines regarding the timing of surgical release. The aim of this study was to investigate the age and factors associated with syndactyly release in the United States.

Methods: A retrospective analysis of the California and Florida State Ambulatory Surgery and Services Databases for patients aged 18 years or younger who underwent syndactyly release surgery between 2005 and 2011 was performed. Demographic data that included the age at release, gender, race and primary payor (insurance) was collected. A sub-analysis was performed to compare the demographic characteristics between those patients undergoing syndactyly release before 5 years of age (‘Early Release’) and at (of after) 5 years (‘Late Release’).

Results: A total of 2,280 children (68% male, 43% Caucasian) were identified. The mean age of syndactyly release was 3.6 years, and 72.9% of patients underwent release before the age of 5 years. A significantly larger proportion of females (p = 0.002), and Hispanics and African Americans (p = 0.024), underwent late release compared to early release. Additionally, a significantly higher percentage of patients undergoing late release utilised private insurance (p = 0.005). However, the actual differences in gender, race and primary payor were small.

Conclusion: The majority of syndactyly releases were performed before school age, which is the primary goal in the management of syndactyly. While gender and racial disparities in the surgical treatment of syndactyly may exist, the differences in the present study were relatively small.

Level of Evidence: Level III (Therapeutic)

Surgical management of constriction ring syndrome (CRS) is individualised due to the heterogenic presentation of the condition. CRS includes constriction rings, acrosyndactyly, nubbins and short digits. Involvement of more than one limb is common and children often need multiple surgeries. Each limb may need staged surgeries. If the child has vascular or lymphatic compromise secondary to a constriction ring, the ring needs to be excised and released in the first few days of life. The rings are released using multiple big Z-plasties in one or two stages. Nerve palsy associated with the rings need early intervention. Tendon transfers may be advised when nerve procedures like neurolysis and nerve reconstruction fail. Acrosyndactyly can be corrected with separation of the fused fingertips. At times, the web is more distal than usual and requires web deepening using partial syndactyly separation techniques and may need full thickness skin grafting. Complex type of acrosyndactyly is difficult to treat as it needs proper planning and staged surgical correction to achieve a five-digit hand. Separation of the syndactyly with web deepening gives more functional length and independence to the digits. The short fingers, especially the thumb, need reconstruction. Non-vascularised toe phalangeal transfer or a microvascular toe transfer can reconstruct a missing digit.

Level of Evidence: Level V (Therapeutic)

The global burden of surgical conditions is becoming increasingly prevalent in the developing world. Hand surgery, in particular congenital hand surgery, presents with its own challenges, particularly the unique skill sets needed, multidisciplinary nature and heterogeneity of cases. The aim of this review article is to present our experience of practising congenital hand surgery in Cambodia, and to explore the feasibility of teaching this specialty in developing countries within a meaningful and sustainable framework. Since 2013, a group of hand surgeons and therapists have visited the Children Surgical Centre in Cambodia. A community-oriented curriculum was developed following the initial visits, with analysis of data and local surgeon’s skill sets. These were further refined using the Pareto analysis and selected competency procedures to develop entrustable professional activities (EPAs). Common paediatric hand cases were identified and taught to the local surgeons. Essential elements of this model include the need for measurable outcomes, clear curriculum goals, long-term partnerships and information technology support.

Ectodermal dysplasia-syndactyly syndrome 1 (EDSS1) is an exceedingly rare condition associated with mutations in the PVL4 gene. It is characterised by sparse, brittle hair, eyebrows and eyelashes, abnormal dentition and nails, along with bilateral cutaneous syndactyly involving the fingers and toes. We report a 2-year-old girl who presented to us with bilateral complete simple syndactyly of the third and fourth web spaces of the hands, along with bilateral syndactyly of both feet involving the second to fourth toes. Upon examination, sparse hair and eyebrows, along with abnormal dentition, were noted. Thorough clinical examination and genetic analysis were conducted on the affected child and her father, who exhibited similar clinical features. Genetic analysis revealed a homozygous nonsense mutation in the PVL4 gene in both individuals. According to the literature, EDSS1 has been reported in only 10 families worldwide, and there are no reported cases from India.

Level of Evidence: Level V (Therapeutic)

Background: Revision surgery after syndactyly separation is challenging. Web creep and scarring have a great impact on function and appearance of the hand. There is a paucity of literature on revision surgery for syndactyly. The aim of this study is to present the outcomes of revision surgery for syndactyly.

Methods: This retrospective study included patients who required revision surgery after syndactyly release for web creep, scar contracture affecting motion and/or bony deformities. Web creep was graded using the Withey classification and scars were graded as minimal, mild, moderate and severe based on the impact on range of motion (ROM). All patients underwent dorsal and volar triangular flaps for correction for web creep, multiple Z-plasty and/or proximal interphalangeal joint (PIPJ) release for correction of scar contractures and osteotomy for correction of angular/rotational bony deformities. Improvement in web creep grading, scar contracture and change in angular and/or rotational deformities were recorded.

Results: The study included 9 hands in 7 patients who required surgery for 13 web creeps (four grade 2, four grade 3 and five grade 4), 20 scar contractures (five mild, seven moderate and eight severe), 6 angular (five ≤15° and one = 40°) and 1 rotational deformity. All web creeps improved to grade 1. Out of the 20 scar contractures, 14 improved to mild and 6 to moderate. Two patients underwent corrective osteotomy, one for rotational deformity and one for a 40° angular deformity which improved to 5°.

Conclusions: Double opposing triangular flap presents a good option for revision of the web with no recurrence of web creep after a 1-year follow-up period. We suggest a grading system which assesses the effect of scarring on finger motion. Finally, avoiding straight volar suture lines decreases the incidence of recurrence.

Level of Evidence: Level IV (Therapeutic)

Background: Currently, large-sample epidemiological studies on congenital upper limb differences (CULD) in China are relatively rare. This report presents our centre’s experience on the spectrum of diseases and related factors.

Methods: Information was collected from patients with CULD who underwent surgical treatment at our centre from September 2018 to October 2023. Data collection included patient name, gender, age, clinical features and diagnosis, family history, parents’ age, parents’ medical history, pregnancy history and family income. Bivariate relationships between these variables were examined.

Results: The average age of the patients was 4 years; 1,398 patients (99%) were aged 9 months to 18 years, and 17 patients (1%) were adults. There were 848 males (60%) and 567 females (40%), with a statistically significant gender distribution (p < 0.05). The most common differences were polydactyly and syndactyly, accounting for 47% and 14%, respectively. Syndromic patients accounted for 14%, and cardiac conditions were the most prevalent non-musculoskeletal issue (55%). First-born patients accounted for 54% of patients, and the average age of parents for first-born patients was 30 ± 5 years for fathers and 29 ± 2 years for mothers. For non-first-born patients, the average age of parents was 33 ± 6 years for fathers and 32 ± 5 years for mothers, with a statistically significant difference between the two groups (p < 0.001).

Conclusions: In China, congenital limb differences are still dominated by polydactyly and syndactyly. Heart conditions occur most frequently in patients with syndromes.

Level of Evidence: Level IV (Epidemiological)