Developing a Parental-Friendly Scoring System for Children with Congenital Hand Differences.

Background: Congenital hand differences (CHD) exhibit enormous diversity and heterogeneity. Surgeons and parents often have different concepts of severity, making things difficult during parental consultation. This study aims to align surgeon/parental views on the severity of the child's CHD using a novel severity classification. Methods: Parents of affected children were asked to score the severity of their child's abnormality pre- and post-consultation using a subjective scale (1-4) without any explanation. Furthermore, parents were asked to rate their concerns about the future function and appearance of their child's hand condition using a similar scale of 1-4. They were then asked to rate the severity of the CHD post-consultation and three months post-operatively following explanation of the 4-point scale, as follows: 1 = treatment possible to normal; 2 = treatment possible to near normal; 3 = treatment possible but always some hand differences; 4 = treatment not possible. The surgeon also independently scored all children using his perception of the scale. Results: Forty-three children with a range of CHD were recruited into the sample. Linear weighted kappa analyses comparing inter-rater agreement showed no agreement between surgeon and parents during the initial scoring without any explanations. However, with explanations added, agreement rose significantly (kappa = 0.437 post-consultation and kappa = 0.706 three months post-op). No correlation was found between severity with both appearance and function (r = 0.277 and r = -0.184, respectively). Conclusions: This study demonstrated that the use of a simple scoring system was able to improve parental understanding of the severity and prognosis of CHD. The system demonstrated a good correlation between surgeon and parents. Such a scoring system can be easily utilised in the outpatient department to manage expectations and reduce anxiety.

What's New in Congenital Hand Surgery.

BACKGROUND: Congenital conditions of the hand and upper extremity are a frequent source of consultation among pediatric orthopaedists and hand surgeons. Advances in the fields of molecular biology and genetics have helped to better understand some of these conditions and redefine previous classification systems. New outcome measurement tools have been used to assess surgical results and have brought into focus a different aspect of the patients' experience. METHODS: We searched PubMed database for papers related to the treatment of congenital hand anomalies published from January 1, 2015 to October 31, 2018. The search was limited to English articles yielding 207 papers. Three pediatric hand surgeons selected the articles based upon the criteria that the topic was germane, the article fell under the subheadings within the manuscript, and the conclusions were meaningful. RESULTS: A total of 40 papers were selected for review, based upon their quality and new findings. Research articles with significant findings were included for syndactyly, symbrachydactyly, cleft hand, polydactyly, radial longitudinal deficiency, congenital radio-ulnar synostosis, and macrodactyly. CONCLUSIONS: Our knowledge of the embryology and pathophysiology of congenital upper extremity conditions continues to evolve. Functional assessments combined with patient and parent-reported outcomes have our understanding of the results following surgical procedures. Further research and standardization of our scientific data will provide better answers and higher quality of evidence. LEVEL OF EVIDENCE: Level V-literature review and expert opinion.

Polydactyly a review and update of a common congenital hand difference.

PURPOSE OF REVIEW: The purpose of this review is to describe various forms of hand polydactyly and their different treatment approaches. Hand polydactyly is commonly classified as ulnar (small finger) or radial (thumb). Polydactyly can be sporadic, genetic, and/or associated with syndromic conditions. RECENT FINDINGS: Both ulnar and radial polydactyly can be surgically treated to optimize hand aesthetics and function. Timing of surgery is based on multiple factors, most notably including safety of anesthesia and socialization of the affected child. The pediatrician should be aware of potential associated conditions, such as chondroectodermal dysplasia or Ellis-van Creveld syndrome for ulnar polydactyly. SUMMARY: Polydactyly is a common congenital hand difference and can be broadly be classified by radial or ulnar involvement. Polydactyly warrants hand surgical referral, as surgical treatment is often indicated. Pediatricians should be aware of treatment options, as well as of commonly associated anomalies and syndromes.

The Second Toe-to-Hand Transfer for Full-length Thumb Reconstruction in Congenital Thumb's Grade IIIb to V Hypoplasia: MTPJ Arthrodesis Instead of Tendon Rebalansing.

Congenital thumb hypoplasia is a rare deformity of the upper extremity. The classification of thumb hypoplasia was created by Blauth in 1967 (types I to V). The base of the first metacarpal bone is absent for hypoplasia types IIIb to V, therefore, toe-to-hand transplantation is not recommended. A stable first carpometacarpal joint has been considered a mandatory factor for a successful toe-to-hand transplantation. The aim of this study is to describe a new technique for thumb reconstruction with the second toe transfer and metatarsophalangeal joint (MTPJ) arthrodesis, which can provide a five-digit hand and restore thumb functionality for thumb hypoplasia IIIb to V. We performed second toe-to-hand transplantation with MTPJ arthrodesis for 3 pediatric patients (mean age, 69 mo) with congenital thumb hypoplasia IIIb (n=2), grade V (n=1). Long-term follow-up evaluated the functions and esthetics of the hands for grade IIIb patients (n=2). We believe the second toe transfer with MTPJ arthrodesis transplantation is a promising method for reconstructing a full-length congenital or traumatic thumb absence for the pediatric population.

Characterization of Hand Anomalies Associated With Möbius Syndrome.

PURPOSE: To investigate the distinguishing morphological characteristics of the upper extremities in children with Möbius syndrome. METHODS: Twenty-seven involved extremities in 14 patients with a diagnosis of Möbius syndrome were identified at 2 institutions. Medical records, radiographs, and clinical photographs were evaluated. Congenital hand differences were classified according to the Oberg, Manske, and Tonkin classification, and hands with symbrachydactyly were classified by the Blauth and Gekeler classification. The presence of other congenital anomalies was catalogued. RESULTS: There was bilateral involvement in 93% of patients with congenital hand anomalies. Twelve patients demonstrated congenital hand anomalies and 2 patients had been diagnosed with arthrogryposis. Among the 12 patients with congenital hand anomalies, 21 hands were classifiable as symbrachydactyly by the Oberg, Manske, and Tonkin classification and could be categorized by the Blauth and Gekeler classification. Short finger type was the most common subtype of symbrachydactyly, present in 13 hands. Eleven of these 13 patients (85%) were primarily affected on the radial side of the hand. Proximal arm involvement was identified in 2 patients with symbrachydactyly, both of whom had Poland syndrome and an absent pectoralis major. CONCLUSIONS: Symbrachydactyly in Möbius syndrome differs from the typical presentation of symbrachydactyly. Characteristically, there is a bilateral presentation with a strong predilection for radially based brachydactyly. These described characteristics may help the hand surgeon appropriately assess patients, especially those with radial-sided symbrachydactyly. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic III.

Symbrachydactyly: Finger nubbins are not always amniotic band disruption sequence.

Although congenital hand anomalies associated with finger nubbins may be produced by amniotic band disruption sequence (ABDS), symbrachydactyly should be considered in the differential diagnosis. ABDS usually affects more than one limb but symbrachydactyly largely is limited to one upper extremity, and has five distinct clinical presentations: short-fingered, atypical cleft, monodactylous, peromelic, and a forearm proximal transverse deficiency. This article discusses the diagnosis of symbrachydactyly compared with ABDS and outlines plans for managing patients with symbrachydactyly.

Treating Congenital Proximal Interphalangeal Joint Contracture.

The management of congenital proximal interphalangeal joint deformity, also known as camptodactyly, is challenging. There are numerous theories on the cause of this abnormal finger posture, leading to variations in classification, definition, and treatment approaches. This article assesses the previous literature and provides clarity and guidance for the practical treatment of camptodactyly.

Polydactyly of the Hand.

Polydactyly is one of the most common congenital hand deformities managed by orthopaedic surgeons. It is most often found in isolation; however, rarely, it may be associated with genetic syndromes. Polydactyly is classified as postaxial, preaxial, or central depending on the radioulnar location of the duplicated digits. Postaxial polydactyly, which affects the ulnar side of the hand, is most common and is typically managed with excision or suture ligation of the supernumerary digit. Preaxial polydactyly, which affects the thumb or radial side of the hand, often requires reconstructive techniques to ensure a functional, stable thumb. Central polydactyly is much less common, and reconstruction can be challenging.

Intraobserver and Interobserver Reliability of the Oberg-Manske-Tonkin (OMT) Classification: Establishing a Registry on Congenital Upper Limb Differences.

BACKGROUND: The purpose of this investigation was to determine the reliability of the Oberg-Manske-Tonkin (OMT) classification system applied to patients enrolled in a prospective, multicenter cohort study of congenital upper limb differences. Our hypothesis was that the OMT classification would exhibit high intraobserver and interobserver reliability and thus would be an appropriate tool for the new Congenital Upper Limb Differences registry. METHODS: Four pediatric hand surgeons independently reviewed the medical records, clinical photographs, and radiographs of 60 randomly selected patients previously enrolled in the Congenital Upper Limb Differences registry. Patient's information was summarized and shared electronically (PowerPoint). Each rater classified each congenital anomaly according to the OMT classification system. Responses were recorded using a web-based data capture tool (REDCap). Three weeks later, the surgeons repeated the process. Intraobserver reliability for each rater was assessed using Fleiss' κ. Interobserver reliability was assessed using percent exact agreement (when all 4 raters were in agreement) as well as Fleiss' κ. RESULTS: Using the OMT classification, intraobserver reliability for the 4 pediatric hand surgeons showed almost perfect agreement, with κ values in the range of 0.89 to 0.93. Interobserver reliability demonstrated substantial agreement, with κ value of 0.79 (95% confidence interval, 0.77-0.82) in the first reading and 0.80 (95% confidence interval, 0.77-0.83) in the second reading. The highest possible agreement (κ=1) was seen for the following diagnoses: congenital dislocation of radial head (OMT I.A.2.v), Madelung deformity (I.A.2.vii), radial polydactyly (I.B.2.iii), triphalangeal thumb (I.B.2.iv), Kirner deformity (I.B.4.vi), and osteochondromatosis (3.B.4.i). CONCLUSIONS: The OMT classification of congenital upper limb differences exhibits substantial to almost perfect intraobserver and interobserver reliability among pediatric hand surgeons at different institutions. The OMT is therefore well-suited for classification of congenital upper limb anomalies in patients enrolled in a multicenter registry. LEVEL OF EVIDENCE: Level II-diagnostic.

Classification of congenital anomalies of the hand and upper limb.

The Oberg, Manske, Tonkin Classification of congenital anomalies of the hand and upper limb utilizes dysmorphological concepts to distinguish Malformations from Deformations and Dysplasias. Malformations are abnormalities of Formation and/or Differentiation of tissues. Deformations are abnormalities which occur after tissue is formed. Dysplasias are abnormalities which result from a lack of normal organization of cells into tissue. Malformations are sub-grouped according to whether the abnormality affects the hand alone or the whole of the upper limb; and according to which, if any, of the three main axes of development are primarily involved. It is not possible to satisfy all criteria which would define an ideal classification. However, this system is practical in that it retains the surgical diagnoses and sub-classifications familiar to surgeons and is easily utilized; it is expandable and may be modified without destroying its structure; it may be used to accumulate prevalence data; and, in the future, gene and chromosomal defects which are identified for specific diagnoses may be incorporated into the system.

Early Results of Surgical Management of Camptodactyly.

BACKGROUND: We hypothesize that surgery for moderate-to-severe camptodactyly (>50 degrees) results in modest gains in range of motion and improved digital position. METHODS: A retrospective analysis of patients undergoing surgery for camptodactyly at a tertiary children's hospital between 2000 and 2014 was performed. Surgery was indicated for patients with persistent, functionally limiting flexion contractures despite observation, therapy, and splinting. Data were collected on demographics, clinical history and presentation, nonoperative management, surgery, and clinical follow-up, focusing on range of motion at the involved joint. Total passive motion (TPM) and total active motion (TAM) at the proximal interphalangeal (PIP) joint was calculated at presentation, preoperative visit, first postoperative visit out of the cast, and last follow-up visit. Average postoperative follow-up was 1.4 years. RESULTS: In total, 31 digits in 22 patients were reviewed. There were 13 males; average age at surgery was 9.6 years. There were 7 type I (infantile), 8 type II (adolescent), and 7 type III (syndromic) patients. All cases involved the PIP joint; 55% involved the small finger. All patients underwent sequential release of contracted structures until maximal extension without compromising vascularity or joint stability was obtained. Z-plasty of the volar skin was performed in 68% of digits, flexor digitorum superficialis tenotomy in 77%, volar plate release in 58%, and collateral ligament release in 48%. All patients were casted postoperatively for an average of 31 days, and 71% of digits had temporary transarticular pin fixation. At initial presentation, mean TPM and TAM were 34 and 24 degrees, respectively. TPM and TAM were 32 and 19 degrees immediately preoperatively, 30 and 13 at the first postoperative visit, and 35 and 25 at the final follow-up. Furthermore, the position of PIP arc of motion was in a more extended position postoperatively. Average TPM arc of motion was from 50 to 82 degrees preoperatively and 28 to 63 degrees at final follow-up; average TAM arc of motion was 62 to 81 degrees preoperatively and 30 to 55 degrees at final follow-up. There were no clinically meaningful differences in results based on camptodactyly type, preoperative motion, or age at surgery. There were no cases of wound infection or dehiscence. Two patients with recurrent contractures opted for subsequent PIP arthrodesis. CONCLUSIONS: Total motion of the PIP joint was similar both preoperatively and postoperatively following surgical release of camptodactyly. However postoperatively, the digit was in a more extended position over this arc of motion. For patients with functionally limiting flexion contractures, surgical release may be beneficial by providing a more extended position, for improved digital release, hygiene, and esthetics. LEVEL OF EVIDENCE: Level IV.

Oligodactyly with Thumb.

BACKGROUND: Oligodactyly of the hand is one of the rarest congenital anomalies of upper extremities and defined as the presence of fewer than five fingers on a hand. Although it usually occurs in association with hypoplasia or absence of ulna, it can occur without abnormality of the forearm bones. The purpose of this study is to present clinical features and radiographic characteristics of hand oligodactyly with thumb. METHODS: Five patients of oligodactyly with thumb who showed normal forearm bones, were evaluated. Two patients had threefingered hand with thumb, and three had two-fingered hand with thumb. We analyzed associated abnormalities of carpal and metacarpal bones and measured the lengths of radius and ulna, and width of the wrist on the simple radiographs. We also devised new classification system of oligodactyly based on the thumb deformities and locations of missing digits. RESULTS: Syndactyly among fingers was associated in four patients, clinodactyly caused by delta bone in one, hypoplasia of the thumb in one, camptodactylies in one, symphalangism in one, and radial head dislocation in one. Considering the abnormalities of the carpal bones, the missing digits were presumed to be ulnar-sided digits in two patients, central digits in one patient and both ulnar-sided and central digits in two patients. In patients with missing of central digits, an adjacent metacarpal was hypertrophied. Although the ulnar variances were within normal range, the average lengths of radius and ulna were 6% and 5% shorter than those of contralateral normal side. The average width of the wrist was 9% narrower than that of contralateral normal side. CONCLUSIONS: Syndactyly and hypertrophied metacarpal were most commonly observed findings in the oligodactyly with thumb. Although oligodactyly with thumb may be a type of ulnar longitudinal deficiency, however, it can also be a type of central deficiency or combined type of ulnar longitudinal deficiency with central deficiency or radial longitudinal deficiency. We suggest a classification system of oligodactyly with thumb based on locations of missing digits and associated thumb deformities.

[Congenital deformities of the hand and upper limb]. / Les malformations congénitales de la main et du membre supérieur.

Congenital deformities of the hand and upper limb include a significant number of clinical situations. Their expression is, as in all congenital diseases, variable. Therefore, we can almost consider that each clinical situation is a bit unique. The difficulty, as any congenital disease, is the fact that the clinical cases are extremely diverse and difficult to classify. So what to do and surgical strategies are often matter of "School". This is even more true that the evaluation of results is very difficult due to low series (poor statistical value) and functional assessment to be partly due to the growth of the child. It is impossible in a few pages to describe all malformations of the hand and upper limb, summarize the indications and evaluate the results. Also, this chapter is not exhaustive and we will focus primarily on the most frequent pathologies.

Congenital Anomalies of the Hand--Principles of Management.

Physicians who specialize in pediatric orthopedics and hand surgery frequently encounter congenital hand abnormalities, despite their relative rarity. The treating physician should be aware of the associated syndromes and malformations that may, in some cases, be fatal if not recognized and treated appropriately. Although these congenital disorders have a wide variability, their treatment principles are similar in that the physician should promote functional use and cosmesis for the hand. This article discusses syndactyly, preaxial polydactyly and post-axial polydactyly, and the hypoplastic thumb.

Symbrachydactyly - Diagnosis, Function, and Treatment.

Symbrachydactyly is a congenital hand difference that presents with diverse morphologic forms and can be confused with many other congenital hand differences. Congenital hand difference classification schemes categorize symbrachydactyly as an undergrowth or failure of axis formation. It is further categorized by the number of affected fingers, by morphologic characteristics, and by the functional status of the hand. Symbrachydactyly represents a disruption of embryonic formation and differentiation that results in a hand shorter and smaller than the contralateral with underdeveloped often short or webbed digits, digital nubbins, or absent digits. Treatment is patient specific and should be guided to improve the function and appearance of the hand.

The OMT Classification of Congenital Anomalies of the Hand and Upper Limb.

The Oberg, Manske and Tonkin (OMT) Classification of congenital anomalies of the hand and upper limb uses dysmorphological terminology, placing conditions in one of three groups: Malformations, Deformations and Dysplasias. The main group, Malformations, is further subdivided according to whether the whole of the limb is affected or the hand plate alone, and whether the primary insult involves one of the three axes of limb development and patterning or is non-axial. The common surgical diagnoses, such as thumb duplication and thumb hypoplasia, are then placed within this framework. Recently the International Federation of Societies for Surgery of the Hand Scientific Committee for Congenital Conditions approved the OMT Classification as a timely and appropriate replacement of the previously accepted Swanson Classification. This review charts the development of and modifications to the OMT Classification and its current status.

The Classification of Swanson for Congenital Anomalies of Upper Limb Modified by the Japanese Society for Surgery of the Hand (JSSH).

The aim of this study is to introduce the classification of Swanson for congenital anomalies of upper limb modified by the Japanese Society for Surgery of the Hand (the JSSH modification) in English. The Swanson classification has been widely accepted by most hand surgeons. However, several authors have suggested that complex cases, particularly those involving the complex spectrum of cleft hand and symbrachydactyly, are difficult to classify into the classification schemes. In the JSSH modification, brachysyndactyly, so-called atypical cleft hand and transverse deficiency are included under the same concept of transverse deficiency. Cleft hand, central polydactyly, and syndactyly are included in the same category of abnormal induction of digital rays. We believe that the JSSH modification system is effective in providing hand surgeons with the clinical features and conditions for congenital anomalies.