[Strategies in Treatment of Congenital Hand Malformation]. / Strategien in der Behandlung von angeborenen Handfehlbildungen.

The aim of the treatment of congenital hand anomalies is to achieve a functional improvement in order to enable patients to lead an independent life. As parents have to decide for their children early in life, they have to be convinced of the treatment proposal. One and the same disease may present with various individual pathologies, which may require intraoperative changes of the previously planned procedure. Because of possible changes during growth, doctors have to keep in touch with the patients up to the end of adolescence. This article presents our algorithm and surgical techniques for dealing with children with hand malformations.

Clinical Presentation, Surgical Treatment, and Outcome in Radial Polydactyly.

Radial polydactyly or 'thumb duplication' is the most common congenital upper limb anomaly ('CULA') affecting the thumb. The clinical presentation is highly diverse, ranging from an extra thumb floating on a skin bridge to complicated thumb triplications with triphalangeal, deviating, and hypoplastic components. Radial polydactyly can be classified into one of 7 osseous presentations using the Wassel classification, with type IV (45%), type II (20%), and type VII (15%) occurring most frequently. When faced with a radial polydactyly case, hand surgeons specialised in congenital anomalies must weigh the preoperative functional potential and degree of hypoplasia of both thumbs in order to decide whether to resect one thumb and reconstruct the other ('resection and reconstruction'), excise a central part of both thumbs and unite the lateral tissues into one thumb ('the Bilhaut procedure'), transfer the better-developed distal tissues of one thumb onto the better-developed proximal tissues of the other ('on-top plasty'), or discard both severely hypoplastic thumbs and pollicise the index finger. Mere excision of the hypoplastic thumb is rarely indicated since it often requires subsequent revision surgery. Even after being treated by experienced surgeons, about 15% of patients with polydactyly will need additional procedures to correct residual and/or new problems such as deviation from the longitudinal axis and joint instability. Nevertheless, radial polydactyly patients usually achieve unimpaired everyday hand function postoperatively.

[Differential Diagnoses of Trigger Thumb]. / Differenzialdiagnosen des Pollex rigidus.

Trigger thumb is one of the most common hand pathologies in toddlers. Its differential diagnoses are thumb-in-palm deformity, hyperflexible thumb, thumb hypoplasia, and congenital stiffness of the distal interphalangeal joint of the thumb. This article describes typical clinical signs of these different diseases in order to enable surgeons to make the correct diagnosis leading to the right treatment.

[Specific Characteristics of Trigger Finger in Children]. / Besonderheiten des schnellenden Fingers im Kindesalter.

BACKGROUND: Trigger finger in children is a rare condition with relevant differences to the more frequent trigger thumb in children and the very frequent trigger finger in adults. PATIENTS AND METHODS: In a retrospective analysis of children who underwent surgery for trigger fingers in 2 specialised centres, we evaluated 42 children with a total of 63 trigger fingers. Due to persistent triggering after release of the A1 pulley based on intraoperative findings in 12 children with a mean age of 3.7 years ranging from 0.6 to 10.2 years and a total of 17 trigger fingers, additional measures after the release of the A1 pulley were required, mainly the resection of one limb of the sublimis tendon. In one of these children another simultaneously operated finger required the release of the A1 pulley alone. We performed a retrospective chart review of these patients and evaluated the results after a mean follow-up time of 4.8 years ranging from 1.2 to 11.8 years by phone call. Functional impairments, persistent pain, satisfaction and complications were monitored. RESULTS: All patients treated with A1 pulley release and resection of one limb of the sublimis tendon had excellent results. One patient had a recurrence and another patient developed a contracture of the PIP joint. In both patients, the widening of the tendon sheath had been done by A2 pulley enlargement in addition to the A1 pulley release. CONCLUSIONS: Persistent triggering of a finger following the release of the A1 pulley in a child can be successfully treated by resection of one limb of the sublimis tendon.

[Epiphyseal Bar Resection for Correction of Clinodactyly]. / Resektion der atypischen Wachstumsfuge zur Korrektur der Klinodaktylie.

BACKGROUND: Clinodactyly is a digital angulation in the radio-ulnar plane. Mostly it is seen on the little finger. The middle phalanx typically has a triangular or trapezoid shape (delta phalanx), resulting in radial deviation of the distal phalanx. Resection of the longitudinal epiphyseal bracket (physiolysis) with fat graft interposition is a possible surgical technique, which uses children's growth potential to correct the axial deviation. AIM: The purpose of our study was to review the degree of correction at least 2 years postoperatively and after an average of 5 years postoperatively, and to find out if children´s age influences the results. PATIENTS AND METHODS: 23 children (43 little fingers) underwent physiolysis and were retrospectively analyzed after a median follow-up of 5 years (2.1-7.9 years). The active range of motion of the little finger's MP, PIP and DIP joints and finger-palm-distance were measured. Lateral deviation was determined by using standardized radiographs and subsequently compared with preoperative values. Patients were divided into 2 subgroups: younger than 3 years (16 fingers, group A), older than 3 years (27 fingers, group B). The achieved correction of the lateral deviation was compared between both groups. RESULTS: All patients showed full active range of motion in all joints of treated fingers. Finger-palm-distance was 0 cm. No complications occurred. The mean preoperative deviation of all patients was 37°±11, which improved after surgery by 17°±11 (i. e. 44.0%±23.1 of initial findings). Group A demonstrated a mean preoperative lateral deviation of 40°±9, and group B a mean deviation of 36°±12. In both groups we saw a similar improvement (group A mean: 17°±10, group B mean: 17°±11). In group A there was a wider dispersion of postoperative results. In the age group 7 to 10, the results of individual cases show the large variability of the corrective potential. X-rays revealed the following incidental findings after surgery: a premature fusion of the proximal radial epiphyseal plates in 2 fingers and a sinuous-shaped proximal radial epiphyseal plate in 12 other fingers. CONCLUSION: Resection of the longitudinal epiphyseal bracket with fat graft interposition is a technically simple and effective treatment option for clinodactyly, particularly in children of 3 to 6 years of age.

[Distraction Osteogenesis is an Effective Method to Lengthen Digits in Congenital Malformations]. / Die Kallusdistraktion ist eine effektive Methode zur Fingerverlängerung bei angeborenen Fehlbildungen.

PURPOSE: The aim of this study was to investigate the feasible amount of lengthening by distraction osteogenesis in congenital hand deficiencies. PATIENTS AND METHODS: A total of 60 patients (1.6-17.8 years) underwent lengthening of 71 bones between 1994 and 2014. Bone lengthening was performed on 46 metacarpals and 25 phalanges. Mostly the first (n=30) and the fifth (n=21) rays were lengthened. Bone lengthening was performed to treat primarily symbrachydactyly (b=32) and amniotic band syndrome (n=10). To analyze the amount of lengthening preoperative radiographs and radiographs taken while removing the external fixator were compared. The charts were reviewed regarding age at surgery, duration of lengthening, duration of bony consolidation, complication, etc. RESULTS: The average of metacarpal distraction was 18.4 mm=73% lengthening with respect to the preoperative length; the average of phalange distraction was 14.0 mm=77% of the preoperative length. In both, metacarpals and phalanges, a lengthening of > 100% of the preoperative bone length was possible. In target length was reached in 89% of the procedures. The average time for consolidation was 6.1 (1-20) days/mm lengthening. The external fixator was in use on average for 140 (50-346) days. After removing of the external fixator an axial K-wire was used to stabilize the callus in 9 procedure, and an iliac bone craft plus axial K-wire in 11 procedures. The rate of complications was 30% (early consolidation, deviation, joint dislocation, pin infection, tendon dislocation). All complications could be treated without with acceptable results. CONCLUSION: Metacarpal and phalangeal distraction lengthening is an effective but demanding technique for ray reconstruction in congenital malformations of the hand. It is possible to lengthen a bone by more than 100%. Complications are common, but in most cases easy to handle.

A clinically weighted approach to outcome assessment in radial polydactyly.

UNLABELLED: Currently available outcome assessment systems for radial polydactyly are mainly based on expert opinion. The aim of this study was to develop an outcome assessment system based on clinical data. We performed linear regression analysis on data from a multicentre study of 121 patients with radial polydactyly types II, IV and VII to develop a clinically weighted outcome assessment system. Items were weighted according to their influence on overall functional and aesthetic outcome in the regression analysis. Active flexion, scar appearance and prominence at amputation site were the main items influencing overall functional and aesthetic outcome (ß = 0.393, ß = 0.326 and ß = 0.288, respectively). Palmar abduction, metacarpophalangeal joint deviation and nail appearance influenced overall functional and aesthetic outcome the least (ß = -0.002, ß = -0.104 and ß = 0.070, respectively). Our proposed assessment system for radial polydactyly reflects the way clinicians value individual aspects of outcome as determinants of overall outcome and helps guide future treatment and evaluation of outcome. LEVEL OF EVIDENCE: III.

Transverse soft tissue distraction preceding separation of complex syndactylies.

UNLABELLED: Various surgical techniques are described for separation of syndactylies leading to good results. However, the use of standard techniques is limited in complex syndactylies with extensive bony fusion and tight soft tissues. The aim of this study was to assess the outcomes of a two-stage procedure involving progressive soft tissue distraction prior to syndactyly release. Between 1996 and 2012 we treated 168 complex syndactylies with this technique. The main indications were syndactylies in Apert syndrome. The digits were distracted through an external fixator at 0.5 mm/day. Distraction of 15-25 mm was achieved. Soft tissue distraction provided additional skin, a wider nail matrix and more bone in the form of callus. Thus subsequent modelling of the fingertips was improved, especially if they were closely fused. This technique facilitates treatment of complex cases and improves aesthetic outcome. LEVEL OF EVIDENCE: Therapeutic IV.

Microduplications encompassing the Sonic hedgehog limb enhancer ZRS are associated with Haas-type polysyndactyly and Laurin-Sandrow syndrome.

Laurin-Sandrow syndrome (LSS) is a rare autosomal dominant disorder characterized by polysyndactyly of hands and/or feet, mirror image duplication of the feet, nasal defects, and loss of identity between fibula and tibia. The genetic basis of LSS is currently unknown. LSS shows phenotypic overlap with Haas-type polysyndactyly (HTS) regarding the digital phenotype. Here we report on five unrelated families with overlapping microduplications encompassing the Sonic hedgehog (SHH) limb enhancer ZPA regulatory sequence (ZRS) on chromosome 7q36. Clinically, the patients show polysyndactyly phenotypes and various types of lower limb malformations ranging from syndactyly to mirror image polydactyly with duplications of the fibulae. We show that larger duplications of the ZRS region (>80 kb) are associated with HTS, whereas smaller duplications (<80 kb) result in the LSS phenotype. On the basis of our data, the latter can be clearly distinguished from HTS by the presence of mirror image polysyndactyly of the feet with duplication of the fibula. Our results expand the clinical phenotype of the ZRS-associated syndromes and suggest that smaller duplications (<80 kb) are associated with a more severe phenotype. In addition, we show that these small microduplications within the ZRS region are the underlying genetic cause of Laurin-Sandrow syndrome.

[Return of sensitivity after digital nerve reconstruction in children: how does age affect outcome?]. / Sensibilitätsrückkehr nach kindlichen Nervenverlet-zungen der Hand - welche Rolle spielt das Patientenalter?

BACKGROUND: The regenerative capacity after nerve reconstruction in children is believed to be superior compared to that in adults. However, the available data on this topic are limited. The aim of this work is to improve the age-dependent assessment of the prognosis after surgical treatment of peripheral nerve injuries of the hand. PATIENTS AND METHODS: 44 of 147 children with complete transections of proper and common digital nerves who were treated from 2000 to 2009 and who were currently 6 years or older, could be included for follow-up (mean time, 7.5 years). In total there were 60 nerve injuries, of which 56 were directly coaptated, 4 needed grafting. Sensitivity of the fingertips was assessed using the 2-point discrimination (2PD) test and the Semmes-Weinstein monofilament test. We also recorded hypersensitivity, sensitivity to cold, and paresthesia. To account for inter-individual differences in normal 2PD, the difference of the 2PD to the uninjured contralateral side was calculated as delta-2PD. The age at the time of the injury, divided into groups of 0-5, 6-10 and 11-15 years was correlated with the clinical outcome (2PD, monofilamenttest). Taking into account the results classified by age presented by Lohmeyer et al. and Mailänder et al., we assessed the correlation between age at injury (0-85 years) and clinical outcome. RESULTS: After 52 of the 56 direct nerve coaptations (93%) normal sensitivity was found with a 2PD<6 mm, 4 times the 2PD was 6 mm. Following nerve grafting a static 2PD of 6-7 mm was measured. Disturbing paresthesia, sensitivity to cold or hypersensitivity were not reported by any patient. The 2PD of the fingers of the opposite uninjured side showed great inter-individual differences. Patient's age and 2PD significantly correlated with significantly poorer results already in the second decade of life. CONCLUSION: In relation to adults, children have an excellent prognosis after nerve reconstruction. The high inter-individual differences in regular sensitivity, depending on age, co-morbi-dities, etc., suggest putting the results of the injured and uninjured sides into relation. Estimation of the Δs2PD may solve this -problem.

Ten-year experience with one-step correction of constriction rings by complete circular resection and linear circumferential skin closure.

BACKGROUND: In amniotic band syndrome (ABS) constriction rings affecting the limbs are regularly seen at birth. Circular resection and closure with multiple Z-plasties has been described as the treatment of choice. However, direct circular closure creating linear circumferential scars might replace multiple Z- or W-plasties to reduce scars and improve aesthetic outcome. METHODS: From 2000 to 2009 we treated 160 patients with ABS. In 43 cases release of isolated constriction rings was performed by circular excision and closure. Constriction rings were completely resected and skin closure could be performed without Z-plasty in all cases. Data from follow-up of all cases were reviewed and photographs and recordings reassessed. The median age at operation was 14.3 months (range 2-32). Constriction rings were localised on the upper arm in five patients, the lower arm in five, the wrist in two and at the metacarpal level in five. At the lower extremity the thigh was affected in three and the lower leg in 23 patients. RESULTS: In the 16 cases of the first treatment period from 2000 to 2004, average follow-up was 6.5 years (range 5.1-10.3). We observed two minor complications following 43 operations (wound dehiscence and secondary healing). No formation recurrence of banding was seen. Aesthetic outcome was overall good. CONCLUSIONS: We found excellent aesthetical and functional results following change of treatment from multiple Z-plasties to linear circumferential closure. Scars were generally less noticeable and no recurrence or scar constriction could be detected with growth. However, radical excision of all constricting tissues prior to wound closure is mandatory.

[Severe side effects after Octenisept irrigation of penetrating wounds in children]. / Schwere Nebenwirkungen nach Octenisept-Spülung von Perforationswunden im Kindesalter.

BACKGROUND: Deep penetrating wounds in children's hands are repeatedly treated in emergency wards conservatively through irrigation, antibiotic therapy and splint immobilisation. After we had seen severest phlegmonous reactions after irrigation with Octenisept followed by long troublesome histories we would like to warn against using this antiseptic agent for irrigation of wounds. We give an overview about the significance of antiseptics and the use of antibiotics in the treatment of deeper contaminated wounds. PATIENTS AND METHODS: Between 2003 and 2007, 5 children (aged 2 to 8 years) were treated for sequelae of local wound irrigation with Octenisept in perforating hand injuries. We describe the early and medium-term aspects after irrigation, the further development, therapeutic measures, long-term damages and necessary reconstructions. We present the results of bacteriological smear tests, laboratory reports and histological examinations as well as allergy tests. RESULTS: All children showed more or less identical hand appearances. Hands were swollen caused by an interstitial oedema, compartment pressures were increased and hand function was completely suspended. The oedemas persisted for weeks and were hardly controllable. Especially serious were injuries at thenar level and in the first web space. Long-term sequelae were contractures caused by fibrotic muscle changes. Neither through bacteriological nor histological analysis were hints of bacterial or viral infections found. An allergic reaction to Octenisept could be excluded in the 3 most heavily affected children by an ROAT test. CONCLUSION: To prevent damage, contaminated wounds should be operatively debrided and not be irrigated with an antiseptic liquid. Octenisept seems to have a toxic effect in non-distinguished tissue. Because of a slow resorption it remains for a long time in the tissue. For therapy we recommend fasciotomy of the mid-hand and probably finger compartments, followed by compression treatment, physiotherapy with lymphatic drainage, dynamic and static splints.

[Therapy of the peromelic form of symbrachydactyly by double second-toe transplantation]. / Die Behandlung der peromelen Form der Symbrachydaktylie durch zweifache freie Zehentransplantation.

From 1989 to 2001 eleven children with the peromelic type of symbrachydactyly underwent a staged double second toes transplantation for restoration of two finger rays. The second toe of the feet were transplanted first to the small finger position and in the second step to the thumb position. One failure occurred in the first toe transplantation and another developed a venous thrombosis with partial necrosis and eventually a useless ray. Eight children were reviewed retrospectively in a mean follow-up of 5.3 years. The ability to pinch was restored in four children. In all patients without complications the function of the hand improved and the sensitivity was good.