The Oberg-Manske-Tonkin (OMT) Classification of Congenital Upper Extremities: Update for 2020.

A new classification for congenital upper-extremity anomalies was first published in 2010. It has come to be known as the OMT classification highlighting the thought leaders behind it: Kerby Oberg, Paul Manske, and Michael Tonkin. Based on a dysmorphology framework, the OMT has been adopted by the International Federation of Society for Surgery of the Hand and surgeons who treat congenital upper-extremity anomalies. As predicted in the first publication, updates will be necessary based on an improved understanding of morphogenesis; the first update was in 2014 and this represents the second update to the original OMT classification. We carefully reviewed all aspects of the OMT classification, its current stratification, and updated literature on the developmental basis of limb anomalies. We also considered the clinical usefulness and challenges of the classification through discussions with stakeholders and those who care for patients with congenital upper-limb anomalies. These factors guided the current modifications of the OMT classification. In providing the updated classification, we provide the rationale for these changes. The updated OMT classification is by no means final. As our understanding of congenital anomalies progresses, we anticipate subsequent updates in the years to come.

ASSH 75 Years: An Update of Progress Over the Past 25 Years.

This article chronicles some of the major advancements made by the American Society for Surgery of the Hand over the past 25 years since the publication of William Newmeyer III's monograph, American Society for Surgery of the Hand: The First Fifty Years, in 1995. What is intangible and impossible to articulate in this article are the countless stories of relationship building, education, and research advancement that the programming and activities the American Society for Surgery of the Hand has provided.

Long-term Outcomes Following Vickers Ligament Release and Growth Modulation for the Treatment of Madelung Deformity.

BACKGROUND: Madelung deformity arises from a partial distal radial growth disturbance in combination with an abnormal hypertrophic ligament spanning the volar radius and carpus, termed, the Vickers ligament. The purpose of this study is to report long-term clinical and radiographic outcomes following Vickers ligament release and distal radial physiolysis in a population of skeletally immature patients with symptomatic Madelung deformity. METHODS: Medical records were retrospectively reviewed of patients with Madelung deformity surgically treated between 1994 and 2005. All eligible patients who underwent a Vickers ligament release and distal radial physiolysis were contacted and invited to return to the clinic for follow-up. RESULTS: Six patients (8 wrists) with Madelung deformity underwent Vickers ligament release and distal radial physiolysis. All were white females with a mean age at initial presentation of 11.4 years (10 to 12.8 y). Mean age at the time of initial surgery was 12.0 years (10.0 to 14.5 y). The median follow-up time was 10.6 years (5.8 to 21.9 y) and the average age at last follow-up was 23.1 years (17.5 to 32.2 y). Pain alone or in combination with concerns for deformity was the chief complaint in 6 of 8 of the wrists. At 1 year of clinical follow-up, 7 of 8 wrists were reported to be pain-free, and 6 of the 8 were noted to be completely pain-free at last follow-up. Motion in flexion, extension, pronation, supination, radial, or ulnar deviation was similar between the preoperative status and long-term follow-up. The average preoperative ulnar tilt was 35.1 degrees (SD: 8.5 degrees), average preoperative lunate subsidence was 1.9 degrees (SD: 1.8 degrees), and average preoperative palmar carpal displacement was 21.9 degrees (SD: 2.9 degrees). At the final follow-up, there was a large progression in lunate subsidence, but minimal change in ulnar tilt and palmar carpal displacement. At last clinical follow-up, 2 of the 6 patients had undergone a subsequent procedure including 1 radial dome osteotomy and 1 ulnar shortening osteotomy. CONCLUSION: In the skeletally immature patient population with Madelung deformity with growth potential remaining, distal radial physiolysis and Vickers ligament release is associated with relief of pain, preservation of motion, and, a reasonable rate of reoperation. TYPE OF STUDY: This was a therapeutic study. LEVEL OF EVIDENCE: Level II.

Effect of Distal Ulna Osteochondroma Excision and Distal Ulnar Tether Release on Forearm Deformity in Preadolescent Patients With Multiple Hereditary Exostosis.

BACKGROUND: Multiple hereditary exostosis is a benign condition that can lead to significant forearm deformity secondary to physeal disturbances. As the child grows, the deformity can worsen as relative shortening of the ulna causes tethering, which may lead to increased radial articular angle, carpal slippage, and radial bowing, over time this tethering can also result in radial head subluxation or frank dislocation. Worsening of forearm deformities often require corrective reconstructive osteotomies to improve anatomic alignment and function. The purpose of this study is to evaluate the effectiveness of osteochondroma excision and distal ulnar tether release on clinical function, radiographic anatomic forearm alignment, and need for future corrective osteotomies. METHODS: The authors reviewed a retrospective cohort of preadolescent patients who underwent distal ulna osteochondroma resection and ulnar tethering release (triangular fibrocartilage complex). Patients were invited back and prospectively evaluated for postoperative range of motion, pain scores, self-reported and parent-reported Disabilities of the Arm, Shoulder, and Hand (DASH) and Pediatric Outcomes Data Collection Instrument (PODCI) scores. In addition, preoperative and final postsurgical follow-up forearm x-rays were reviewed. RESULTS: A total of 6 patients and 7 forearms were included in our study with an average age of 7.9 years at time of surgery. The average final follow-up was 7.4 years. With respect to range of motion, only passive radial deviation demonstrated improvement -20 to 14 degrees (P=0.01). Although there was not statistically significant change in radial articular angle, this study did find an improvement in carpal slip 75.7% to 53.8% (P=0.03). At final follow-up DASH score was 5.71 (σ=5.35), PODCI Global Function score was 95.2 (σ=5.81), and PODCI-Happiness score 98 (σ=2.74). Visual analogue scale appearance and visual analogue scale pain assessment were 1.67 (σ=1.21) and 1.00 (σ=1.26), respectively, at final follow-up. No patient in the cohort developed a radial head dislocation. Only one of 7 forearms required a corrective osteotomy within the study's follow-up time period. CONCLUSIONS: Surgical excision of forearm osteochondromas with ulnar tether release in the preadolescent patients improves carpal slip, may help to prevent subsequent surgical reconstruction and provides satisfactory clinical results at an average 7-year follow-up. LEVEL OF EVIDENCE: Level III-therapeutic study.

Distal Dorsal Dimelia: A Disturbance of Dorsal-Ventral Digit Development.

PURPOSE: Congenital palmar nail (distal dorsal dimelia [dDD]) of the hand is a rare malformation most commonly affecting the little finger. The purpose of this report was to review the features and associations of this rare disorder and discuss the suspected underlying etiology in light of our current understanding of developmental biology. METHODS: In this retrospective cohort study from 3 practices, we describe our collective experience and review the reported literature on this disorder both as an isolated condition and in conjunction with other anomalies. RESULTS: We examined 15 fingers with dDD, 5 of which involved little fingers. We also found dDD in 6 cases with radial polydactyly (preaxial polydactyl type II [PPD2]) and in 1 case of cleft hand involving digits adjacent to the clefted web space (the index and middle fingers). Cases of little finger dDD were also associated with prominent clefting of the adjacent web space in 4 of 5 cases. All cases had stiffness of the interphalangeal joints and loss of palmar creases consistent with dorsalization of the palmar aspect of the digit. When combined with 63 fingers reported in the literature with dDD, 3 patterns were evident. The most common form occurred in little fingers (n = 50; 64%; dDDu). The next most common form was reported in association with cleft hands (n = 16; 21%; dDDc). Radial digits in association with either radial polydactyly (PPD2) or radial longitudinal deficiency were also susceptible to dDD (n = 12; 15%; dDDr). CONCLUSIONS: Congenital dDD is a disturbance of terminal dorsal-ventral digit patterning. The distribution of this condition with little fingers, clefting, and altered radial digit formation (PPD2 or radial longitudinal deficiency), as well as recent genetic and animal studies, suggests that dDD and altered dorsal-ventral patterning are linked to abnormal apical ectodermal ridge boundary formation. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic IV.

Outcomes After Primary Repair and Staged Reconstruction of Zone I and II Flexor Tendon Injuries in Children.

BACKGROUND: Delayed diagnosis of flexor tendon injury in children is common, and consequent flexor sheath scarring may necessitate a 2-stage reconstruction. Previous studies show variable outcomes after 2-stage flexor reconstruction in children, especially those below 6 years old. We evaluated functional and subjective outcomes of primary repair and staged reconstruction of zone I and II tendon injuries in children under 6 years of age. METHODS: A retrospective chart review identified 12 digits in 10 patients who had undergone surgical treatment of a zone I or II flexor tendon injury. Seven digits had a primary repair and 5 had a 2-stage reconstruction. Time delay from injury to surgery for primary repairs averaged 18 weeks and for 2-stage reconstruction averaged 24 weeks. Outcomes included total active motion, tip pinch and grip strength, sensation, and the Pediatric Outcomes Data Collection Instrument (PODCI). RESULTS: Average follow-up was 8 years. At final follow-up, mean total active and passive motion of the involved digit was similar between the primary reconstruction and staged groups, and 58% had a "good" or "excellent" American Society for Surgery of the Hand; total active motion (ASSH TAM) result (71% in the primary repair group, 40% in the 2-stage reconstruction group). All regained grip and pinch strength equal to the contralateral hand. The average PODCI Upper Extremity score was 99 (99 in the primary repair group, 98 in the 2-stage reconstruction group) and PODCI Global Function score was 94 (97 in the primary repair group, 91 in the 2-stage reconstruction group). No complications occurred. CONCLUSIONS: Our small study demonstrates that both primary repair and 2-stage flexor tendon reconstruction have acceptable long-term functional and subjective outcomes in children below 6 years old, although staged reconstruction had a lower overall ASSH TAM score and subcategorical PODCI scores. Although staged reconstruction has acceptable outcomes in this population, prompt primary repair of flexor tendon injuries in children should always be attempted. LEVEL OF EVIDENCE: Level 4-therapeutic.

Camptodactyly Treatment for the Lesser Digits.

Camptodactyly of the lesser digits is commonly seen in patients with arthrogryposis. The flexed posture of the digit can be functionally limiting and necessitate surgical treatment to improve grasp function. In digits with normal bony anatomy, a lateral stiletto-shaped transposition flap combined with flexor digitorum superficialis tendon release, can improve finger deformity and function.

Ulna Growth Patterns After Soft Tissue Release With Bilobed Flap in Radial Longitudinal Deficiency.

BACKGROUND: Centralization is commonly utilized for treating the severely deviated wrist in radial longitudinal deficiency (RLD). Individuals with RLD have congenital shortening of the ulna and previous studies have shown that traditional centralization, in particular with notching of the carpus, results in additional ulnar growth retardation. At our institution, we use a technique of soft tissue release with bilobed flap. We examined if this technique preserves the growth potential of the distal ulna, therefore, avoiding an additionally shortened forearm. METHODS: We retrospectively reviewed serial radiographs of 16 patients with 18 wrists who had at least 3 years of follow-up after a soft tissue release with bilobed flap. Radiographic lengths were measured using the method described by Heikel. Percentage of normal growth was calculated using normative data published by Maresh. Comparisons were made with preoperative, postoperative, and final follow-up studies. RESULTS: The average length of follow-up was 9.2 years (range, 3 to 16.3 y) with an average age of 11.6 years (range, 5.2 to 17.5 y). The average age at the time of surgery was 27 months (range, 14 to 48 mo). A minimum of 3 radiographic studies were available for each subject. The average ulna length preoperatively was 63.9% of age-matched normal length (51.4% to 75.3%). The average ulna length at final follow-up was 61.9% of age-matched normal length (48.5% to 70.3%). The difference was not statistically significant. In addition, there were no distal ulnar physeal arrests. CONCLUSIONS: Soft tissue release with bilobed flap does not affect ulna growth like traditional centralization procedures can. This procedure has previously been shown to retain motion and have similar recurrence rates to formal centralizations. Therefore, we advocate that it be considered more widely for use in patients with RLD and significant wrist deviation to maximize growth and improve hand position. Soft tissue release with bilobed flap can be safely used on young children and preserve ulnar growth. LEVEL OF EVIDENCE: Level IV-therapeutic.

"On-Top Plasty" for Radial Polydactyly Reconstruction.

PURPOSE: To introduce the "on-top plasty" technique and report our long-term outcomes. METHODS: We evaluated 5 thumbs in 5 patients who underwent radial polydactyly reconstruction using the "on-top plasty" technique between 1998 and 2003. This technique was used when it was felt that neither thumb possessed adequate proximal and distal structures to provide a functional and aesthetically pleasing thumb. Our study group included 1 Flatt type III and 4 Flatt type VII thumbs. The average age at time of surgery was 1.4 years and at final follow-up was 13.5 years. Subjective patient evaluation, objective outcomes values, and validated patient-oriented outcome measures were obtained. RESULTS: There was no soft tissue loss and union was achieved in all thumbs with no further surgery required in any thumb. Mean flexion-extension arc for the metacarpophalangeal joint was 60° (range, 10° extension to 70° flexion) and at the interphalangeal joint was 19° (range, 25° extension to 35° flexion). Mean percentage of age-matched norms for lateral, tripod, and tip pinch were 47.0%, 45.9%, and 47.8%, respectively. Mean grip strength was 54.2% of age-matched norm. The mean Pediatric Quality of Life Inventory (PedsQL) score for parent questionnaires was 89.0 and for teen/child questionnaires was 89.1. The Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire revealed a mean score of 4.3. CONCLUSIONS: For patients with radial polydactyly in which neither thumb possesses adequate distal and proximal components, the on-top plasty is a reliable method of polydactyly reconstruction with durable results at longer than 10 years' follow-up. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Long-term Follow-up of Neonatal Brachial Plexopathy: Psychological and Physical Function in Adolescents and Young Adults.

PURPOSE: The prevalence of neonatal brachial plexus palsy (NBPP) has been increasing since the early 1980s. No known studies have examined long-term psychological health and quality of life (QOL) in young adults. The purpose of this study was to investigate the psychosocial and intellectual aspects of NBPP during adolescence into young adulthood. METHODS: A total of 31 patients were enrolled in the adolescent group (16 to 18 y) and 25 in the young adult group (23 to 28 y). Clinical assessment included functional ability, range of motion and strength, weight and body mass index, and education level. Patients were administered measures of psychiatric symptomatology, self-concept, QOL, and cognitive function. RESULTS: Narakas injury level for the adolescent group included 11 level I, 6 level II, 8 level III, and 6 level IV. The young adult group had 10 level I, 2 level II, 9 level III, and 4 level IV. The degree of physical impairment determined by the Modified Mallet Classification showed persistent impairment in both groups. The average DASH scores were higher than the normal range for the adolescent and young adult groups. Forty-five percent of the adolescents and 68% of the young adults were either overweight or obese. All received high school diplomas with 20 of the young adults pursuing higher education.Scores on measures of psychiatric symptomatology and self-concept showed that both groups fell within the normal range. QOL for both groups was also within the normal range. All participants scored average to above average on the cognitive assessment. All measurements were patient reported. CONCLUSIONS: Patients with NBPP can adapt and participate in most activities. This patient sample demonstrated persistent functional limitations and a higher rate of comorbid obesity. However, these patients function psychologically and cognitively within the normal range and many have pursued higher education. LEVEL OF EVIDENCE: Level IV.

Flexor Digitorum Superficialis Opposition Transfer for Augmenting Function in Types II and IIIA Thumb Hypoplasia.

PURPOSE: To report the functional outcomes associated with the flexor digitorum superficialis (FDS) opposition transfer for types II and IIIA thumb hypoplasia and determine if there is any noteworthy difference in the outcome dependent on the pulley used. METHODS: We included patients who underwent a ring FDS opposition transfer and had at least 2 years follow-up. The study group consisted of 36 patients with 40 treated thumbs. All patients underwent follow-up examination and standardized testing. Outcome functional measures were recorded using the Pediatric Outcomes Data Collection Instrument (PODCI). There were 9 type II and 31 type IIIA hypoplastic thumbs. All patients underwent a ring FDS opposition transfer and a 4-flap z-plasty for first web space deepening. The pulley for opposition was flexor carpi ulnaris in 19 and the transverse carpal ligament in 21. Thirty-six thumbs had ulnar collateral ligament reconstructions, with 5 of the 36 undergoing combined ulnar collateral ligament/radial collateral ligament stabilizations. Average follow-up was 7.6 years (range, 2-16 years). RESULTS: Average postoperative Kapandji score was 8 (range, 4-10). Grip, lateral pinch, and tripod pinch strengths averaged 46%, 49%, and 48% of age- and sex-matched normal controls, respectively. There was no significant difference between surgical pulleys used. The Pediatric Outcomes Data Collection Instrument global was 91 (range, 53-100), and PODCI happiness was 87 (range, 15-100). CONCLUSIONS: We found the ring FDS opposition transfer to be an effective method for providing opposition for both type II and IIIA thumb hypoplasia. At follow-up, excellent opposition function and PODCI scores were noted, with no difference related to the type of pulley used. Type II thumbs had significantly greater grip and pinch strengths when compared with type IIIA thumbs.

Somatic gain-of-function mutations in PIK3CA in patients with macrodactyly.

Macrodactyly is a discrete congenital anomaly consisting of enlargement of all tissues localized to the terminal portions of a limb, typically within a 'nerve territory'. The classic terminology for this condition is 'lipofibromatous hamartoma of nerve' or Type I macrodactyly. The peripheral nerve, itself, is enlarged both in circumference and in length. It is not related to neurofibromatosis (NF1), nor is it associated with vascular malformations, such as in the recently reported CLOVES syndrome. The specific nerve pathophysiology in this form of macrodactyly has not been well described and a genetic etiology for this specific form of enlargement is unknown. To identify the genetic cause of macrodactyly, we used whole-exome sequencing to identify somatic mutations present in the affected nerve of a single patient. We confirmed a novel mutation in PIK3CA (R115P) present in the patient's affected nerve tissue but not in blood DNA. Sequencing PIK3CA exons identified gain-of-function mutations (E542K, H1047L or H1047R) in the affected tissue of five additional unrelated patients; mutations were absent in blood DNA available from three patients. Immunocytochemistry confirmed AKT activation in cultured cells from the nerve of a macrodactyly patient. Additionally, we found that the most abnormal structure within the involved nerve in a macrodactylous digit is the perineurium, with additional secondary effects on the axon number and size. Thus, isolated congenital macrodactyly is caused by somatic activation of the PI3K/AKT cell-signaling pathway and is genetically and biochemically related to other overgrowth syndromes.

PIK3CA-related overgrowth spectrum (PROS): diagnostic and testing eligibility criteria, differential diagnosis, and evaluation.

Somatic activating mutations in the phosphatidylinositol-3-kinase/AKT/mTOR pathway underlie heterogeneous segmental overgrowth phenotypes. Because of the extreme differences among patients, we sought to characterize the phenotypic spectrum associated with different genotypes and mutation burdens, including a better understanding of associated complications and natural history. Historically, the clinical diagnoses in patients with PIK3CA activating mutations have included Fibroadipose hyperplasia or Overgrowth (FAO), Hemihyperplasia Multiple Lipomatosis (HHML), Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Scoliosis/Skeletal and Spinal (CLOVES) syndrome, macrodactyly, Fibroadipose Infiltrating Lipomatosis, and the related megalencephaly syndromes, Megalencephaly-Capillary Malformation (MCAP or M-CM) and Dysplastic Megalencephaly (DMEG). A workshop was convened at the National Institutes of Health (NIH) to discuss and develop a consensus document regarding diagnosis and treatment of patients with PIK3CA-associated somatic overgrowth disorders. Participants in the workshop included a group of researchers from several institutions who have been studying these disorders and have published their findings, as well as representatives from patient-advocacy and support groups. The umbrella term of "PIK3CA-Related Overgrowth Spectrum (PROS)" was agreed upon to encompass both the known and emerging clinical entities associated with somatic PIK3CA mutations including, macrodactyly, FAO, HHML, CLOVES, and related megalencephaly conditions. Key clinical diagnostic features and criteria for testing were proposed, and testing approaches summarized. Preliminary recommendations for a uniform approach to assessment of overgrowth and molecular diagnostic testing were determined. Future areas to address include the surgical management of overgrowth tissue and vascular anomalies, the optimal approach to thrombosis risk, and the testing of potential pharmacologic therapies.

Surgical Treatment of Macrodactyly.

Macrodactyly, enlargement of one or multiple digits, was described in the literature nearly 200 years ago. This is an exceptionally uncommon diagnosis that has led to a paucity of descriptive literature on the treatment options. Because the literature is scarce, and the frequency with which hand surgeons encounter macrodactyly is even scarcer, treatment can be a formidable task often left exclusively to those trained in congenital hand deformity. This article presents our algorithm and surgical techniques for dealing with children with macrodactyly in such a way that should make a complex problem more easily approachable.

Defining Features of the Upper Extremity in Holt-Oram Syndrome.

PURPOSE: To investigate the distinguishing morphological characteristics of children with radial longitudinal deficiency (RLD) in Holt-Oram syndrome (HOS). METHODS: One hundred fourteen involved extremities in 62 patients with a diagnosis of HOS were identified at 3 institutions. Medical records and radiographs were evaluated. Radial longitudinal deficiency and thumb hypoplasia were classified according to the modified Bayne and Klug classification and Blauth classifications, respectively, when possible. Other unusual or distinguishing characteristics were catalogued. RESULTS: There was bilateral involvement in 84% of patients. The forearm was involved in 81% of the extremities and a shortened distal radius (Bayne and Klug type I RLD) was the most commonly identified forearm anomaly (40%). Radioulnar synostosis was present in 15% of the extremities, all in the proximal forearms with reduced radial heads. Thumb aplasia (Blauth type V hypoplastic thumb) was the most common type of classifiable thumb abnormality and occurred in 35% of involved thumbs. Twenty-seven percent of abnormal thumbs affected were not classifiable according to the Blauth classification, and 19% of involved thumbs (hypoplastic or absent) had first-web syndactyly. CONCLUSIONS: The upper extremity in HOS differs from the typical presentation of RLD. The forearm is more often involved and may demonstrate radioulnar synostosis. The thumb is frequently unclassifiable by the Blauth classification and has first-web syndactyly. The presence of radioulnar synostosis and syndactyly of the radial 2 digits in RLD should prompt the hand surgeon to obtain a cardiac evaluation and consider genetic testing for HOS. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic III.

Soft tissue release and bilobed flap for severe radial longitudinal deficiency.

PURPOSE: To report the hand position, range of motion, functional results, and radiographic outcomes associated with treating radial longitudinal deficiency with release of constricting or deforming soft tissue and resurfacing of the radial skin deficiency with a bilobed flap. METHODS: We recalled and reviewed patients with at least a 3-year follow-up who had undergone soft-tissue release and coverage with a bilobed flap. The study group consisted of 16 patients and 18 wrists. All patients underwent follow-up examination and radiographs. Outcome measures using Pediatric Outcomes Data Collection Instrument (PODCI), Disabilities of the Arm, Shoulder, and Hand (DASH), and visual analogue scale (VAS) scores were recorded. RESULTS: At a mean of 9.2 years follow-up, the average final resting wrist radial deviation angle was 64° compared with 88° preoperatively. The average active wrist flexion-extension arc was 73°. Average DASH score was 27 (range, 5-54). PODCI global was 88 (range, 75-97), PODCI happiness was 86 (range, 70-100), and VAS overall satisfaction (range, 0-10) was 1.2 (range, 0-8). At final follow-up, no physeal growth arrests were noted on radiographs, and no patients to date have required ulnocarpal arthrodesis. CONCLUSIONS: Soft-tissue release and coverage with a bilobed flap should be considered in the treatment algorithm for patients with radial longitudinal deficiency. Outcome measures show that these patients maintain useful active motion, and along with their parents, are satisfied with both the appearance and function. Some recurrence of radial deviation was noted, which was similar to results previously reported following centralization/radialization procedures, although with a lower inherent risk of both physeal injury to the ulna and stiffness. In addition, potential future procedures are not compromised by this surgical approach. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Digital Chondrolysis and Epiphysiolysis Associated With Snakebite: A Report of 2 Cases.

BACKGROUND: Here, we present 2 cases of the unusual sequelae of a venomous bite to the finger in children resulting in chondrolysis and physeal loss. There have been few isolated case reports documenting this phenomenon. Currently, there is no preventative treatment, and patients should be warned of this possible complication of envenomation. METHODS: Two patients with chondrolysis and physeal loss have been seen in our practice. RESULTS: Chondrolysis and epiphysiolysis occurred in 2 patients. One patient was treated with proximal interphalangeal joint fusion and one is being managed conservatively. CONCLUSION: The toxic action of snake venom may cause loss of the growth plate and chondrolysis in the pediatric hand. LEVEL OF EVIDENCE: Level IV.

Long-term outcomes following radial polydactyly reconstruction.

PURPOSE: To report long-term outcomes (> 10 y) after radial polydactyly reconstruction. METHODS: We evaluated 43 surgically reconstructed thumbs in 41 patients with radial polydactyly whom we had observed for more than 10 years. The study group included 12 Flatt type II, 8 type III, 17 type IV, and 6 type V. The average age of surgery was 1 year and mean follow-up was 17 years. Objective outcome values and validated patient-oriented outcome evaluations were obtained. RESULTS: No early postsurgical complications were encountered. Eight patients had 10 revision procedures at an average of 8 years after the initial procedure. Five patients had interphalangeal joint arthrodesis, all for angulation with accompanying pain. The average Tada score was 4.1. Lateral, tripod, and tip pinch strengths were 96%, 86%, and 92%, respectively, of the unaffected side. As a group, the treated thumbs had significantly weaker tip and tripod pinch strengths than the untreated thumbs. The average Disabilities of the Arm, Shoulder, and Hand score was 4.5 and the average Pediatric Quality of Life Inventory score was 87 when administered to both the patient and the parent. CONCLUSIONS: Long-term results after surgical reconstruction for radial polydactyly were excellent but the revision rate trended upward over time despite maintenance of favorable scores on the objective outcome measures used. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Congenital band syndrome with pseudarthrosis of the radius and ulna and impending vascular compromise: a case report.

BACKGROUND: Although amniotic band syndrome is relatively rare, reports of pseudarthrosis in conjunction with amniotic band syndrome are even rarer, as are reports of impending vascular compromise in the neonatal period. Careful serial examinations and timely surgical intervention can successfully avoid the catastrophic event of limb loss. We report on a case of upper extremity amniotic band syndrome with pseudarthrosis of the radius and ulna that was complicated by vascular compromise in a neonate. METHODS: Chart and radiographic data for this single case were reviewed and reported retrospectively. RESULTS: A 1-day-old neonate born at 28 3/7 weeks of gestational age was transferred to our institution for increased swelling to the forearm distal to a congenital band associated with an underlying radius and ulna pseudarthrosis. Although the forearm and hand were soft and viable initially, severe edema and swelling occurred after fluid resuscitation, and on the fourth day of life, the patient underwent simple band releases at bedside with 2 longitudinal incisions over the radius and ulna. Circulation was restored, and the pseudarthrosis healed with no further surgical intervention. Successful delayed reconstruction of the band with Z-plasties was performed when the baby was 7 months of age. CONCLUSIONS: In this case, a relatively simple, straightforward procedure that is familiar to most pediatric orthopaedists salvaged a compromised neonatal limb with amniotic band syndrome and allowed healing of a pseudarthrosis, allowing more complex reconstruction to be performed in a delayed, elective manner. Careful observation is necessary in the neonatal period of the baby with a severe band; a viable, well-perfused, compressible extremity may still be at risk.

Segmental metadiaphyseal humeral bone loss in pediatric trauma patients: a case series.

BACKGROUND: The management of posttraumatic bone loss is complicated and often requires complex reconstructive procedures. No options exist that are specific to the treatment of the growing skeleton that has intercalary bone loss. We have observed reconstitution of the humerus in 2 cases that have precluded extensive management. METHODS: Two pediatric patients sustained traumatic injuries to the upper extremities, including humeral bone loss, and are presented after spontaneous reconstitution of the segmental bone loss. RESULTS: With treatment restricted to soft-tissue injury and bone stabilization with external fixation, both patients demonstrated radiographic healing of humeral segmental bone loss. Both patients were thought to have a partially intact periosteal sleeve. They have returned to sporting activities with mild loss of function. CONCLUSIONS: In certain pediatric injuries, spontaneous healing of segmental bone defects can occur. This response may obviate the need for complex, interventional procedures. LEVELS OF EVIDENCE: Level IV-case series.