Does Preservation of Metacarpophalangeal Joint Vascularity Protect Against Physeal Arrest in Index Finger Pollicization?

PURPOSE: 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. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Elbow Release and Tricepsplasty in Arthrogrypotic Patients: A Long-Term Follow-Up Study.

PURPOSE: Obtaining elbow flexion to improve hand-to-mouth reach capability is an essential component of achieving functional independence in pediatric patients with arthrogryposis. This study analyzed the long-term outcomes of elbow release and tricepsplasty in a series of children with arthrogryposis at a tertiary institution. METHODS: Medical records of patients with arthrogryposis who underwent elbow release and tricepsplasty from 1993 to 2015, with at least 2 years of follow-up, were reviewed. Collected measures included preoperative elbow passive range of motion (ROM), postoperative elbow passive and active ROM, shoulder passive and active ROM, and Pediatric Outcomes Data Collection Instrument (PODCI) scores. Our analysis compared pre- and postoperative follow-up of elbow passive ROM and reviewed PODCI scores with age-adjusted normative values. RESULTS: Seventeen patients (4 female and 13 male) with 24 affected upper extremities (10 left elbow and 14 right elbow) were included in final analysis. Age at final follow-up averaged 11 years (range, 4-20 years), mean age at surgery was 2.7 years (range, 9.6 months-9.3 years) with mean follow-up by extremity at 8.3 years (range, 2-18 years). Differences in pre- and post-operative passive elbow ROM were significant for extension, flexion, and total arc of motion. Most parent and self-reported PODCI scores were less than the age-adjusted normal population, except in the domains of Comfort and Pain and Happiness. CONCLUSION: Long-term follow-up of elbow release and tricepsplasty in patients with arthrogryposis indicates both increased and sustained elbow flexion and arc of motion. Although PODCI scores were lower compared with the age-adjusted norm, pediatric patients with arthrogryposis were just as happy and had no more discomfort than their unaffected age-adjusted norms. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

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.

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.

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.

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.

Treatment of the Upper Extremity Contracture/Deformities.

Patients with arthrogryposis multiplex congenita have a characteristic upper extremity resting posture consisting of internal rotation of the shoulders, elbow extension, flexed wrists, thumb-in palm deformities, and variable degrees of finger contractures. Treatment of these patients is aimed at improving independence and performance of activities of daily living. Although each area needs to be assessed independently for the most appropriate surgical procedure, often multiple areas can be addressed at the same operative setting. This limits the number of anesthetic exposures and cast immobilization time. The following is a synopsis of treatment strategies presented at the second international symposium on Arthrogryposis which took place in St Petersburg in September 2014.

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.

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.

Hand therapy following elbow release for passive elbow flexion and long head of the triceps transfer for active elbow flexion in children with amyoplasia.

Children born with the Amyoplasia form of arthrogryposis multiplex congenita (AMC) who lack elbow flexor muscles and have elbow extension contractures usually require assistance in performing ADL's that require reaching their hand to their face, head, and upper body. For tasks involving the UE, the elbow is the key to functional independence. Children born with Amyoplasia may benefit from selective surgeries to enhance functional independence and improve quality of life. Home therapy and splinting following these surgeries is an important part of gaining motion and improving function. This abstract will review the rehab and splinting following an elbow release to gain passive elbow flexion, and a long head of the triceps transfer for active elbow flexion in children with Amyoplasia.

A case of familial syndactyly associated with eye and dental abnormalities.

Syndactyly occurs in 1 in 2,000 live births and is more common in white children. This article describes a patient with syndactyly and additional abnormalities indicating oculodentodigital dysplasia.

Type A ulnar polydactyly of the hand: a classification system and clinical series.

PURPOSE: To propose a classification system for type A ulnar polydactyly based on radiographic findings and characterize the demographic features of patients with these deformities. METHODS: We identified 49 patients with type A ulnar polydactyly of the hand who were seen in our institution over 20 years. Patients' medical records and radiographs were retrospectively reviewed and used to distinguish morphological subtypes. RESULTS: Ninety-six percent of the deformities (64/67) were allocated to 1 of the 5 subgroups of our suggested classification, and the type that originated from the metacarpophalangeal joint was the most common. Sixty-nine percent of patients in our series (34/49) had either bilateral type A or a contralateral type B ulnar polydactyly, and 63% (31/49) had ulnar polydactyly of one or both feet. Twenty-four percent of patients (12/49) had associated syndromes or congenital anomalies involving areas other than the hand or foot. The most common syndrome associated with type A ulnar polydactyly was chondroectodermal dysplasia (n = 3). Sixty-five percent of the patients (32/49) were Caucasian, 20% were Hispanic (10/49), 12% were African American (6/49), and one was Asian. The percentage of African Americans in our series was similar to that in the general patient population seen in our institution. CONCLUSIONS: The majority of type A ulnar polydactyly can be classified into 1 of 5 morphological subtypes that have potential clinical relevance regarding surgical treatment. In patients with type A ulnar polydactyly, contralateral hand and foot polydactyly is frequent. Associated congenital anomalies and syndromes can also be present.

Technique of forearm osteotomy for pediatric problems.

Correction of a rigid forearm deformity in children is often desired in congenital radioulnar synostosis, brachial plexus palsy, cerebral palsy, or posttraumatic torsional deformity. Osteotomies at the diaphyseal level present difficulties with maintenance of reduction, whether or not internal or pin fixation is used. The stabilizing and healing potential of the periosteum in these cases can be used to advantage in the correction of these deformities.

Unilateral congenital terminal finger absences: a condition that differs from symbrachydactyly.

PURPOSE: To describe a type of nonhereditary unilateral transverse deficiency, which we have named hypodactyly, that is distinct from symbrachydactyly or amniotic disruption sequence. METHODS: We identified 19 patients with unilateral congenital anomalies consisting of absent or short bulbous fingers that lack terminal ectodermal elements. Medical records and radiographs were retrospectively reviewed and contrasted with the typical findings of symbrachydactyly and amniotic disruption sequence. RESULTS: No associated syndromes or potentially causative diagnoses were identified in the hypodactyly patients. The digital absences were of a truncated pattern with thickened, tubular soft tissue coverage. Radiographs revealed a pattern of severity progression that is different from that of symbrachydactyly. Distal phalanges were the bony elements absent most frequently, followed sequentially by the middle phalanx and proximal phalanx. In all cases, metacarpals were present. Unlike symbrachydactyly, the ulnar 2 digits were more involved than the index and long fingers, and the thumb was the least involved digit. CONCLUSIONS: Hypodactyly appears to be a congenital hand anomaly that is clinically and radiographically different from symbrachydactyly or amniotic disruption sequence and is presumed to be caused by a distinct pathomechanism. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

Venous thrombosis and pulmonary embolus in pediatric patients with large upper extremity venous malformations.

Patients with large venous malformations are at risk for deep venous thrombosis and pulmonary embolus. Currently, there is no general consensus on the proper treatment for these patients. We present 3 preadolescent patients with large upper extremity venous malformations, who developed deep venous thrombosis; 2 had documented pulmonary emboli, one of which was fatal. It is imperative that patients and families be educated regarding the potential life-threatening sequelae that may be associated with these large vascular malformations.

Thrombocytopenia absent radius syndrome: presence of brachiocarpalis muscle and its importance.

PURPOSE: Common clinical manifestations associated with thrombocytopenia absent radius (TAR) syndrome have been reported in the literature since its initial description in 1959. The purpose of this study was to report a clinical observation and describe the anatomic location of this brachiocarpalis muscle. METHODS: The radiographs of 20 patients (39 extremities) with TAR syndrome were reviewed and are the focus of this report. The presence or absence of an abnormal muscle crossing both the humero-ulnar and ulno-carpal joint, and its anatomic origin and insertion, were documented by radiographs, surgery, or both. A total of 12 patients underwent surgery on 19 extremities. Furthermore, we compared the radiographs of these 20 patients (39 extremities) with the radiographs of 20 arbitrarily selected patients with non-TAR type IV radial dysplasia, which were part of our database. RESULTS: An abnormal brachiocarpalis muscle was noted radiographically in all 39 of the extremities and was observed surgically in all 19 patients who underwent surgery. It was absent in all 40 extremities of the non-TAR type IV radial dysplasia patients. One TAR patient had only unilateral radial dysplasia. This anomalous muscle has an abnormally high origin on the anterolateral aspect of the proximal humerus, and an insertion into the radial side of the carpus. As such, it has a deforming force at both the wrist and the elbow. CONCLUSIONS: We report the consistent finding of a brachiocarpalis muscle in patients with TAR syndrome. Because of its location, the presence of this muscle may influence the surgical treatment of these patients. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic IV.

Macrodactyly: decision-making and surgery timing.

Macrodactyly is a rare condition in which fingers, hands or limb growth is unregulated, resulting in overgrowth of tissues in the affected extremities. It is critical to properly assess these extremities for signalling pathway, psychological impact and potential surgical intervention, to achieve the best possible outcome for each patient. Treatment approaches can vary, and patient and family expectations weigh heavily on care complexity. Common surgical procedures may include epiphysiodeses, osteotomies, debulking procedures, carpal tunnel releases, toe transfers and amputations. The selection and timing of these surgeries is a vital component of the approach, as delayed healing and excessive scarring may occur. The purpose of this review is to assist in the navigation of decision-making and surgical timing for patients presenting with overgrowth manifesting itself as macrodactyly.

Exertional Compartment Syndrome with Amniotic Band Disruption Sequence: A Report of Two Cases.

CASES: We report the cases of two female patients who presented with symptoms of lower-extremity exertional compartment syndrome years after initial amniotic band reconstruction. These patients underwent extensive fascial release for treatment of amniotic band disruption sequence (ABDS) of the lower extremity, with resolution of their symptoms. We describe our current technique that is now performed at the time of initial reconstruction for addressing all layers of the deformity. CONCLUSION: The surgical reconstruction of ABDS requires addressing all layers of the deformity. Failure to do so may lead to long-term sequelae. Both patients in this report had exertional compartment syndrome following incomplete fascial release at their primary surgery, requiring subsequent fasciotomies.

Treatment of Kienbock's disease with capitohamate arthrodesis: pain relief with minimal morbidity.

Despite the large number of procedures available for treatment of Kienbock's disease, no single method has emerged as being clearly superior. Ultimately, the goal of treatment must be the relief of pain and maintaining wrist range of motion. The authors' experience with 45 consecutive wrists that had undergone capitohamate fusion for treatment of Lichtman's stage 1, 2, or 3 Kienbock's disease is presented. Average follow-up was 32 months (range, 4 to 107 months). All arthrodeses healed with an average time to fusion of 1.9 months. Postoperatively, 93 percent of patients had either no pain or less pain than they had preoperatively, with preservation of wrist range of motion and improved grip strength (52 percent of normal preoperatively to 72 percent of normal postoperatively). The authors conclude that capitohamate arthrodesis relieves pain in 93 percent of patients with stage 1, 2, or 3 Kienbock's disease and is an effective treatment for this disease.