Janith Mills

Thrombocytopenia Secondary to High Valproate Levels in Children With Epilepsy

We reviewed the frequency of valproate-induced thrombocytopenia in children with epilepsy in our institution. Sixty-four (21%) of 306 children taking valproate developed thrombocytopenia. Thirty-two of these 64 patients had at least one platelet count lower than 100 × 103/mm3. Eight patients developed signs of bleeding. Low platelet levels were typically noted in patients with serum valproate levels of over 140 μg/mL, and reduction of the medication dose usually resulted in a prompt increase in the number of platelets. Only one patient developed thrombocytopenia unrelated to high serum drug levels, and her platelet count did not improve until the drug was discontinued. Neither the age of the patient nor the use of additional antiepileptic medication correlated with the platelet count. However, duration of valproate use was related. These data suggest that, although valproate may cause thrombocytopenia via more than one mechanism, by far the most common factor is the presence of high valproate levels. Thus, the medication can be safely lowered in most patients with thrombocytopenia rather than discontinued altogether. Platelet counts should probably be monitored more carefully in patients known to have higher drug levels. (J Child Neurol 1994;9:311-314).

Upper-extremity Phocomelia Reexamined: A Longitudinal Dysplasia

BACKGROUND In contrast to longitudinal deficiencies, phocomelia is considered a transverse, intercalated segmental dysplasia. Most patients demonstrate severe, but not otherwise classifiable, upper-extremity deformities, which usually cannot be placed into one of three previously described phocomelia groups. Additionally, these phocomelic extremities do not demonstrate true segmental deficits; the limb is also abnormal proximal and distal to the segmental defect. The purpose of this investigation was to present evidence that upper-extremity abnormalities in patients previously diagnosed as having phocomelia in fact represent a proximal continuum of radial or ulnar longitudinal dysplasia. METHODS The charts and radiographs of forty-one patients (sixty extremities) diagnosed as having upper-extremity phocomelia were reviewed retrospectively. On the basis of the findings on the radiographs, the disorders were categorized into three groups: (1) proximal radial longitudinal dysplasia, which was characterized by an absent proximal part of the humerus, a nearly normal distal part of the humerus, a completely absent radius, and a radial-sided hand dysplasia; (2) proximal ulnar longitudinal dysplasia, characterized by a short one-bone upper extremity that bifurcated distally and by severe hand abnormalities compatible with ulnar dysplasia; and (3) severe combined dysplasia, with type A characterized by an absence of the forearm segment (i.e., the radius and ulna) and type B characterized by absence of the arm and forearm (i.e., the hand attached to the thorax). RESULTS Twenty-nine limbs in sixteen patients could be classified as having proximal radial longitudinal dysplasia. Systemic medical conditions such as thrombocytopenia-absent radius syndrome were common in those patients, but additional musculoskeletal conditions were rare. Twenty limbs in seventeen patients could be classified as having proximal ulnar longitudinal dysplasia. Associated musculoskeletal abnormalities, such as proximal femoral focal deficiency, were common in those patients. Eleven limbs in ten patients were identified as having severe combined dysplasia, which was type A in seven of them and type B in four. Four patients with severe combined dysplasia had congenital cardiac anomalies, and four had associated musculoskeletal abnormalities. Three of the four patients with the type-B disorder had a contralateral ulnar longitudinal dysplasia. CONCLUSIONS We propose that cases previously classified as upper-extremity phocomelia represent a spectrum of severe longitudinal dysplasia, as none of the sixty extremities that we studied demonstrated a true intercalary deficiency. These findings have both developmental and genetic implications.

Intraobserver and interobserver reliability of two ultrasound measures of humeral head position in infants with neonatal brachial plexus palsy.

BACKGROUND Ultrasonographic evaluation of the hip in infants is considered both reliable and reproducible in the diagnosis of developmental dysplasia of the hip. Ultrasonographic evaluation of the shoulder in infants has been reported as a valuable diagnostic aid in dysplastic development following neonatal brachial plexus palsy. To our knowledge, there has been no study of the intraobserver reproducibility and interobserver reliability of sonography of the shoulder in infants with and without suspected posterior shoulder dislocation. METHODS Two identical but randomly ordered sets of the same deidentified sonographic images of shoulders in infants were given to radiologists, pediatric orthopaedists and orthopaedic residents, and fellows with varying degrees of experience in the evaluation of shoulder pathology in infants, who measured the position of the humeral head relative to the axis of the scapula. Intraobserver reproducibility and interobserver reliability of the measurements were assessed. RESULTS For the position of the humeral head with respect to the glenoid in both normal and abnormal conditions, the Pearson correlation coefficient for intraobserver reproducibility was 0.91 and the intraclass correlation coefficient for interobserver reliability was 0.875. For estimating the percentage of the humeral head posterior to the axis of the scapula, the Pearson correlation was 0.85 and the intraclass correlation coefficient was 0.77. CONCLUSIONS Ultrasonographic examination of the shoulder in infants to assess for the position of the humeral head with respect to the scapula showed high intraobserver reproducibility and interobserver reliability. It is recommended as a reliable technique for evaluating shoulder position in infants with neonatal brachial plexus palsy.

OnabotulinumtoxinA Injection as an Adjunct in the Treatment of Posterior Shoulder Subluxation in Neonatal Brachial Plexus Palsy

BACKGROUND Botulinum toxin A is used to treat contractures in children with spasticity by temporarily interfering with neural transmission at the motor end plate. In infants with brachial plexus palsy, posterior shoulder subluxation and dislocation are the result of muscle imbalance, in which neurologic recovery is evolving, and spasticity is not a deforming force. We postulated that temporary weakening of the shoulder internal rotator muscles with botulinum toxin A would facilitate reduction of the glenohumeral joint in such infants with early posterior shoulder subluxation or dislocation. METHODS Thirty-five infants with posterior subluxation or dislocation of the shoulder due to brachial plexus palsy were treated with botulinum toxin A between January 1999 and December 2006, and were followed for a minimum period of one year. Records were reviewed for the severity of the palsy, age at time of treatment, recurrence of subluxation or dislocation, and the subsequent need for further treatment to reduce the glenohumeral joint. RESULTS The average age at the time of shoulder reduction and botulinum toxin-A injection was 5.7 months. Six patients had a second injection. Reduction of the shoulder was maintained in twenty-four (69%) of the thirty-five patients. There were no complications related to the use of botulinum toxin A. CONCLUSIONS Although there may be specific risks associated with its use, botulinum toxin-A injection into the internal rotator muscles is a useful adjunct to the treatment of early posterior subluxation or dislocation of the shoulder in infants with neonatal brachial plexus palsy, and may help to avoid the need for open surgical procedures to restore or maintain shoulder reduction.

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.

Incidence and prognosis of neonatal brachial plexus palsy with and without clavicle fractures.

OBJECTIVE: To report the incidence of neonatal brachial plexus palsy with and without ipsilateral clavicle fracture in a population of newborns and to compare the prognosis between these subgroups. METHODS: This was a retrospective review of 3,739 clavicle fractures and 1,291 brachial plexus palsies in neonates over a 24-year period from a geographically defined health care system with reference to county-wide population data. RESULTS: A referral clinic for children with brachial plexus palsies evaluated 1,383 neonates, of whom 320 also had ipsilateral clavicular fracture. As a result of referral patterns within the region, it is likely that this represents nearly all infants from the area with persistent brachial plexus injury after 2 months of age. Among the children evaluated without concomitant clavicular fracture, 72% resolved spontaneously (154/214); among those with concomitant clavicular fracture, 74% healed spontaneously (55/74). Limiting the analysis to neonates delivered at Parkland Memorial Hospital and assuming that those neonates with a discharge diagnosis of brachial plexus injury with or without clavicular fracture who did not present to the referral brachial plexus injury clinic had complete resolution, 94.4% without clavicular fracture resolved and 98.1% with clavicular fracture resolved (P=.005). CONCLUSIONS: The risk of persistent neurologic deficit from a birth-related brachial plexus palsy is lower than what has been reported, and the presence of a clavicle fracture may improve the likelihood of recovery. LEVEL OF EVIDENCE: III

Long-term outcome following carpal wedge osteotomy in the arthrogrypotic patient.

BACKGROUND Wrist flexion and ulnar deviation deformity is a common presentation in children with amyoplasia congenita. Multiple surgical procedures have been reported to correct the deformity to enhance functional independence and improve quality of life. We performed a retrospective review to detail our long-term results with carpal wedge osteotomy in these patients. METHODS Medical records of all patients with the amyoplasia form of arthrogryposis who underwent carpal wedge osteotomy between 1994 and 2008 were reviewed. Patients with a follow-up of two years or less were excluded. Preoperative and postoperative resting position and range of motion of the wrist were recorded. Interviews and questionnaires were completed to assess the mean overall satisfaction level of the parent or guardian with the outcome of surgery, function, and task completion with use of parent-guardian surveys, the Manual Ability Classification System, and the ABILHAND-Kids measure of manual ability. RESULTS Seventy-five wrists in forty-six patients who met the inclusion criteria were reviewed. The average age of the patients at the time of surgery was 4.3 years (range, nine months to eighteen years; median, 2.7 years). The average duration of follow-up was 5.7 years (range, two to 10.3 years; median, 5.3 years). The average resting position of the wrist postoperatively (11° of flexion) was significantly different from that measured preoperatively (55° of flexion) (p < 0.001). The arc of wrist motion measured preoperatively (32°) did not differ significantly from that measured postoperatively (22°) (p = 0.4903). The location of the motion arc was significantly improved to a more functional position. The average active extension of the wrist changed from -37° of extension preoperatively to -11° of extension postoperatively (p < 0.001). Active wrist flexion also significantly changed from 69° preoperatively to 33° postoperatively (p < 0.001). Parent-guardian surveys indicated that the mean overall satisfaction score after surgery was 9.1 of 10 possible points and that the mean ranking for task completion in activities of daily living was 4 (easier following surgery). CONCLUSIONS Long-term outcomes reveal that surgical correction of wrist flexion posture in children with amyoplasia congenita results in improvement that is sustained over time. The surveys and questionnaires completed by parents or guardians indicated that they were satisfied with the results of the operation.

Volar Ligament Release and Distal Radial Dome Osteotomy for the Correction of Madelung Deformity: Long-Term Follow-up

BACKGROUND Madelung deformity is a disorder of growth of the distal aspect of the radius that is usually recognized in late adolescence near skeletal maturity. It results in a characteristic wrist deformity, decreased wrist motion, and wrist pain. The purpose of this study was to evaluate long-term results in patients treated by volar ligament release and distal radial dome osteotomy for Madelung deformity. METHODS Patients who had undergone volar ligament release and dome osteotomy for Madelung deformity at our institution from 1990 to 2002 and who were the subjects of a previous report on this treatment were contacted for clinical and radiographic evaluation at mid-term to long-term follow-up. Forearm and wrist motion was evaluated. Posteroanterior and lateral radiographs of both forearms were assessed for radial inclination, lunate subsidence, and arthritis changes. A Disabilities of the Arm, Shoulder and Hand (DASH) survey was completed. RESULTS Twenty-seven patients underwent volar ligament release and distal radial dome osteotomy. Eight patients were either lost to follow-up or were unable to return for follow-up. Nineteen patients with thirty-one operatively treated wrists were available for follow-up. After further review, eighteen patients and twenty-six wrists were included in the study. The average age at the time of follow-up was twenty-five years (range, nineteen to thirty-one years), with an average length of follow-up of eleven years (range, seven to fourteen years). There was no change in radial inclination or in wrist motion between the immediate postoperative and long-term follow-up evaluations. There was a positive correlation between the amount of deformity correction based on more severe preoperative parameters and an increased arthritic grade at the time of follow-up. There was positive correlation between an increased DASH score and arthritis grade as well as a correlation between whole bone deformity and increased arthritis grade and DASH score. CONCLUSIONS Volar ligament release and distal radial dome osteotomy for Madelung deformity provides lasting correction of the deformity. Long-term follow-up shows maintenance of original radiographic correction with good to excellent functional outcome. Patients with radiographic evidence of more severe disease preoperatively and the whole bone variety of Madelung deformity have poorer radiographic outcomes and trend toward poorer functional outcomes.

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.

Ulnar Polydactyly Long-Term Outcomes and Cost-Effectiveness of Surgical Clip Application in the Newborn

Background. Postaxial polydactyly type B (PAPD-B) refers to the nonfunctional, floppy extra digit on the ulnar border of the hand. Suture ligation is applied in the newborn unit if the base is narrow or pedunculated. However, wart-like scars, residual bumps, or neuromas are frequent complications. Wider-based extra digits are treated at a later age by surgical excision under general anesthesia. Surgical clip application expands the indications for PAPD treatment in the newborn unit or outpatient setting with lesser incidences of complications. Design. A retrospective review identified 231 hands with PAPD-B in 132 newborns treated with surgical clips between January 1, 1996, and November 30, 2010, having a minimum of 2 years of follow-up. Medical records were queried for complications, revision procedures, and parent satisfaction. A relative cost survey compares the costs of surgical clips to surgery. Conclusions. In all, 16 extremities in 9 patients (7%) required surgical scar revision. No wound complications were noted.