Four-Year Follow-Up of the World's First Pediatric Bilateral Hand-Forearm Transplants: Do They Grow as Expected?

BACKGROUND: In 2015, the first bilateral pediatric hand-forearm transplant was performed in an 8-year-old boy. The growth rate of the transplanted upper extremities was unknown at the time. METHODS: Forearm and hand radiographs were obtained annually. Radius and ulna measurements were performed by multiple coauthors and mathematically normalized using a standardized methodology. The Greulich and Pyle atlas was used to estimate hand bone age. RESULTS: From July of 2015 to July of 2019, unadjusted bone length (metaphysis to metaphysis) increased 38.8 and 39.6 mm for the left radius and ulna, and 39.5 and 35.8 mm for the right radius and ulna, respectively. Distal physes of the donor limbs increasingly contributed to overall bone length relative to proximal physes. Normalized growth between the two limbs was statistically similar. At each annual follow-up, the bone age increased by 1 year. CONCLUSIONS: Successful pediatric hand-forearm transplantation offers the advantage of growth similar to that of nontransplanted pediatric patients. The transplanted distal physes contributes more to the overall growth, paralleling normal pediatric growth patterns. Chronologic age parallels the increase in bone age. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Early initiation of smoking and alcohol drinking as a predictor of lower forearm bone mineral density in late adolescence: a cohort study in girls.

BACKGROUND: Adolescence is a critical stage for bone accrual. It is also decisive for the establishment of behaviors such as smoking and alcohol drinking. OBJECTIVE: To quantify the short- and long-term associations between smoking and drinking initiation and bone mineral density in adolescent girls. METHODS: We used prospective data from 731 girls identified in public and private schools in Porto, Portugal. Evaluations were conducted when participants were 13 and 17 years old. Bone mineral density (BMD) was measured at the forearm by dual-energy X-ray absorptiometry and weight, height and fat-free mass were measured. Pubertal development status was estimated using menarche age. Self-administered questionnaires were used to collect data on smoking and alcohol drinking, physical exercise and calcium and vitamin D intakes. BMD in early and late adolescence was analyzed as a continuous or dichotomous (Z-score cutoff: -1.0) variable. Associations were calculated using linear or logistic regression. RESULTS: Over one quarter of these girls had tried smoking by 13, while 59% had drunk alcoholic beverages and 20% had experienced both behaviors by that age. Lower mean BMD at 17 years of age was observed in girls who had ever smoked by 13, as well as in those who reported drinking at that age. There were no significant cross-sectional associations between experience and frequency of smoking or drinking and BMD at 13 years of age. However, we observed significant associations between BMD z-score<-1 in late adolescence and having ever smoked by 13, after adjustment for menarche age and sports practice, (OR = 1.92; 95% CI: 1.21, 3.05) and with ever smoking and drinking in the same period (OR = 2.33; 95% CI: 1.36, 4.00). CONCLUSION: Our study adds prospective evidence to the role of early initiation of smoking and alcohol drinking as relevant markers of lower bone mineral density in late adolescence.

Radiographic early to midterm results of distraction osteogenesis in radial longitudinal deficiency.

PURPOSE: To analyze early to midterm radiographic results after forearm lengthening in children with radial longitudinal deficiency. METHODS: We conducted a retrospective chart review of patients with radial longitudinal deficiency undergoing distraction osteogenesis with an Ilizarov device. We retrospectively reviewed 8 lengthening procedures in 6 children with respect to distraction details and assessed anteroposterior and lateral radiographs of the hand and forearm of the preoperative and postoperative follow-up investigations. RESULTS: The mean age at time of ulna lengthening was 9.9 years (range, 6.3-14.0 y). The mean follow-up period was 4.7 years (range, 1.0-8.5 y). Mean lengthening of the ulna was 7.0 cm (range, 3.5-8.7 cm), and the mean length gain of the ulna compared with its preoperative length was 75% (range, 42% to 103%). The mean ulna bowing was 25° preoperatively (range, 7° to 42°), 6° after forearm distraction (range, 0° to 14°), and 17° at latest follow-up (range, 0° to 45°). The mean hand-forearm angle was 25° of radial deviation preoperatively (range, 15° ulnar to 60° radial deviation), 11° of radial deviation after distraction (range, 0° to 41°), and 23° at latest follow-up (range, 0° to 45°). We encountered 2 major complications: 1 ulna fracture after removal of the Ilizarov device and 1 insufficient bone regenerate during lengthening. CONCLUSIONS: We achieved both deformity correction and improvement of limb length after distraction osteogenesis with an Ilizarov device. However, some of the deformity-in particular, ulnar bowing and radial deviation of the hand-recurred at midterm follow-up.

Correction of severe wrist deformity following physeal arrest of the distal radius with the aid of a three-dimensional computer simulation.

Growth arrest following physeal injury may result in severe limb deformity. We report a case of complex wrist deformity caused by injury to the distal radial physis resulting in radial shortening and abnormal inclination of the radial articular surface, which was successfully treated by gradual correction after computer simulation. The simulation enabled us to develop an appropriate operative plan by accurately calculating the axis of the three-dimensional (3D) deformity using computer bone models. In the simulative surgery with a full-size stereolithography bone model, an Ilizarov external fixator was applied to the radius such that its two hinges were located on the virtual axis of the deformity, which was reproduced in the actual surgery. This technique of 3D computer simulation is a useful alternative to plan accurate correction of complex limb deformities following growth arrest.

Radiological aspects of the Poland syndrome and implications for treatment: a case study and review.

UNLABELLED: Poland syndrome (PS) (OMIM 173800) is a rare congenital anomaly classically consisting of the combination of unilateral aplasia of the sternocostal head of the major pectoral muscle and an ipsilateral hypoplastic hand with simple syndactyly and short fingers. The aetiology is most probably a vascular disruption sequence of the subclavian arteries. In most cases, PS is sporadic. Familial occurrence suggests that genes exist which are involved in the pathogenesis as paradominant traits. The syndrome may include mammary hypoplasia and further muscle abnormalities which can be accurately defined by sonography and MRI. The evaluation of the vascular status can be performed using colour coded duplex sonography for peripheral arteries and contrast-enhanced MR-angiography for supra-aortic arteries. CONCLUSION: We report a 7-year-old girl with unilateral right sided Poland syndrome with particular emphasis on the radiological investigations of vascular abnormalities. A review of the literature concerning the origin, outcome, and implications for treatment is given.

[Ulnar lengthening in osteochondroma (multiple cartilagenous exostoses) of the forearm]. / Die Ulnaverlängerung beim Osteochondrom (multiple kartilaginäre Exostosen) am Unterarm.

The osteochondroma is the most frequent bone tumor to occur during the period of growth. The multiple hereditary form often involves the forearms. Depending on localisation and size of the tumor, shortening of the bones in the forearm, angular malalignments and functional impairment of the wrist and elbow joints may result. Early diagnosis and surgery in the growing child can prevent these complications. 13 children were operated on altogether 15 forearms. In nine cases lengthening of the ulna was necessary to correct ulnar instability of the wrist as well as improving the support of the carpus and preventing dislocation of the radial head.

Radial growth and function of the forearm after excision of the radial head. A study of growing macaque monkeys.

In five immature macaque monkeys, the right radial head was excised and the left forearm served as a control. Growth of each radius, ulna, and proximal and distal physis was followed for eight years. Total radial growth was slightly less on the side that was operated on than it was on the control side. After excision of the radial head, 95 per cent of radial growth came from the distal physis, compared with 71 per cent on the control side. A regenerated radial head, which had an irregular surface of fibrocartilaginous tissue, accounted for the remainder of growth on the side that had been operated on. Incongruous contact of articular surfaces resulted in degenerative changes in the capitellum. The arms that had been operated on were left with a flexion contracture that was an average of 12 degrees greater than that of the normal elbows, and they had a carrying angle that was an average of 6 degrees greater than that on the normal side. The arc of pronation and supination was decreased for the forearms that had been operated on, but motion of the wrist was unaffected. In our study, excision of the radial head in growing monkeys resulted in minimum radial shortening, slight deformity of the elbow, and moderate impairment of rotation of the forearm.

[Disturbed growth in height in multiple cartilaginous exostoses (author's transl)]. / Zum Problem der Längenwachstumsstörung bei multiplen cartilaginären Exostosen.

Basing on the hypothesis that reduced body height in patients with multiple cartilaginous exostoses would be mainly accounted for by shorter extremities, not by a shorter trunk, the authors clinically examined 19 exostosis patients in respect of thigh, lower leg, upper and lower arm, as well as height of the seated patient. The dimensions were compared by the method of matched pairs, with 19 volunteers without diseased skeleton, who corresponded with one of the exostosis patients in respect of age, sex and height of seated patient. Results were evaluated according to Wilcoxon's test. This showed a statistically significant reduction in length of the extremities in adults with multiple cartilaginous exostoses.