Eight-plate epiphysiodesis: are we creating an intra-articular deformity?

Aims: Guided growth using eight-plates is commonly used for correction of angular limb deformities in growing children. The principle is of tethering at the physeal periphery while enabling growth in the rest of the physis. The method is also applied for epiphysiodesis to correct limb-length discrepancy (LLD). Concerns have been raised regarding the potential of this method to create an epiphyseal deformity. However, this has not been investigated. The purpose of this study was to detect and quantify the occurrence of deformities in the proximal tibial epiphysis following treatment with eight-plates. Patients and Methods: A retrospective study was performed including 42 children at a mean age of 10.8 years (3.7 to 15.7) undergoing eight-plate insertion in the proximal tibia for correction of coronal plane deformities or LLD between 2007 and 2015. A total of 64 plates were inserted; 48 plates (34 patients) were inserted to correct angular deformities and 16 plates (8 patients) for LLD. Medical records, Picture Archive and Communication System images, and conventional radiographs were reviewed. Measurements included interscrew angle, lateral and medial plateau slope angles measured between the plateau surface and the line between the ends of the physis, and tibial plateau roof angle defined as 180° minus the sum of both plateau angles. Measurements were compared between radiographs performed adjacent to surgery and those at latest follow-up, and between operated and non-operated plateaus. Statistical analysis was performed using BMDP Statistical Software. Results: Slope angle increased in 31 (49.2%) of operated epiphyses by a mean of 5° (1° to 23°) compared with 29 (31.9%) in non-operated epiphyses (p = 0.043). Roof angle decreased in 29 (46.0%) of operated tibias and in 25 (27.5%) of non-operated ones by a mean of 5° (1° to 18°) (p = 0.028). Slope angle change frequency was similar in patients with LLD, varus and valgus correction (p = 0.37) but roof angle changes were slightly more frequent in LLD (p = 0.059) and correlated with the change in inter screw angles (r = 0.74, p = 0.001). Conclusion: The use of eight-plates in the proximal tibia for deformity correction and limb-length equalization causes a change in the bony morphology of the tibial plateau in a significant number of patients and the effect is more pronounced in the correction of LLD. Cite this article: Bone Joint J 2018;100-B:1112-16.

Treatment of Achilles tendon calcinosis in juvenile dermatomyositis with external ilizarov fixator.

Calcinosis is a devastating complication of juvenile dermatomyositis and a challenging therapeutic problem. We report the use of an external Ilizarov fixator for the treatment of Achilles tendon calcinosis causing severe disability in a young girl with juvenile dermatomyositis.

Prenatal ultrasound diagnosis of club foot: outcome and recommendations for counselling and follow-up.

Club foot was diagnosed by ultrasonography in 91 feet (52 fetuses) at a mean gestational age of 22.1 weeks (14 to 35.6). Outcome was obtained by chart review in 26 women or telephone interview in 26. Feet were classified as normal, positional deformity, isolated club foot or complex club foot. At initial diagnosis, 69 feet (40 fetuses) were classified as isolated club foot and 22 feet (12 fetuses) as complex club foot. The diagnosis was changed after follow-up ultrasound scan in 13 fetuses (25%), and the final ultrasound diagnosis was normal in one fetus, isolated club foot in 31 fetuses, and complex club foot in 20 fetuses. At birth, club foot was found in 79 feet in 43 infants for a positive predictive value of 83%. Accuracy of the specific diagnosis of isolated club foot or complex club foot was lower; 63% at the initial ultrasound scan and 73% at the final scan. The difference in diagnostic accuracy between isolated and complex club foot was not statistically significant. In no case was postnatal complex club foot undiagnosed on fetal ultrasound and all inaccuracies were overdiagnoses. Karyotyping was performed in 25 cases. Abnormalities were noted in three fetuses, all with complex club foot and with additional findings on ultrasound.

Immobilisation of forearm fractures in children: extended versus flexed elbow.

Redisplacement of unstable forearm fractures in plaster is common and may be the result of a number of factors. Little attention has been paid to the influence of immobilisation with the elbow extended versus flexed. We prospectively treated 111 consecutive children from two centres with closed forearm fractures by closed reduction and casting with the elbow either extended (60) in China or flexed (51) in Israel. We compared the outcome of the two groups. There was no statistically significant difference in the distribution of the age of the patients, the site of fracture or the amount of angulation and displacement between the groups. During the first two weeks after reduction, redisplacement occurred in no child immobilised with the elbow extended and nine of 51 children (17.6%) immobilised with the elbow flexed. Immobilisation of unstable forearm fractures with the elbow extended appears to be a safe and effective method of maintaining reduction.

Computerized analysis of sleep recordings applied to drug evaluation: midazolam in normal subjects.

Computerized analysis of polygraphic sleep recordings was carried out for the evaluation of midazolam, a benzodiazepine hypnotic. The analysis was carried out in real time on a small laboratory computer, and the output included the hypnogram and relative power profiles for the main electroencephalogram activities. Analysis showed a slight "intranight rapid eye movement rebound" during medication and reduction of sleep stage IV after withdrawal. The relative power of the delta frequency band did not change during medication or withdrawal.

Tuboovarian autoamputation and infertility.

Asymptomatic or undiagnosed tuboovarian autoamputation in teenagers may have its impact on fertility later in life. Two cases are presented with infertility following unilateral adnexal autoamputation. The importance of awareness for possible subtorsion or torsion of adnexa in childhood and adolescence and its impact on fertility are discussed.

Testicular fine needle aspiration as a diagnostic method.

We believe testicular FNA is a reliable, simple, and accurate method for the assessment of testicular pathology and can replace the biopsy. Only when insufficient conclusions may be drawn from the cytologic smears obtained is open biopsy indicated.

The relationship between plasma levels of gonadotropins, androgens, and prolactin in azoospermic men with their testicular spermatogenic pattern.

OBJECTIVE: To assess the relationship between plasma levels of gonadotropins, androgens, and PRL with testicular spermatogenic pattern. DESIGN: Patient series. SETTING: University affiliated medical center. PATIENTS: One hundred twenty azoospermic infertile men. INTERVENTIONS: Testicular fine needle aspirations and determination of plasma levels of FSH, LH, T, free T, and PRL. MAIN OUTCOME MEASURES: Gonadotropins, androgens, and PRL plasma levels as a diagnostic criterion of testicular spermatogenic patterns. RESULTS: No statistically significant differences were detected in plasma levels of LH, androgens, and PRL among patients with Sertoli cell only, spermatogenic arrest, or full spermatogenesis. Elevated plasma levels of FSH threefold above the upper normal limit preclude, with a probability of 95%, the existence of full testicular spermatogenesis, but are not valid for the diagnosis of either Sertoli cell only syndrome or spermatogenic arrest. CONCLUSIONS: Luteinizing hormone, androgens, and PRL plasma levels are of no diagnostic value in predicting any specific spermatogenic pattern, and plasma FSH levels can not be used for diagnosing Sertoli cell only syndrome.

Orthopaedic manifestations of familial dysautonomia. A review of one hundred and thirty-six patients.

BACKGROUND: Familial dysautonomia is a hereditary multisystemic disease primarily affecting people of Ashkenazi Jewish descent. Musculoskeletal problems are related to gait disorders, spinal deformities, foot deformities, fractures, and arthropathies. METHODS: The charts and radiographs of 136 patients who ranged in age from three months to forty-six years (mean, sixteen years) were reviewed. Sixty-four patients were available for follow-up examination. RESULTS: Spinal deformity was the most common orthopaedic problem and was diagnosed in seventy-eight patients starting at the age of four years, with a prevalence of 86 percent (forty-eight of fifty-six) by the age of fifteen years. Forty-one (53 percent) of the seventy-eight patients had scoliosis only, thirty-four (44 percent) had kyphoscoliosis, and three (4 percent) had kyphosis only. Bracing was accompanied by emotional, pulmonary, and skin problems, leading to a high rate of noncompliance and progression of the curve. Twenty-four patients had an operation at a mean age of thirteen years (range, five to eighteen years): twenty patients had posterior spinal arthrodesis, and four had combined anterior and posterior arthrodesis. Fifteen patients had a total of nineteen complications, of which seven were systemic and twelve were related to the spinal fixation. Eight patients had revision surgery. At the time of the surgery, scoliosis was corrected from a mean of 55 degrees to a mean of 35 degrees and kyphosis was corrected from a mean of 69 degrees to a mean of 61 degrees. After a mean duration of follow-up of sixty-five months, scoliosis measured 49 degrees (range, 18 to 62 degrees) and kyphosis measured 67 degrees (range, 30 to 115 degrees). Postoperative progression of the deformity was caused by failure of the instrumentation or progression in unfused segments. Walking was delayed in 72 percent (ninety-four) of the 130 patients who were of walking age. All sixty-four of the patients who were examined had an ataxic gait. Foot deformities were found in sixteen patients, six of whom were treated surgically. Two patients had Charcot joints. Fifty-five patients sustained at least one fracture before skeletal maturity, with a mean of 1.5 fractures per patient. All but one of the fractures was treated nonoperatively, and fracture-healing was often accompanied by profuse callus formation. CONCLUSIONS: Spinal deformity is common in patients with familial dysautonomia. Bracing is of questionable benefit, and surgical intervention should be considered once curve progression is well documented. Arthrodesis should be extended as far proximally as possible to prevent junctional kyphosis. Swelling and warmth in a limb should raise suspicion of an undiagnosed fracture.

External fixation or flexible intramedullary nailing for femoral shaft fractures in children. A prospective, randomised study.

We report the outcome of 19 children aged 5.2 to 13.2 years with 20 fractures of the femoral shaft requiring surgery, who were randomly assigned to have external fixation (EF) or flexible intramedullary nailing (FIN) (10 fractures each). The duration of the operation averaged 56 minutes for the EF group with 1.4 minutes of fluoroscopy, compared with 74 minutes and 2.6 minutes, respectively, for the FIN group. The early postoperative course was similar, but the FIN [corrected] group showed much more callus formation. The time to full weight-bearing, full range of movement and return to school were all shorter in the FIN group. The FIN complications included one transitory foot drop and two cases of bursitis at an insertion site. In the EF group there was one refracture, one rotatory malunion requiring remanipulation and two pin-track infections. At an average follow-up of 14 months two patients in the EF group had mild pain, four had quadriceps wasting, one had leg-length discrepancy of over 1 cm, four had malalignment of over 5 degrees, and one had limited hip rotation. In the FIN group, one patient had mild pain and one had quadriceps wasting; there were no length discrepancies, malalignment or limitation of movement. Parents of the FIN group were more satisfied. We recommend the use of flexible intramedullary nailing for fractures of the femoral shaft which require surgery, and reserve external fixation for open or severely comminuted fractures.

Monitoring of the sciatic nerve during hamstring lengthening by evoked EMG.

Traction injury to the sciatic nerve can occur during hamstring lengthening. The aim of this study was to monitor the influence of hamstring lengthening on conduction in the sciatic nerve using evoked electromyography (EMG). Ten children with spastic cerebral palsy underwent bilateral distal hamstring lengthening. Before lengthening, the evoked potential was recorded with the patient prone. During lengthening, it was recorded with the knee flexed to 90 degrees, 60 degrees and 30 degrees, and at the end of lengthening with the hip and knee extended. In all patients, the amplitude of the evoked EMG gradually decreased with increasing lengthening. The mean decrease with the knee flexed to 60 degrees was 34% (10 to 77), and to 30 degrees, 86% (52 to 98) compared with the pre-lengthening amplitude. On hip extension at the end of the lengthening procedure, the EMG returned to the pre-lengthening level. Monitoring of the evoked EMG potential of the sciatic nerve during and after hamstring lengthening, may be helpful in preventing traction injury.

Ultrasonography of the hip in developmental hip dysplasia.

Ultrasonography of the hip was performed sequentially by two different examiners in 75 infants. The ultrasound strips were reviewed twice by three paediatric orthopaedic surgeons and classified by the Graf method. The intraobserver and interobserver agreement between the interpretations was analysed using simple and weighted kappa coefficients calculated for agreement on the Graf classification and for grouping as normal (types 1A to 2A), and abnormal requiring treatment (types 2B to 4). When examining the same ultrasound strip, intraobserver agreement for the Graf classification was substantial (mean kappa 0.61), but interobserver agreement was only moderate (kappa 0.50). For the grouping into normal and abnormal, the mean kappa value for intraobserver agreement was 0.67 and for interobserver agreement 0.57. Because of the significant differences in agreement between normal and abnormal hips, we analysed a subgroup of those with at least one abnormal interpretation. Intraobserver agreement within this subgroup showed moderate reliability (kappa 0.41), but interobserver agreement was only fair (kappa 0.28). Interpretations of two different strips performed sequentially showed significantly lower agreement with an intraobserver kappa value of 0.29 and an interobserver value of 0.28. In the subgroup with at least one abnormal reading, the intraobserver kappa was 0.09 and the interobserver 0.1. Our findings suggest that both the technique of performing ultrasonography and the interpretation of the image may influence the result.

Congenital insensitivity to pain. Orthopaedic manifestations.

We reviewed 13 patients with congenital insensitivity to pain. A quantitative sweat test was carried out in five and an intradermal histamine test in ten. DNA examination showed specific mutations in four patients. There were three clinical presentations: type A, in which multiple infections occurred (five patients); type B, with fractures, growth disturbances and avascular necrosis (three patients); and type C, with Charcot arthropathies and joint dislocations, as well as fractures and infections (five patients, four with mental retardation). Patient education, shoeware and periods of non-weight-bearing are important in the prevention and early treatment of decubitus ulcers. The differentiation between fractures and infections should be based on aspiration and cultures to prevent unnecessary surgery. Established infections should be treated by wide surgical debridement. Deformities can be managed by corrective osteotomies, and shortening by shoe raises or epiphysiodesis. Joint dislocations are best treated conservatively.

Automatic classification of visual evoked responses.

An automatic method performing selective averaging of visual evoked potentials depending on the state of the EEG background activity is described. The method is based on adaptive segmentation of the EEG signal and on fuzzy clustering. A simulation example, extracting two different square pulses from the background activity, is given, as well as an example averaging two types of visual evoked potentials from the background EEG signal.

Early innominate osteotomy as a treatment for avascular necrosis complicating developmental hip dysplasia.

The clinical and radiographic outcomes of 25 patients with avascular necrosis (AVN) of the femoral head complicating developmental hip dysplasia (DDH) were analyzed. Seven patients (group A) had an innominate osteotomy 1-3 years after the ischemic insult. Eight patients (group B) had a pelvic osteotomy between 5 and 10 years after the insult, and 10 patients (group C) did not have a pelvic osteotomy. The minimum follow-up period was 10 years from the time of the ischemic insult. The hips in group A patients (early osteotomy) showed significantly better radiographic outcomes as assessed by a modified Severin grading. These patients also had less pain, fewer gait disturbances, and required fewer additional procedures for limb length discrepancy or greater trochanteric overgrowth.

Eosinophilic granuloma of the spine.

Twenty patients treated for eosinophilic granuloma of the spine were studied. Only 40% demonstrated the classical radiographic picture of vertebra plana. In 60% a lytic lesion of the vertebral body or the posterior elements was found. Seven patients underwent surgery; the indications were neurological involvement or failure of the biopsy to disclose the diagnosis. At an average follow-up period of 7 years, 17 patients are well and alive with no residual spinal pain, neurological compromise, recurrent disease, or extraskeletal involvement. Vertebral body collapse underwent some regeneration but did not regain full body height. In several patients this resulted in a local deformity. In patients with unifocal spinal eosinophilic granuloma, watchful observation with no treatment other than spinal support is warranted. In patients with neural involvement or multifocal lesions, a more active treatment, including surgery, may be indicated.

Traumatic myositis ossificans of the quadriceps in infants.

Acute traumatic myositis ossifications is uncommon and usually occurs in adolescents and young adults after a significant direct blow to the affected muscle. It is extremely rare in infants, and we have been able to find only two other cases in the English literature. We present two cases of traumatic myositis ossificans in infants who, except for the use of diagnostic ultrasonography, would have been misdiagnosed as having osteomyelitis (Patient 1) and a possible malignancy (Patient 2).

[Epididymal fine needle aspiration replacing vasography].

Epididymal fine needle aspirations (EFNA) were performed on 29 epididymides of 17 infertile, azoospermic men in whom testicular cytology revealed adequate spermatogenesis. Sonograms confirmed the presence of all the excretory male organs. EFNA was performed to diagnose and locate the site of genital tract occlusion. The presence of cuboidal epithelial cells in the aspirate was the sole indication that epididymal content was aspirated. In 10 (34%) of the 29 aspirates there were only cuboidal epithelial cells and no spermatozoa, indicating proximal occlusion in the rete testis, in the canaliculi efferents or in the most proximal segment of the epididymis itself. Occlusion in the distal segments of the epididymis or in the vas deferens was diagnosed in 13 epididymal aspirates (45%) which contained both cuboidal cells and spermatozoa. In 6 epididymides (21%) no conclusion could be reached because of failed aspirations. There are a variety of treatment modalities available for male genital tract obstruction, such as reconstructive surgery or IVF. The success rate of treatment is mainly dependent on the site of obstruction. Since vasography has its own limitations and hazards, diagnostic EFNA should be performed before treatment in men in whom genital obstruction is suspected.

[Relationship between spermatogenic maturation stages and male hormone levels].

The relationship between stages of the spermatogenic maturation process and male hormone levels was evaluated in 41 azoospermic, infertile men. Patients were categorized into groups according to the most mature spermatogenic cell type present in testicular aspirates: spermatocytes, spermatids or spermatozoa. High FSH and LH plasma levels were found in those with spermatocytes. Their hormone levels differed statistically (p < 0.005) from those in patients with maturation arrest at the spermatid stage, or those with spermatozoa in their testes. No statistically significant differences were found between the 3 groups with regard to plasma levels of testosterone, free testosterone and prolactin. However, there were positive correlations, higher for free testosterone than for testosterone, with stage of spermatogenic maturation.

[Are testes in oligo/azoospermia homogenous or heterogenous?].

We determined whether a single testicular specimen is sufficient to represent qualitatively the spermatogenic process within the testes of azoospermic or severely oligospermic infertile men. In 191 testes of azoospermic patients and in 26 of those with severe oligospermia, fine needle aspirations at 3 different sites of each testis were performed. Aspirated material from each puncture was stained and in each smear all spermatogenic cells, as well as Sertoli cells, were identified. Testes were classified according to the most mature spermatogenic cell type present, or the presence of only Sertoli cells. The homogeneity of the testicular spermatogenic process was then evaluated. There was an overall intratesticular difference between aspirates in 14.1% of azoospermic testes and in 26.9% of severely oligospermic testes with regard to the most mature spermatogenic cell type. When spermatozoa were the most mature cell type, they were detected in all of the 3 aspirates in 71.4% of the testes. In 18.4% or 10.2% of this group of testes they were retrieved in only 1 or 2 of the aspirates, respectively. In testes in which spermatids or spermatocytes were the most mature spermatogenic stage, these cell types were detected in all 3 aspirates in only 36.4% and 68.0%, respectively. In azoospermic patients with full testicular spermatogenesis, the likelihood of retrieving spermatozoa from the testes was 84.3%, 92.7% and 100% in 1, 2 and 3 specimens, respectively. The following conclusions were drawn: There is a wide range of testicular heterogeneity in azoospermia or very severe oligospermia for diagnosing the testicular spermatogenic pattern. In azoospermia, specimens from several testicular sites are required. It is strongly recommended that no assisted fertilization be offered to azoospermic patients unless prior evaluation of the spermatogenic pattern in the seminiferous tubules is determined.