Pattern of Syringomyelia in Presumed Idiopathic and Congenital Scoliosis.

STUDY DESIGN: Retrospective study. PURPOSE: This study was undertaken to compare the patterns of syringomyelia in patients with presumed idiopathic and congenital scoliosis. OVERVIEW OF LITERATURE: The incidence of neuraxial anomalies presenting as idiopathic scoliosis ranges from 2% to 14%; the common ones are idiopathic syringomyelia (IS) and Chiari malformation type 1 (CM1) with syringomyelia. Some authors have speculated that scoliosis is caused by the asymmetrical compression of anterior horn cells by a syrinx, which causes an imbalance of the trunk musculature. In congenital scoliosis, syringomyelia is the second commonest cord anomaly, and the deformity progression depends upon the underlying vertebral abnormality, the location of the abnormality, and the age of patient, and is independent of the intraspinal anomaly. METHODS: We analyzed the radiological records of 44 consecutive patients with scoliosis and syringomyelia. Of these 44 patients, 13 had IS, 12 had CM1, and 19 had congenital scoliosis. The radiographs were evaluated to determine the curve magnitude, sagittal alignment, side of convexity, and type of vertebral anomaly, if any. T1- and T2-weighted magnetic resonance imaging of the whole spine was analyzed to determine the presence of craniovertebral anomalies, syrinx length, syrinx diameter, and syrinx-cord ratio (SCR). RESULTS: The frequency of left convex curves was 26.1%, with no significant differences across the three groups. The mean length of the syrinx was 7.2±4.9 vertebral levels, and the mean SCR was 0.39±0.2. The mean syrinx length was significantly higher in patients with CM1 and IS, compared to patients with congenital scoliosis. The mean SCR was highest in patients with CM1. In congenital scoliosis, syringomyelia was seen most frequently in patients with a failure of formation, and 63.2% had concomitant cord anomalies. CONCLUSIONS: Syrinxes were smaller in size and length in patients with congenital scoliosis, and are distinct from those seen in IS and CM1.

Congenital pseudoarthrosis of the radius in Neurofibromatosis Type 1: An entity not to be missed!

Congenital pseudoarthrosis of the radius is exceedingly rare. We report an isolated pseudoarthrosis of the radius in a five-year-old girl child with Neurofibromatosis type-I (NF-I). She underwent excision of the pseudoarthrosis tissue, ulna osteotomy, iliac crest cortico-cancellous bone grafting, and K wire fixation of both bones. A sound union of the radius and the ulna was achieved after 4 months. At two-year follow-up, mild shortening of the forearm persisted with maintenance of sound union of both bones. Accurate diagnosis of the condition is central in avoiding complications and the need for complex surgical procedures.

Vertebral, intraspinal and other organ anomalies in congenital scoliosis.

AIMS: This study was undertaken to describe the pattern of vertebral, intraspinal and other organ anomalies in patients with congenital scoliosis and to determine the correlation between them. METHODS: Complete medical and radiological records of 227 consecutive patients with congenital scoliosis were analysed. The radiographs were examined for type of vertebral anomaly, location and severity of deformity. The median curve progression index (MCPI) was calculated in 198 patients. The magnetic resonance imaging (MRI) of the whole spine was analysed to detect the presence of cord abnormalities. The presence of other organ-system anomalies was also noted. The independent sample t test was used to compare severity of deformity between those with and without cord anomalies. The Chi-square test was used to compare frequency of cord abnormalities in different vertebral and organ-system anomalies. RESULTS: Hemivertebra with contralateral bar had the highest MCPI, while block vertebrae and wedge vertebrae had the lowest MCPI. Forty-eight patients had 83 cord anomalies. There was no statistically significant difference in severity of deformity, between those with and without cord anomalies. Failure of segmentation had the highest frequency of cord anomalies (p = 0.01). There was no significant difference in the frequency of cord anomalies between those with and without other organ defects. CONCLUSION: Curve progression can be predicted by the underlying vertebral abnormalities. However, it cannot predict cord and other organ-system anomalies. Thus, all patients with congenital scoliosis must undergo MRI of the spine, electro- and echocardiography and ultrasonography of the abdomen to detect occult abnormalities and optimize the patient prior to deformity correction.

Hemiepiphysiodesis using 2-holed reconstruction plate for correction of angular deformity of the knee in children.

BACKGROUND: This study assesses the outcome of hemi epiphysiodesis with a 2-holed reconstruction plate using the tension band principle in angular deformities of the knee and variables influencing the outcome. METHODS: Prospective study from 2012 to 2016 included 23 children with coronal deformities of the knee joint. IMD, ICD, BMI, mLDFA, mMPTA, mTFA, Mechanical axis deviation, Perpendicular mechanical axis ratio, and screw angle measured. Correction and rate of correction compared with BMI, age, etiology, PMAR and MAD, deformity, a segment of bone and the screw angle. RESULTS: Total 65 segments operated in 23 children with 40 knees. Out of the total 65, 36 plates were kept in distal femur physis and 29 in the proximal tibial physis. Thirteen knees had genu varum and 27 had genu valgum. The mean age at the time of surgery was 11.25 months. The mean follow-up of 36 months and the average time for correction was 18.64 months. 77.5% correction achieved with ROC maximum in the initial 6 months. ROC in TFA per year were 7.80(femur), 50(tibia), and 120(femur with tibia). Complications included broken implant, screw back out, screw irritation and rebound phenomenon. CONCLUSIONS: 2-holed reconstruction plate gives similar correction to that of the 8-plate within a comparable period without additional complications. It has the advantage of being cost effective and readily available. Age, BMI, type of deformity, amount of initial deformity, do not play a significant role in the outcome of correction. Screw placement may have a role in the final correction of the deformity. LEVEL OF EVIDENCE: Level 3.

A rare case of rubber band syndrome of wrist with distal radius and ulna fracture.

Rubber band syndrome (Dhaga Syndrome) is a very rare condition seen mostly among children in whom elastic bands worn around the wrist or forearm for religious or decorative purpose and its presence is overlooked by the parents. These forgotten bands slowly cuts through the skin and soft tissue underneath causing constriction and damage to the underlying structures. Circumferential constricting scar with discharging sinus is the cardinal feature. We report a case of rubber band syndrome in a 2 year old boy in which the constriction was severe enough to cause fracture of the distal radius and ulna.

Bilateral Bifid Median Nerve with Bilateral Carpal Tunnel Syndrome: An Atypical Presentation, a Rare Cause and a Familiar Disease.

We report a case of bilateral carpal tunnel syndrome (CTS) with bilateral bifid median nerve (BMN) without a persistent median artery diagnosed by clinical examination, NCV and Ultrasonography. Bilateral BMN to produce bilateral CTS is extremely rare and is contrary to the popular classifications which state that BMN without persistent median artery or its anomalies will not produce CTS. Ultrasonogram is of additional help to identify such aberrations in the median nerve to prevent iatrogenic injuries during carpal tunnel decompression.

Massive Embryonal Rhabdomyosarcoma of the Hand in an Infant With Metastasis at Birth: Management Dilemma.

BACKGROUND: Rhabdomyosarcomas are malignant tumors arising from striated muscle but can be often confused with primitive neuroectodermal tumors and Ewing sarcoma. They are often classified based on age of presentation and histological features. Three major types of rhabdomyosarcomas are embryonal, alveolar, and pleomorphic with characteristic presentations. METHOD: Here, we present a case of embryonal rhabdomyosarcoma in the hand of a 5-month-old child with lymphatic metastasis, the age, site, and metastasis being unusual feature for this type of rhabdomyosarcoma. RESULT: The child succumbed to the complications soon after initiation of chemotherapy. CONCLUSION: Age, site, metastasis at birth and treatment related complications contribute to the poor outcome.

Birth brachial plexus palsy: a race against time.

A 5-year-old child presented to us with weakness of the left upper limb since birth. With the given history of obstetric trauma and limb examination, a diagnosis of birth brachial plexus palsy was made. Brachial plexus exploration along with microsurgery was performed at the same time which included extrinsic neurolysis of the roots and trunks and nerve transfer for better shoulder external rotation and elbow flexion. Both the movements were severely restricted previously due to co-contractures with the shoulder internal rotators and triceps. The problem of birth brachial plexus palsy is proving to be a global health burden both in developed countries and in developing countries such as India. The lack of awareness among the general public and primary healthcare providers and inadequate orthopaedic and neurosurgeons trained to treat the condition have worsened the prognosis. This case lays stress on the delayed complications in birth brachial palsy and its effective management.