Vrisha Madhuri

Lipofibromatosis: report of a rare paediatric soft tissue tumour

The clinical, radiological and pathological features of a case of lipofibromatosis, a rare paediatric soft tissue neoplasm, are described. The tumour involved the foot of a male infant and was present at birth. Magnetic resonance imaging showed a lipomatous mass, with splaying of muscles of the sole by lobules of fat. Histopathological examination revealed typical findings of an admixture of mature adipose tissue and fibroblastic elements. The radiological and pathological features helpful in differentiating this entity from other fibro-fatty paediatric soft tissue tumours is discussed, and the relevant literature is briefly reviewed.

Schwannoma of the fibula

We describe a schwannoma located in the mid-diaphyseal region of the fibula of a 14-year-old boy. Radiologically this was an expansile, lytic, globular and trabeculated lesion. MRI showed a narrow transition zone with a break in the cortex and adjacent tissue oedema. Differential diagnosis included schwannoma, fibrous dysplasia, giant cell tumour and aneurysmal bone cyst. The tumour was excised en bloc, with marginal resection limits, and there has been no recurrence two years after surgery. Histopathological examination confirmed the diagnosis of classic schwannoma. There were typical hypercellular Antoni A zones, less cellular Antoni B zones, and diffuse immunoreactivity to S100 protein. This is the first report of schwannoma involving a long bone in a child.

Correction of post burns contracture of wrist with Ilizarov method

A case of severe post burns contracture of the wrist, of 43 years duration resulting in recurrent ulceration of the skin was successfully treated by gradual distraction using the Ilizarov method. This method is superior to plaster of Paris cast and is more versatile than other forms of external fixation.

A descriptive analysis of 14 cases of progressive-psuedorheumatoid-arthropathy of childhood from south India: Review of literature in comparison with Juvenile Idiopathic Arthritis

BACKGROUND Progressive-psuedorheumatoid-arthropathy of childhood (PPAC) is an autosomal recessive single gene skeletal dysplasia involving joints. The gene attributed to its cause is WNT1-inducible-signaling pathway protein3 (WISP3). OBJECTIVE To study the clinical and radiographic presentation of PPAC in Indian patients and to compare with described features of PPAC and Juvenile Idiopathic Arthritis (JIA) from published literature. METHODS All cases (n = 14) of PPAC seen in the Rheumatology and Clinical Genetics outpatient clinic between 2008 and 2011 with classical, clinical, and radiological features were studied. The demographic and clinical data were obtained from medical records of the outpatient visits. RESULTS Slight female preponderance (57%) and history of consanguinity in parents (43%) was observed in this group. The median age at onset was 4.5 years (range from birth to 9 years of age). Early presentation below the age of 3 years was seen in 3/14 patients (21%) in this group. The growth of all the patients fell below the 3rd percentile for the age. Historically, hip joint involvement was the most common presenting feature; however, elbow, wrist, knees, feet, spine, shoulder joints and small joints, namely proximal interphalangeal (PIP), distal interphalangeal (DIP), metacarpophalangeal (MCP), metatarsophalangeal joints (MTP), and interphalangeal joints (IP) of the feet, were also involved, either clinically or radiologically in varying proportions. Platyspondyly was noted in all. Molecular analysis of the WISP3 gene identified mutations in all the 5 individuals in whom it was done. CONCLUSION This descriptive case series of PPAC from India reports distinctly differentiating clinical, radiological, and molecular markers in contrast with classically described features of JIA, its mimic. Early presentation (age of onset below 3 years) with involvement of interphalangeal joints seen in three patients (21%) was a unique finding, with missense WISP3 gene mutations in all of them. Timely diagnosis of this entity can spare the patient from unnecessary investigations and toxic medications.

Slipped upper femoral epiphysis: Outcome after in situ fixation and capital realignment technique

Background: Slipped upper femoral epiphysis (SUFE) is the gradually acquired malalignment of the upper femoral epiphysis (capital) and the proximal femoral metaphysis. SUFE is uncommon in India, and there are no previous studies on outcome and clinical characteristics of patients with SUFE from India. This study evaluates the presentation, disease associations and outcome of SUFE from a tertiary care centre in India. Materials and Methods: Twenty six consecutive children with SUFE seen over a period of 4 years were reviewed. The clinical presentations, severity of the slip, surgical interventions (n=30) were assessed. Twenty one boys and five girls with a mean age 13.1 years (range 10-16 years) were included in the study. Four children had bilateral involvement. There were 4 rural and 22 urban children from the eastern and southern states of the country. The presentation was acute in 7, acute on chronic in 5, and chronic in 14, with a mean duration of symptoms of 51 days (range 3-120 days). Slips were stable in 16 and unstable in 10 children. Two children had adiposogenital syndrome. Body mass index was high in 12 out of 23 children. Vitamin D levels were low in 20 out of 21 children, with a mean vitamin D level of 12.61 ± 5 ng/ml. Eighteen children underwent in situ pinning. Eight children underwent capital realignment. Results: Clinical outcome as assessed by Merle d’ Aubigne score was excellent in 6, good in 10, fair in 6 and poor in 1. Half of the in situ fixation patients underwent osteoplasty procedure for femoroacetabular impingement and 5 more were symptomatic. The head neck offset and α angle after in situ pinning were −1.12 ± 3 mm and 66.05 ± 9.7°, respectively and this improved to 8.7 mm and 49°, respectively, after osteoplasty. One child in the pinning group had chondrolysis. Eight patients with severe slip underwent capital realignment. Mean followup was 20.15 months. The anterior head neck offset and α angle were corrected to 6.8 ± 1.72 mm and 44.6 ± 7.0° mm, respectively. Two children with unstable slip in the capital realignment group had avascular necrosis which was diagnosed at presentation by bone scan. Conclusion: High BMI, vitamin D deficiency and endocrine disorders are associated with SUFE in India and should be evaluated as some of these are amenable to prevention and treatment. Most patients treated with in situ pinning developed femoroacetabular impingement. The early results after capital realignment procedure are encouraging and help to avoid a second procedure which is needed in a majority of patients who underwent in situ pinning.

South Indian calcaneal talar facet configurations and osteoarthritic changes

The purpose of this study was to determine the talar facet configuration of South Indian calcanei, measure the angle between the anterior and middle facet planes of these calcanei, and assess the relation between the above parameters and the degenerative changes in the subtalar joints. The angle between the anterior and middle talar facets was measured in 222 South Indian adult calcanei. The degree of sclerosis was measured on radiographs of the calcanei. Lipping and osteophytes around the joints were recorded by visual inspection. The facet patterns observed were fused anterior and middle facets (Type I), three separate facets (Type II), absence of the anterior facet (Type III), three merged facets (Type IV), and a new pattern of absent anterior and fused middle and posterior facets (Type V). An anterolateral impression was present in nine calcanei. Type I was the predominant pattern (72%). Type II configuration had the least mean angle (125°) and had less number of calcanei with significant osteoarthritic changes. A wider angle was observed in Type I and Type III calcanei. Type IV and Type V were observed in only three and one calcanei, respectively. Lipping and osteophytes were observed in Type I to IV configurations. There was no correlation between the facet configuration and the radiological subchondral sclerosis in the posterior talar facet of the calcanei. This study reveals that the talar facet configuration of calcanei and the angle between the anterior and middle facets influence the stability of the subtalar joints and development of osteoarthritis. Clin. Anat. 21:581–586, 2008.

Slipped capital femoral epiphysis associated with vitamin D deficiency: A series of 15 cases

Slipped capital femoral epiphysis (SCFE) is uncommon in India and we routinely look for associated metabolic or endocrine abnormalities. In this study we investigated a possible association between vitamin D deficiency and SCFE. All children presenting with SCFE during the study period had their 25-hydroxyvitamin D levels measured as part of an overall metabolic, renal and endocrine status evaluation, which included measurement of body mass index (BMI). Vitamin D status was compared with age-, gender- and habitat-matched controls with acute trauma or sepsis presenting to our emergency department. A total of 15 children (12 boys and three girls) with a mean age of 13 years (sd 1.81; 10 to 16) presented for treatment for SCFE during a two-year period beginning in January 2010. Renal and thyroid function was within the normal range in all cases. The mean BMI was 24.9 kg/m(2) (17.0 to 33.8), which was significantly higher than that of the controls (p = 0.006). There was a statistically significant difference between the mean values of 25-hydroxyvitamin D in the children with SCFE and the controls (11.78 ng/ml (SD 5.4) versus 27.06 ng/ml (SD 5.53), respectively; p < 0.001). We concluded that, along with high BMI, there is a significant association of vitamin D deficiency and SCFE in India.

A retrospective analysis of loss of reduction in operated supracondylar humerus fractures

Background: Loss of reduction following closed or open reduction of displaced supracondylar fractures of the humerus in children varies widely and is considered dependent on stability of the fracture pattern, Gartland type, number and configuration of pins for fixation, technical errors, adequacy of initial reduction, and timing of the surgery. This study was aimed to evaluate the factors responsible for failure of reduction in operated pediatric supracondylar fracture humerus. Materials and Methods: We retrospectively assessed loss of reduction by evaluating changes in Baumanns angle, change in lateral rotation percentage, and anterior humeral line in 77 consecutive children who were treated with multiple Kirschner wire fixation and were available for followup. The intraoperative radiographs were compared with those taken immediately after surgery and 3 weeks postoperatively. Multivariate logistic regression analysis was performed by STATA 10. Results: Reduction was lost in 18.2% of the patients. Technical errors were significantly higher in those who lost reduction (P = 0.001; Odds Ratio: 57.63). Lateral pins had a significantly higher risk of losing reduction than cross pins (P = 0.029; Odds Ratio: 7.73). Other factors including stability of fracture configuration were not significantly different in the two groups. Conclusions: The stability of fracture fixation in supracondylar fractures in children is dependent on a technically good pinning. Cross pinning provides a more stable fixation than lateral entry pins. Fracture pattern and accuracy of reduction were not important factors in determining the stability of fixation.

Florid reactive periostitis of the forearm bones in a child

Florid reactive periostitis is a pronounced periosteal reaction, usually affecting the hands and feet, for which there is no obvious cause. It is rare in children and in long bones. We report an unusual case of florid reactive periostitis in a ten-year-old girl that involved both bones of the forearm. The lesion resolved over a period of one year, leaving a residual exostosis. She developed a physeal bar in the distal ulna in the region of the lesion at one-year follow-up. This was thought to be a complication of the biopsy procedure and was treated by resection and proximal ulnar lengthening.

Intra-operative femoral head vascularity assessment: An innovative and simple technique

Background: Documentation of femoral head blood flow before, during and after head preserving surgery is important for safeguarding vascularity to the femoral head and for documentation in patients in whom the blood flow is compromised. Laser Doppler flowmetry and microsensor intracranial pressure (ICP) transducers have been used to satisfactorily depict such changes. However, these devices are expensive and not universally available in orthopedic operating rooms. We describe a new technique for the assessment of intra-operative blood flow to the femoral head. This is a technical description of a simple system utilized in eight patients to assess the femoral head vascularity using equipment available with the anesthetist. Materials and Methods: A standard epidural catheter attached to an arterial pressure transducer is introduced into the femoral head from the margin of the articular surface via a small hole drilled with a K wire. The pressure wave within the epiphysis is detected on the anesthesia monitor. Pressure within the femoral head is used as a surrogate for blood flow. The pressure and the wave form are correlated with the electrocardiogram (ECG) wave on the anesthetic machine. The technique was used in eight children with hip pathology requiring hip dislocation for documenting the hip vascularity status. Result: There was good correlation between the pressure wave and the ECG for a patient with presumed normal femoral head vascularity, whereas the pressure measurements were greatly reduced and the wave form was absent in a femoral head wih radiographic or bone scan evidence of avascular necrosis. Conclusion: This new technique is a cheap and readily accessible alternative to Laser Doppler flowmetery and ICPs monitoring probes for the assessment of blood flow to the femoral head.