Joint hypermobility in Indian children

OBJECTIVES: To study the prevalence of joint hypermobility in children from Mumbai, India and to study its association with malnutrition. METHODS: In a cross-sectional field study from September '02 to February '03 in Mumbai, 829 children of the lower urban socio-economic strata, between 3 and 19 years of age were evaluated independently by two observers for hypermobility using the Beighton 9-point scoring system. A score of >or= 4/9 was considered positive. Their nutritional status was stratified using standard Indian growth charts and hypermobility was quantified in various nutritional groups. Musculoskeletal symptoms were assessed by a questionnaire given to parents. Standard tests of significance (Chi square test, p<0.05-significant) were applied. RESULTS: 58.7% of the population studied, had a Beighton score >or= 4/9. There was a declining prevalence of joint hypermobility noted with increasing age. Near equal sex incidence was noted. A higher incidence of finger signs was noted in comparison to elbow hyperextension, knee hyperextension and hands-to-floor. 26% of the hypermobile population had musculoskeletal symptoms as compared with 17.2% of the non-hypermobile population (p<0.05). A positive Beighton score was found in 452/734 (61.5%) children with Grade 3 and 4 malnutrition in comparison to 35/95 (36.8%) children with normal nutrition or mild grades (Grade 1 and 2) of malnutrition (p<0.05). In the group with Grade 3 and 4 malnutrition, 26.1% of those hypermobile had musculoskeletal symptoms in comparison to 17.7% of their non-hypermobile counterparts (p<0.05). CONCLUSIONS: In our study population: 1. A high prevalence of hypermobility using Beighton's score was noted; 2. Finger signs of the Beighton score were more common than the other signs; 3. Moderate and severe malnutrition were associated with hypermobility; 4. Musculoskeletal symptoms were linked to joint hypermobility; 5. Moderate and severely malnourished hypermobile children were more likely to have musculoskeletal symptoms as compared to their non-hypermobile counterparts.     

Relative Prevalence and Outcome of Fetal Neural Tube Defect in a Developing Country

Objectives

To find out the relative prevalence of fetal neural tube defect (NTD) and its outcome in terms of survival at birth and beyond 2 years of age.

Methods

A 10-year prospective (2008–2018) observational study was performed, which included all prenatally detected fetal NTD. Two-year follow-up was done in cases of pregnancies resulting in live birth, in terms of their survival, physical morbidity and developmental delay.

Results

NTD was seen in 401/648 (62%) cases among the central nervous system malformations. More than half of the cases (54.1%) presented after 20 weeks of gestation, and 42.8% of the mothers were primiparous. Spina bifida was seen in 206 cases, anencephaly in 144, encephalocele in 43, whereas iniencephaly was seen in only eight cases. Associated anomalies were present in 51.2%. Only 19.0% cases were live-born, and merely 11% were alive beyond 2 years of age. Among types of spina bifida, lumbosacral meningomyocele was the most common (41.6%), whereas thoracic was the rarest (8.7%). After 2 years, physical disability was observed in more than half of the cases who survived.

Conclusions

NTD is one of the commonest malformations with high mortality, and the physical and mental sub-normality is high among those who survive.

     

Longitudinal evaluation of time related femoral neck narrowing after metal-on-metal hip resurfacing

AIM: To track the short-term neck narrowing changes in Birmingham metal-on-metal hip resurfacing (MOMHR) patients. METHODS: Since 2001, the Center for Hip and Knee Replacement started a registry to prospectively collect data on hip and knee replacement patients. From June 2006 to October 2008, 139 MOMHR were performed at our center by two participate surgeons using Birmingham MOMHR prosthesis (Smith Nephew, United States). It is standard of care for patients to obtain low, anteriorposterior (LAP) pelvis radiographs immediately after MOMHR procedure and then at 3 mo, 1 year and 2 year follow up office visits. Inclusion criteria for the present study included patients who came back for follow up office visit at above mentioned time points and got LAP radiographs. Exclusion criteria include patients who missed more than two follow up time points and those with poor-quality X-rays. Two orthopaedic residency trained research fellows reviewed the X-rays independently at 4 time points, i.e., immediate after surgery, 3 mo, 1 year and 2 year. Neck-to-prosthesis ratio (NPR) was used as main outcome measure. Twenty cases were used as subjects to identify the reliability between two observers. An intraclass correlation coefficient at 0.8 was considered as satisfied. A paired t-test was used to evaluate the significant difference between different time points with P < 0.05 considered to be statistically significant. RESULTS: The mean NPRs were 0.852 ± 0.056, 0.839 ± 0.052, 0.835 ± 0.051, 0.83 ± 0.04 immediately, 3 mo, 1 year and 2 years post-operatively respectively. At 3 mo, NPR was significantly different from immediate postoperative X-ray (P < 0.001). There was no difference between 3 mo and 1 year (P = 0.14) and 2 years (P = 0.53). Femoral neck narrowing (FNN) exceeding 10% of the diameter of the neck was observed in only 4 patients (5.6%) at two years follow up. None of these patients developed a femoral neck fracture (FNF). CONCLUSION: Femoral neck narrowing after MOMHR occurred as early as 3 mo postoperatively, and stabilized thereafter. Excessive FNN was not common in patients within the first two years of surgery and was not correlated with risk of FNF.     

Ewing’s sarcoma of the maxilla

A case of primary Ewing’s sarcoma of the maxilla in a 14-year old male is reported. The disease and review of literature is briefly discussed.     

Paediatric myocardial protection-strategies, controversies and recent developments

During the last two decades, there has been a phenomenal rise in the number of patients undergoing early primary repair for congenital heart defects. Repair of these intracardiac defects usually requires open heart surgery that necessitates cardiopulmonary bypass, aortic cross clamping and administered cardiac arrest. To achieve this goal, cardioplegia is administered at predetermined intervals to ensure a quiescent heart and protection of the myocardium from ischaemia at the same time. Cardioplegia administration is usually done in conjunction with hypothermia to decrease the metabolic demands of the arrested heart as hypothermia alone is inferior to the combination of hypothermia and cardioplegia in providing adequate myocardial protection. The types and methods of cardioplegia in use today are as diverse as individual surgeons; and most institutions have over time developed their own preferred myocardial protection techniques that have proven to be safe and effective. Most of the available literature and concepts in pediatric myocardial protection today have been borrowed from observations in adults and ex- vivo and in-vivo animal models. The extrapolation of these concepts to pediatric myocardium is inappropriate as immature myocardium is not simply a “small adult heart”. It has unique differences and susceptibilities. This review provides a synopsis of pediatric myocardial protection including types, mechanisms, composition and comparative features of pediatric cardioplegia solutions currently in use all over the world. As of now, there is no evidence favoring one technique or strategy over the other. Pediatric myocardial protection protocols in general are currently experience based.