Normative magnetic resonance imaging measurements of orbital structures in pediatric population of North-Eastern India: A retrospective cross-sectional study.

Purpose: The people of Northeast India comprise mostly of Mongoloid descent with characteristic craniofacial features. The purpose of this study was to evaluate the normal orbital structures with MRI and determine normative data in the Northeast Indian pediatric population. Methods: MRI images of the 302 orbits of 151 pediatric patients below 16 years of age were retrospectively evaluated to measure the various orbital structures. Both axial and coronal MRI images were utilized for various orbital measurements. An independent sample t-test was done to compare various orbital data according to the sex and side. Linear regression was also done. Results: The mean age of the pediatric population was 9.64 ± 1.47 years with a male: Female ratio of 1.13:1. The mean interzygomatic line was 88.4 ± 9.09 mm and the interorbital line was 22.18 ± 3.62 mm. The mean thicknesses of medial, lateral, superior, and inferior recti muscles were 2.58 ± 0.46, 2.34 ± 0.42, 2.16 ± 0.40, and 2.53 ± 0.49 mm in males and 2.41 ± 0.41, 2.08 ± 0.34, 2.08 ± 0.46, and 2.46 ± 0.49 mm in females. The mean horizontal orbital, vertical orbital diameters, orbital index, mean volume of eyeball, and orbital cavity were 30.27 ± 2.97,3 7.06 ± 3.57, 122.58 ± 7.39, 4.63 ± 0.84, and 15.29 ± 3.52 in males, while 29.16 ± 3.23, 34.96 ± 3.99,119.96 ± 7.31, 4.49 ± 0.87, and 14.65 ± 3.47 in females, respectively. With an increase in age, the interzygomatic line (r = 0.883, r2 = 0.780; P < 0.0005), anterior medial interorbital line (r = 0.808, r2 = 0.652; P < 0.0005), mean volume of eyeball (r = 0.915, r2 = 0.838; P < 0.0005), orbital cavity (r = 0.924, r2 = 0.854; P < 0.0005), and distance between the optic nerve entry site (r = 0.829, r2 = 0.687; P < 0.0005) were increased. Conclusion: This study provides normative data of various orbital structures in a pediatric population and these data likely to be useful for diagnosing various pediatric orbital disorders and helps in the planning of various surgical procedures of orbits.

Long telomeres cooperate with p53, MDM2, and p21 polymorphisms to raise pediatric solid tumor risk.

BACKGROUND: While leukocyte telomere length has been linked with altered risk in adult cancer, limited information is available on its association with risk in pediatric solid tumors. We investigated the association of telomeric alterations with risk of pediatric solid tumors. We also investigated whether altered telomeres cooperated with the TP53 rs1042522, MDM2 rs2279744 and CDKN1A (p21cip1 ) rs1059234 single-nucleotide polymorphisms to modify cancer risk. METHODS: A total of 101 tumor patients and 202 controls were recruited for this age- and gender-matched case-control study. Relative telomere length (RTL) was determined in peripheral blood leukocytes using quantitative real-time polymerase chain reaction (PCR), and the polymorphisms were genotyped using PCR-restriction fragment length polymorphism. RESULTS: Using median RTL in the healthy controls as a cut-off, children with longer telomeres were at an increased risk of developing a solid tumor (OR, 2.70; P < 0.01). When participants were categorized according to control RTL quartiles, a significant dose-response relationship was observed (χ2  = 10.95; P < 0.001). The risk for tumors increased nearly threefold (P = 0.001) for the triple interaction RTL × TP53 rs1042522 × p21cip1 rs1059234 compared with the maximum effect of any single factor, although the interaction effect was less than additive. The MDM2 rs2279744 GG genotype reduced pediatric solid tumor risk significantly (OR, 0.51). CONCLUSION: Combined analysis of telomeres and genetic polymorphisms in the TP53 pathway can provide important clues to understanding pediatric solid tumor etiology.

Anterior encephaloceles in children of Assamese tea workers.

OBJECT: Anterior encephaloceles are rare congenital malformations. Most of the cases in the literature are reported from Southeast Asia. In India it is seen more frequently among manual laborers in the tea gardens of Assam. A brief background of the patients, clinical presentation, operative treatment, and outcome are discussed, with a review of the relevant literature. The causes and pathogenesis of anterior encephaloceles are discussed. The authors' surgical approach to repair of the defect, postoperative complications, and results are described. METHODS: Twenty-eight patients (mean age 38 months, range 1 month-12 years) with anterior encephaloceles who presented between 1998 and 2007 are included in the study. Patients were assessed for physical and psychological growth and development; any associated anomalies were noted. A detailed history of the patient's family, including the prenatal history, was obtained. Lesions were classified with the help of neuroimaging studies (skull x-ray, CT, ultrasonography, and MR imaging studies) and confirmed at surgery. RESULTS: Sixteen patients had nasofrontal, 9 had nasoethmoidal, and 3 had nasoorbital encephaloceles, and 12 patients had associated hydrocephalus. The modes of presentation were nasofrontal swelling, watering from the eyes, CSF leakage, fever, and vomiting. Parents of all the patients were ethnic tea garden workers. The average parental age at the time of marriage was 24 years for men and 18 years for women. Consanguinity was present in 6 patients. Alcohol consumption and tobacco chewing were a common practice in both parents. A total of 42 surgical procedures were done in 16 patients, one of whom died of postoperative meningitis. The mean follow-up duration was 38 months (1-92 months). CONCLUSIONS: Anterior encephaloceles are rare in Western countries and other states in India, but this defect is more commonly seen among the ethnic tea garden workers in Assam. Transcranial repair is the treatment of choice. Unlike encephaloceles in other locations, anterior encephaloceles have a better clinical outcome after surgery.

A novel, easy, non-operative method of treating prolapsed colostomy.

Colostomy prolapse is a major cause of morbidity in paediatric patients with Hirschsprung's disease and anorectal malformations. Although it is commonly associated with the distal loop of a transverse colostomy, a sigmoid stoma can also be affected. We report six babies with anorectal malformations between day 10 and 6 months presenting with incessant crying and irritability following prolapsed colostomy stomas. In four patients only the distal stomas were involved but in the other two both proximal as well as distal loops had prolapsed. All the babies had poor oral intake and had bleeding from the prolapsed stomas. Manual reposition with sedation did not help. After reducing the prolapsed part a no. 7 or 7.5F Romsons tracheostomy tube was introduced through the stoma. A cotton tap (16-mm wide) was tied to the flanges of the tracheostomy tube and fastened around the flank for proper fixation. None of the babies had further prolapse and were passing stool through the tube. The tubes were kept for an average period of 4(1/2) (3-9(1/2)) months. Only one patient had frequent displacement of the tube, which the mother learned to reposition without any other problems. We feel that this non-operative simple procedure can be used for treatment of massive colostomy prolapse without any complications. Parents can learn this procedure easily thus avoiding frequent hospitalisation and other invasive procedures.

Embryogenesis of esophageal atresia: Is localized vascular accident a factor?

Several theories on embryogenesis of esophageal atresia have been proposed, none could explain the whole spectrum of this anomaly. We report a new variant of esophageal atresia in which the two blind pouches were joined by an atretic band. Histology of the atretic part showed groups of striated muscle arranged haphazardly without any lumen. The existing theories on etiology of esophageal atresia cannot explain this variant. However, localized vascular accident during intrauterine life resulting in disturbances in regional microcirculation could be a possible factor as demonstrated by Louw and Barnard in relation to jejunoileal atresia. This is contrary to the current understanding that disproportionate growth of the horizontal esophageal folds results in esophageal atresia.