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Though folic acid supplementation has reduced the incidence of Neural Tube Defects (NTD), NTD still constitutes one of the important congenital malformations having wide medical, social and ethical implications. Zinc deficiency has been reported to produce NTD in animals. This study was designed to evaluate zinc status of the newborn babies with NTD and their mothers. Eighty newborn babies with NTD and their mothers served as cases. Eighty apparently normal newborn babies and their mothers served as controls. Serum and scalp hair zinc levels were analyzed by atomic absorption spectrophotometry. The mean (+/- SD) serum and hair levels in normal mothers were 74.1 +/- 4.1 micrograms/dl and 142.3 +/- 8.0 micrograms/g respectively. The mean (+/- SD) serum and hair levels of the mothers who delivered NTD babies were 75.7 +/- 5.6 micrograms/dl and 129.9 +/- 5.3 micrograms/g respectively. The mean (+/- SD) serum and hair levels in normal newborn babies were 77.8 +/- 5.3 micrograms/dl and 188.8 +/- 6.2 micrograms/g respectively. The mean (+/- SD) serum and hair levels in NTD babies were 80.1 +/- 12.9 micrograms/dl and 174.2 +/- 10.7 micrograms/g respectively. The hair zinc levels of the affected babies and their mothers were significantly lower (P < 0.001) than the controls. This study has found association between NTD and decreased hair zinc levels and large population based studies are recommended to confirm the association between zinc and NTD and to investigate whether zinc supplementation would reduce the overall incidence of NTD. [ABSTRACT FROM AUTHOR]

The improved survival of neonates with esophageal atresia and tracheo-esophageal fistula reflects the advancement in neonatal care and anaesthesia over the years. Chick embryo studies have given new insights in the embryopathy of esophageal atresia. It is now apparent that the various types of esophageal atresia could be explained due to selective discrepancy in the growth of the 3 folds in the region of tracheo-esophageal separation. The early disturbances in organogenesis which result in esophageal atresia also lead to other associated anomalies, the incidence of which varies from 40 to 55%. These anomalies have an important bearing on the survival outcome. The physiological aspects of esophageal atresia such as esophageal dysmotility and gastro-esophageal reflux are also vital in the long term and proper treatment of the associated defects. The criteria for an ideal esophageal substitute in long gap esophageal atresia have been determined and several options are now available with good results, such as: gastric transposition, colon, gastric tube and small intestine. IN developing countries, however, a high mortality is still attributed to late referrals, low birth weight, hypothermia and chest infection. [ABSTRACT FROM AUTHOR]

It is a common clinical practice to estimate FSH, LH and testosterone levels in patients with unilateral undescended testis (U/L UDT) as a prognostic pointer to fertility potential. Is this practice correct? To ascertain this aspect, new born rats were operated to create experimental U/L UDT by gubernaculectomy and anchoring the gubernaculum to anterior abdominal wall. Fertility and hormone levels were evaluated later in adult life. Though fertility of rats with U/L UDT was significantly less (p < 0.01) than the controls, no significant alterations were found in the levels of serum testosterone, FSH and LH. Even though variations in the hormone levels may be responsible, to a certain extent, for the decrease in fertility potential in U/L UDT, estimation of sex hormone levels in U/L UDT is not a sensitive indicator of fertility potential. U/L UDT may additionally be affecting fertility through non-endocrinological mechanisms. [ABSTRACT FROM AUTHOR]

This retrospective study discusses the incidence of symptomatic voiding dysfunction, the etiological factors and the changes with age and with increasing duration of follow up in 67 boys treated for posterior urethral valves and followed up for a period ranging from 1-21 years. Twenty three of the 67 patients (34.3%) had symptomatic voiding dysfunction; the commonest symptom being nocturnal enuresis with diurnal urgency and frequency. Data in this study suggests a definite decrease in the incidence of urinary incontinence as the patient grows older or as the duration of follow up after valve fulguration increases. A Tc-99m DRCG based uroflowmetry was available in 31 of the 67 patients at the time of this review and evidence of detrusor dysfunction was identified in 9 of the 31 patients on the basis of uroflowmetry. Of significance was the detection of detrusor dysfunction on uroflowmetry in 3 patients with a history of normal urinary control and urinary stream with persistent post treatment hydroureteronephrosis eventually culminating into renal failure. Imipramine therapy was given to 10 of the 17 patients with symptoms of urinary frequency, urgency and nocturnal enuresis with a significantly symptomatic improvement in 9 of these patients. Urinary incontinence, detrusor dysfunction and its effect on the upper tracts and renal function is a significant determinant of the long term outcome of the boys treated for posterior urethral valves and this paper emphasises on the need to carefully evaluate and treat this aspect of all valve patients. [ABSTRACT FROM AUTHOR]

Etiological factors associated with portal hypertension in children influence the decision about therapy and the prognosis. This cross-sectional observational study was performed at a tertiary care centre in northern India from January, 1990 to December, 1994. Children below the age of 14 years with suspected portal hypertension were prospectively assembled into a cohort to determine the etiology and clinical profile of portal hypertension. Of the 115 patients with portal hypertension, 76.5% had extrahepatic portal hypertension (EHPH). Remaining 23.5% of the cases had intrahepatic and post-hepatic causes of portal hypertension. Children with EHPH had a significantly earlier onset of symptoms as compared to those with intrahepatic portal hypertension (p = 0.002) and bled significantly more frequently (p = 0.00). Forty per cent of patients with chronic liver disease (CLD) never had jaundice. History suggestive of potential etiological factors could be elicited in only 7% of EHPH patients. The commonest site of block in splenoportal axis was at the formation of the portal vein. An inverse relation of bleeding rates with duration of illness was seen in EHPH. Of the 10 CLD patients in whom liver biopsy could be done, cirrhosis was present in 6 patients. Understanding the natural history of EHPH and portal hypertension due to other etiologies may have significant implications in choosing the appropriate intervention and predicting the outcome. [ABSTRACT FROM AUTHOR]

Depending upon various factors the surgical procedures in childhood are divided into three groups--immediate, intermediate and elective. The timing of surgery is probably the most important factor governing the outcome of surgical correction in pediatric surgery. With continuing research and clinical experience our understanding of the various conditions have improved and with that has come a change in the optimum timing of many surgical procedures. This paper highlights the best timing of surgery for some of the commoner pediatric surgical procedures and the reasons behind these so that the children may be referred to pediatric surgeons in time. [ABSTRACT FROM AUTHOR]

The routine use of frozen section biopsy to identify distal ganglionic bowel in Hirschsprung's disease is the accepted norm in well developed centers. With the aim of overcoming non-availability of frozen section biopsy and reducing the duration of definitive surgery, an alternative algorithm, based on extramucosal seromuscular biopsy at the time of preliminary fecal diversion and its examination by routine paraffin sections and staining, was developed and used in 100 patients at our centre. This technique accurately marked the ganglionic colon for pull-through operation in 96.2% of cases with rectosigmoid transition zone and in 91.7% of those with transition zone in the descending colon, thus obviating the need for frozen section biopsy and saving nearly one hour during the pull-through surgery. However, in the more proximal varieties of Hirschsprung's disease frozen section biopsy was still considered to be indispensable. [ABSTRACT FROM AUTHOR]

Despite extensive research, controversies still exist regarding the etiology, pathology and management of biliary atresia. It is now thought to be a progressive panductal inflammatory obliterative process and not a developmental anomaly. The histologic changes are indistinguishable from neonatal hepatitis but some changes have prognostic significance. The clinical presentation is that of infantile obstructive cholangiopathy-waxing and waning icterus, clay coloured stools and high coloured urine from early neonatal period. The diagnosis is suggested by the absence of intestinal excretion on HIDA scan and confirmed on operative cholangiogram. Of utmost importance towards the final prognosis is early detection, prompt confirmation and surgical treatment before 2 months of age. Even with early treatment the result of bilioenteric drainage procedures have been discouraging in the long term. Portoenterostomy (PE) done in older children has been largely unsuccessful all over the world. The poor results of PE prompted the search for alternative treatment and liver transplantation (LT) has emerged as a viable treatment option both as a primary procedure and after failed PE. Although the technical know-how and infrastructure are available, LT in children has still not been done in India because of various economic and social constraints. It is hoped that all physicians and surgeons dealing with such patients would also consider this treatment modality. [ABSTRACT FROM AUTHOR]

The clinical and myelographic evaluation in 24 children with spina bifida occulta has been correlated with the operative findings. Myelograms were done using Myodil in 11 patients and Metrizamide in 13 patients. The diagnostic quality of myelograms done with Metrizamide was comparatively better. Myodil myelography failed to demonstrate diastematomyelia in one case and a dural sac in 3 cases of lipomeningomyelocele, whereas, Metrizamide failed to demonstrate a sac in one case of lipomeningomyelocele only. Worsening of the neurological status was observed in only 3 cases in whom myelography was done with Myodil. The radiographic and operative correlation has been discussed. [ABSTRACT FROM AUTHOR]

The profile of clinical presentation and management of 13 children with hydatid cysts of the lungs and abdomen is presented. The ages ranged from 3.5 to 13 years puv there were four females and nine males. The diagnosis could only be made after ultrasonography and chest X-rays in 12 patients. In one it was made postoperatively. Casoni's test was unreliable but indirect haemagglutination was positive in all the nine patients in whom it was done.The treatment consisted of either enucleation or excision and all the patients were treated with mebendazole postoperatively. The management of echinococcosis has been discussed in the light of recent literature. [ABSTRACT FROM AUTHOR]

Thirteen children with thalassemia, idiopathic thrombocytopenic purpura and hereditary spherocytosis who underwent splenectomy are presented. The clinical presentation, indications for surgery and response after splenectomy are evaluated. The current knowledge on the indications of splenectomy in children, the risks of septic complications and the benefits of surgery are berifly reviewed. [ABSTRACT FROM AUTHOR]

Suprapubic micturating cystourethrography is a technique of great value in evaluating lower urinary tract in infants and children. It has been employed in 28 patients of age group ranging from newborn to 12 years, without any serious complication. The minor complication of extravasation of contrast media into the perivesical and peritoneum was observed in three patients. The method is easy, safe, reliable and time saving. [ABSTRACT FROM AUTHOR]

Extralobar pulmonary sequestration is a rare pulmonary parenchymal anomaly which rarely may be present in an intra-abdominal location. The authors report a case of intra-abdominal extralobar pulmonary sequestration which presented to us as an antenatally diagnosed suprarenal mass and was worked up as such. The diagnosis was revealed only at laparotomy. Intra-abdominal extralobar pulmonary sequestration should also be kept in differential diagnosis in cases of masses in the suprarenal location, especially on the left side. [ABSTRACT FROM AUTHOR]

Objective: To evaluate for the occurrence of occult NVD in children with anorectal malformations (ARM) using urodynamic evaluation.Methods: This prospective study was carried out on children with ARM prior to and following definitive procedure. Urodynamic studies were performed on the Phoenix Griffon machine (Albyn Medical) using Phoenix plus software.Result: Nineteen children in the age range of 3 months to 156 months (mean = 19.2) were included in this study. Among these 19 children 13 underwent re-evaluation after definitive surgery for ARM. There were 11(57.9%) males and 8(42.1%) females. Of the 19 children 14 (73.7%) were cases of high anorectal malformation (HARM) and 5 (26.3%) were cases of low anorectal malformation (LARM). Baseline evaluation done in 19 children revealed seven urodynamic patterns: Normal capacity, compliant without uninhibited contractions (UIC) (21.1%); Normal capacity, compliant with UIC (5.3%); Normal capacity, poorly compliant without UIC (5.3%); Normal capacity, poorly compliant with UIC (10.5%); small capacity, compliant with UIC (5.3%); Small capacity, poorly compliant with UIC (26.3%) and large capacity, complaint with UIC (26.3%). Thirteen patients were evaluated post operatively also and in only 23% (3 of 13) no change in urodynamic pattern were observed. In the remaining 76.9% (10 of 13) some changes in urodynamics pattern were observed. The deleterious changes observed were appearance of UIC in 30.8% (4 of 13), decrease in the bladder capacity in 23% (3 of 13) and decrease in bladder compliance in 15.4% (2 of 13).Conclusion: Only 9 of of the 19 patients had normal urodynamics pre-operatively and post-operatively 3 more patients worsened. Incidence of occult NVD is high in patients with ARM even in the absence of clinical and radiological evidence of vertebral or lower urinary tract abnormalities. Though there seems to be a high incidence of changes in the neurovesical functions of these patients following definitive corrective surgery for ARM only time will show whether this has any deleterious effect on the upper tracts. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the incidence, types and the effect on outcome of associated anomalies in neonates with anorectal malformations (ARM).Methods: This retrospective study was carried out on all neonates with ARM admitted to the neonatal surgical intensive care unit (NSICU) from 1998 through 2003.Results: Of the 754 neonates admitted to the NSICU during the study period of 6 years, there were 124 (16.4%) neonates with anorectal malformations. Of these 110 were included in the study. 73 % were male and 27% female. 86% of these were high ARM (HARM) while only 14% were low ARM (LARM). Associated anomalies were seen in 68% of patients. The incidence was 72% for HARM and 50% for LARM. The major associated anomalies consisted of esophageal (13%), gastrointestinal (GIT) (11%), genitourinary (GUT) (32%), skeletal (26%), cardiac (33%) and miscellaneous 26%. The overall survival rate was 84% (82% for HARM and 94% for LARM). The survival among those with associated esophageal anomalies was 43%, GIT 67%, GUT 80%, cardiac 61%, skeletal 76% and miscellaneous 79% respectively. This difference in survival was significant only for those with esophageal (p=0.004) and cardiac anomalies (p=0.0026). The survival rates among those with one, two or more than two organ systems involved with associated anomalies were 88%, 82% and 58% respectively. This difference was significant only for more than two organ systems involvement (p=0.003).Conclusion: Associated anomalies are common in neonates with ARM, the incidence being similar for HARM and LARM. The survival depends upon the number and severity of associated anomalies both in patients with LARM and HARM. Neonates with more number of organ systems involved have a poorer survival specially when associated with esophageal and cardiac anomalies. All neonates with ARM merit a meticulous search for associated anomalies so that the management can be tailored for each baby. [ABSTRACT FROM AUTHOR]

Zinc is an important trace element having a definitive role in the metabolism, growth and development and reproduction. During pregnancy the requirements for zinc increase. This study was designed to evaluate the zinc status of normal women, normal pregnant women and their newborn babies. Forty normal adult females, 40 normal pregnant women and their newborn babies were randomly selected and their serum and hair zinc levels were analysed using atomic absorption spectrophotometer. The mean serum and hair zinc levels in normal women were 69.47 +/- 1.4 micrograms/dl and 147.45 +/- 6.12 micrograms/g respectively. The mean serum and hair zinc levels in normal pregnant women were 69.0 +/- 3.22 micrograms/dl and 142.83 +/- 4.39 micrograms/g respectively while the mean serum (cord blood) and hair levels in normal new born babies were 72.77 +/- 5.14 micrograms/dl and 188.36 +/- 4.12 micrograms/g respectively. There was a significant (p < 0.001) decrease in hair zinc levels during pregnancy. There was a significant (p < 0.05) decrease in zinc levels in new born babies when the time interval between the previous delivery and the present delivery was less than 3.4 years. The results of the present study reinforce the need for zinc supplementation during pregnancy especially if the interval between pregnancies is short. [ABSTRACT FROM AUTHOR]

Familial male pseudohermaphroditism (MPH) due to 17,20-desmolase deficiency is rare. Here we present two siblings with MPH possibly due to 17,20-desmolase deficiency. The first patient presented with unambiguous female external genitalia and hypergonadotrophic hypogonadism. Chromosomal analysis revealed 46 XY. Ultrasound evaluation of pelvis revealed gonads in the inguinal canal, and no uterus. These findings were confirmed on laparotomy. Histology revealed the gonads to be testes. The second patient had ambiguous genitalia (perineoscrotal hypospadias, bifid scrotum with palpable gonads) with a 46 XY chromosomal pattern. Both patients had high plasma 17-hydroxy progestrone (17 OHP), low normal dehydro epiandrosterone sulphate (DHEAS) and low plasma testosterone. Plasma testosterone and DHEAS showed no response to ACTH or HCG. These features are compatible with the diagnosis of 17,20-desmolase deficiency. [ABSTRACT FROM AUTHOR]

Between 1977 and 1985, 80 children with tracheobronchial foreign bodies were treated. Of these, 10 required open surgical procedures for extraction of the foreign bodies following unsuccessful bronchoscopy including 4 tracheotomies, 2 tracheotomies with bronchotomies, 2 bronchotomies and 2 pulmonary resections. There was no mortality. The associated problems and reasons for open surgical procedures are discussed. [ABSTRACT FROM AUTHOR]

Ultrasonography is a recently acquired diagnostic modality in our sub-continent. The initial experiences with this technique in 55 children with abdominal masses are presented. In 15 children ultrasonography merely confirmed the diagnosis which was arrived at by other means; a positive contribution towards the final diagnosis was made in 33 children, while in 7, ultrasonography did not contribute towards the diagnosis. The scope, advantages and pitfalls of ultrasonography are briefly discussed. [ABSTRACT FROM AUTHOR]

Trichobezoars are infrequently encountered today. They are rare in children. Four cases, encountered between 1978-1982 have been presented and the literature has been reviewed. Trichophagy is considered a manifestation of personality maladjustment and may be habit forming. Trichoberzoars have to be surgically or endoscopically removed and guidance is necessary to prevent recurrences. [ABSTRACT FROM AUTHOR]

Intracranial pressure was measured in 12 hydrocephalic children during ketamine anesthesia. Of the four who received no premedication, intracranial pressure increased in three and decreased in one patient. Seven out of eight children who received premedication with trimeperazine tartrate (3 mg/kg) orally showed a decrease in pressure. One patient who had a high initial pressure showed a steep rise necessitating termination of the study. Second dose of ketamine (1 mg/kg iv) produced a fall in all patients, being more prominent in those who had an elevation after the first dose. It is possible that the premedicants alter the response of cerebral vasculature to ketamine leading to a fall in intracranial pressure. Another possibility is that the standard intravenous dose of ketamine (2 mg/kg) is not uniformly effective in all patients of hydrocephalus. [ABSTRACT FROM AUTHOR]

In a review of 34 children with foreign bodies in the airway, between 1978 and 1982,24 were males and 10 females. Eighteen of them were between one and two years of age. Vegetable matter was the causative agent in 76-47 percent with 47.06 percent due to a portion of groundnut A history of foreign body aspiration was obtained in 24 patients and obstructive emphysema demonstrated on plain X-ray of the chest in 21 patients. Radioopaque foreign bodies were seen in only 6 patients. Foreign bodies were seen with almost equal frequency on both sides, 13 in the right bronchus and 14 in the left bronchus. All patients had their foreign bodies removed-one through the endotracheal tube, 29 bronchoscopically. 2 by tracheotomy and 2 by bronchotomy. There was no mortality. The details of the diverse clinical presentations, findings and method of treatment are also discussed. [ABSTRACT FROM AUTHOR]

In the period 1972-81, 572 hydrocephalic children were treated using the Upadhyaya shunt valve, One hundred and seventys-even of these patients needed a total of 350 shunt revisions. Indications for revisions were; shunt blockage 245 (70% of total revisions) shunt infections 40 (11.5%) catheter disconnections/fractures 19 (5.5%) and intvacranial hemorrhage 9 (2.5%). Intracranial bleeds were more commonly seen in hydrocephalus associated with tuberculous meningitis and were invariably fatal. Infrequent complications were CSF leak, shunt extrusion, valve incompetence, pulmonary hypertension and pleural effusion. Thirty-nine patients died. Overall results of the Upadhyayay shunt valve compare favourably with other reported series. [ABSTRACT FROM AUTHOR]

Four siblings in a family with alveolar microlithiasis are reported. All the children were asymptomatic. Silent chest with alarming findings in the x-ray chest is characteristic of the disease. [ABSTRACT FROM AUTHOR]