Recent advances in the management of congenital diaphragmatic hernia.

The management of congenital diaphragmatic hernia (CDH) is undergoing continual change and refinement, fuelled by recent advances in this field. Although many studies have documented the benefits of these recent advances but definite recommendations are lacking. Also, injudicious use of some of these strategies may be counterproductive, underscoring the importance of evidence based treatment strategy. This article discusses the utility of the recent advances in the management of CDH.

Peripherally inserted central venous lines versus central lines in surgical newborns - A comparison.

Objective. To compare the insertion characteristics, utilization profile, life span and the complication rates of Central lines (CL) and Peripherally inserted central lines (PICL). Methods. A prospective study of all CL or PICL insertions during January 2007 to September 2007 in the Neonatal Surgical Intensive Care Unit of a tertiary care center was done. The number of attempts, procedure time, duration of catheter stay, number of dressing done, complication during insertion and maintenance and cause of removal were noted and the differences analyzed statistically using Pearson chi square / t test. P value . 0.05 was considered significant. Results. Ninety two neonates were included in the present study of whom 60 were PICL insertions and 32 CL insertions. The two groups were comparable in terms of age, weight and the use of total perental nutrition (TPN) through the catheters. On comparing the PICL and CL groups, the number of attempts for successful insertion (p=0.003), the time taken (p=0.005), the number of dressing changes required during the indwelling period (p=0.005) and the overall complication rates (p=0.002) were significantly less in the PICL group. The PICL could be maintained for longer periods of time (p= 0.005) and only in 11.5% of the patients it had to be removed before completion of therapy as compared to 37.5% early removals for CL (p=0.02) Conclusion. PICL is a safe, effective and reliable method of providing prolonged IV access in newborns. It also has the least incidence of complications during insertion and maintenance over prolonged period of time when compared to CL and should be recommended for routine use in neonatal surgical patients.

Occult neurovesical dysfunction with anorectal malformations.

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.

Anorectal malformations and their impact on survival.

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.

Diffuse intra-abdominal fibromatosis-report of a new entity with review of literature.

Fibromatosis, arare non-neoplastic spindle cell proliferation of unknown aetiology, can occur anywhere in the body. Though extra-abdominal sites are commonly involved, intra-abdominal fibromatosis has also been described. Described herein is an unusual case of diffuse intra-abdominal fibromatosis in a 9-year-old boy, who could not be salvaged despite extensive medical management.

Duplications of the alimentary tract in children.

This study was conducted to assess the clinical, diagnostic and therapeutic approach in patients with alimentary tract duplication and review the relevant literature. A retrospective analysis of 28 patients of alimentary tract duplications treated between January 1990-December 1999 was carried out. There was a male preponderance (25:3); 71% of the patients were under 2 years of age. The presenting features were related to the anatomic location. Three patients were diagnosed antenatally. Real time ultrasonography and CT scan was undertaken for preoperative evaluation in the majority of patients. In selected cases a 99m-Tc-pertechnetate scan and barium meal follow through examination were also performed. Majority of duplications were in the jejunum and ileum. Twenty percent of our patients had thoracoabdominal duplication as compared to 2% reported in literature. Four patients required to be operated upon in the emergency for related complications. In the remaining patients complete excision of the cyst was done in 21 and only mucosectomy in 3 patients. Because these lesions are rare and can present with a wide range of clinical manifestations or may even be encountered intraoperatively, the appropriate surgical management requires the surgeon to be familiar with the anatomy and clinical characteristics of these lesions.

Generating finances for the chemotherapy of children with cancer.

BACKGROUND: To develop and evaluate a system of obtaining financial assistance for the treatment of children with solid tumours by involving individual members of society. METHODS: This prospective project was carried out at the Paediatric Solid Tumours Clinic of the Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, from January 1994 to December 1998. Donor families were enlisted by talking to affluent people. Families who agreed to help in this effort were told that they could 'adopt' a child for the purpose of his/her treatment and that they should purchase the prescribed chemotherapeutic drugs and give them to the family of the affected child. Therapy was started once the drug was received at the hospital. This process was repeated at each subsequent visit. RESULTS: Of the 291 children with solid tumours registered at the clinic, 45 (15.5%) received financial assistance by this method. The proportion of children receiving financial assistance increased from 8.6% in 1994 to 23% in 1998. Of all those who received assistance, 20 (44.4%) have completed therapy and are surviving, 11 are still on therapy, 12 died and 2 decided to discontinue therapy because of progressive disease. CONCLUSIONS: The advantages of this system far outweigh its disadvantages. This method of generating finances for children with cancer can be recommended to all doctors treating such children.

Use of mycelial suspension and metabolites of Paecilomyces lilacinus (Fungi: Hyphomycetes) in control of Aedes aegypti larvae.

Mycelial suspension of possible was assessed to examine its Paecilomyces lilanicus, a fungus, detrimental effects on fourth instar larvae of Aedes aegypti. The immature stages suffered 64-68% mortality with 0.5-1% mycelial suspension. There was 12-16% adult emergence which was statistically significant (P < 001). Czapeckdox and PYG media metabolites were used against the third instar larvae in various concentrations. The effects were evaluated on several parameters like mortality, mean survival time and time taken for adult emergence. The results indicate that the fungus does not producae any toxic metabolites.

Esophageal atresia and tracheo-esophageal fistula: a review.

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.

Bioactivity of ethanol extract of Karanja (Pongamia glabra vent) seed coat against mosquitoes.

Laboratory evaluation revealed that the treatment of larvae of Aedes aegypti and Culex quinquefasciatus with ethanol extract of karanja seed coat (ALKSC) significantly increased the larval mortality and developmental period proportianately with increase in the extract concentrations. Aedes proved more sensitive to the effect of extract in terms of mortality than Culex. At a high concentration (8 ppm), 100% Aedes larvae died in the first instar within two days whereas, 16 pmm was required for achieving the same level of mortality in Culex. Culex was also found more sensitive than Aedes regarding IGR effect. In emerging Culex pupae several abnormalities were observed while Aedes pupae did not exhibit any abnormalities. However larvae which pupated successfully resulted in adults with several structural abnormalities in both the species.

Congenital true pancreatic cyst: presentation and management.

Congenital true pancreatic cyst is a very rare cause of a cystic lesion of the pancreas comprising less than 1% of all pancreatic cysts. Most of these are reported in children less than 2 years of age. Described here is a case of congenital true pancreatic cyst in a 6 years old girl with the clinical, radiological and pathological details and the surgical treatment.

Endocrinological parameters in experimental unilateral undescended testis.

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.

Stone's enterostomy crushing clamp for modified Duhamel's procedure.

AIM: To evaluate the safety and advantages of the use of Stone's enterostomy crushing clamps in modified Duhamel procedure. MATERIAL AND METHODS: Prospective study at a tertiary care paediatric surgery centre between February 1994 through February 1998 involving all cases undergoing modified Duhamel procedure for Hirschsprung's disease. RESULTS: Twenty eight patients, ranging from 5 months to 14 years were included in the study. The clamp was found to be easier to apply, needed no extra pelvic dissection and there were no immediate or delayed complications. The children were more comfortable and could be mobilized out of bed sooner post-operatively. It is a much cheaper option than staplers. CONCLUSION: The Stone's enterostomy crushing clamp is a reasonably cheap, reusable, safe and effective alternative for the Kocher's clamp and stapling devices for modified Duhamel procedure.

Evaluation of the knee pathology by ultrasonography.

Fourteen patients undergoing total knee replacement for the arthritic conditions of the knee were evaluated by ultrasonography (USG) prior to surgery. USG findings (synovium, fluid, femoral cartilage, cyst and loose bodies) were compared with surgical findings. USG and surgical findings matched for synovial pathology in 57%, for presence fluid in 71% and for cartilage damage in 45%. Loose bodies and cyst present in one patient was not picked up by USG. Baker's cyst was detected in one patient.

Clinical course and management of pancreatoblastoma in children.

Pancreatoblastoma is a rare malignant tumour. Two children with this tumour were managed in the last 2 years. Both presented with progressively increasing abdominal mass. The diagnosis was established only after laparotomy. In the first child, an 8 year old girl, the mass was arising from the body of the pancreas and only incomplete resection was feasible. She received postoperative chemotherapy and went into remission for a few months before presenting with jaundice and abdominal pain due to recurrent, metastatic disease in the liver and porta hepatitis. Further therapy was refused by the patient because of anorexia and social problems. The second patient, a 5-year-old girl, underwent distal pancreatectomy for complete removal of a large mass arising from the tail of the pancreas. Chemotherapy was begun postoperatively but discontinued by the patient. However, she has remained disease free 1 year after diagnosis. Histologic, histochemical and ultrastructural features of the tumour are detailed and the management discussed.

Occult spinal dysraphism.

When spina bifida is associated with skin abnormalities such as dimples, sinus tracts hypertrichosis, or capillary hemangiomas, there is a high incidence of an occult intraspinal lesion such as epidermoid and dermoid tumours, lipomas, diastematomyelia, dural bands and tethered spinal cord. The present study consists of 50 patients with occult spinal dysraphism treated with the diagnosis of lipomeningomyelocoele (20), lumbosacral lipoma (15) and diastematomyelia (15). The clinical presentation varied from lipoma in the lumbosacral region, dermal sinus, cutaneous hemangioma and hypertrichosis. The age range varied from 2 months to 7 years with an average of 2 years. 40% patients had neurological deficit at the time of presentation which varied from lower limb weakness to bladder & bowel involvement. All patients underwent laminectomy of the lumbar and lumbo-sacral spine with excision of intraspinal lipoma, excision of bony or cartilaginous spur in diastematomyelia and detethering of the conus medullaris & cauda equina. No patient developed late neurological deficit.

Management of hydrocephalus.

The present study is an analysis of 747 patients with hydrocephalus, treated and followed up in the Hydrocephalus Clinic run by the department of Paediatric Surgery at the All India Institute of Medical Sciences, New Delhi. The distribution of patients was: congenital-46%, post-meningomyelocoele excision-28%, post-meningitic-21% and others-5% (including post haemorrhagic and post encephalocoele excision hydrocephalus. The average age was 7 months in the shunted group and 10 months in the medical group with overall male to female ratio of 2.3:1. The data were analysed to study the effect of treatment on ventriculomegaly and mental development with special reference to the type of treatment (shunt versus medical) and age at starting treatment. The probability of shunt failure was also studied. A comparison of ventricular size in US/CAT scans between the time of starting treatment and last follow-up revealed improvement in ventriculomegaly in 60% of the shunted patients but only 30% of the medically treated patients. A significant difference was particularly noted in patients with severe hydrocephalus, 72% and 22%, respectively. Comparison of the mean Mental Performance Quotient (MPQ) scores in the shunted & medically treated patients also revealed significantly better MPQ scores in the shunted group (p = < 0.001). Probability of shunt survival, as depicted by the Kaplan-Meier survival curve, revealed that there is a high rate of shunt failure in the first 12 months, followed by a dramatic slowing down. Our observations support the contention that CSF shunt surgery offers better outcome than medical management even when ventriculomegaly is severe at the time of presentation.