Immunohistochemical differentiation between spindle cell lipoma and neurofibroma of oral cavity using CD34 and SOX10

Spindle cell lipoma (SCL), also called as pleomorphic adenoma, is a rare variant of lipoma histopathologically characterized by an admixture of mature fat cells with spindle cells and occasionally mast cells with myxoid connective tissue stroma and thick bends of birefringent collagen. Although buccal mucosa is the most common location for oral lipomas, for SCL, it is an exceedingly rare location. We report a case of an asymptomatic swelling of buccal mucosa that simulated the features of neurofibroma on histopathological examination, and the final diagnosis of SCL was made on the basis of immunohistochemical features. This is the first documentation of oral SCL using SOX10 to achieve the final diagnosis.

Transfusion-transmitted hepatitis E: Is screening warranted.

Hepatitis E virus (HEV) is an emerging infectious threat to blood safety. In recent years, there have been a number of publications delineating this threat by providing evidence of the transmissibility of this virus through transfusions. The extent of transmission and its clinical relevance are issues under debate at present. HEV usually causes a self-limiting illness which subsides in a few weeks barring a few cases where fulminant hepatic failure occurs. The virus poses a risk of higher morbidity and mortality in pregnant females, patients with pre-existing liver disease and solid organ transplant recipients. As these categories of patient often require repeated transfusions or massive transfusions, they are exposed to a greater risk of transmission of HEV. At present, there is little evidence to advocate universal screening for this virus but considering that there is no definitive treatment for HEV induced hepatitis, selective screening should be advocated in blood products for high risk recipients in endemic areas.

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.

Circadian rhythm of peak expiratory flow rate in healthy north Indian men.

Peak expiratory flow rate (PEFR) variability follows a specific pattern in asthmatics as well as in healthy individuals. There is scarcity of data for Indian healthy subjects. The PEFR (L/min.) was measured with Wright's portable peak flow meter at 05:00, 8:00, 11:00, 14:00, 17:00, 20:00 and 23:00 hours in 42 healthy, non-smoking adults of age group between 18-26 years. The variability of PEFR revealed a circadian pattern. PEFR levels tend to increase from morning at 5:00 hours till evening at 17:00 hours, with peak PEFR in evening at 17:00 hours, after which there was a progressive fall in PEFR levels, till morning 5:00 hours. This study provides the preliminary reference data of circadian pattern of PEFR in healthy individuals.

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.

AIIMS clinical score: a reliable aid to distinguish neonatal hepatitis from extra hepatic biliary atresia.

It is important to distinguish neonatal hepatitis (NH) from extra hepatic biliary atresia (EHBA) in a neonate presented with jaundice as the former is purely medical management and the latter requires surgical procedure at the earliest. The observations on the critical evaluation of the neonatal jaundice cases led us to propose AIIMS clinical score (ACS) and this retrospective study was designed to evaluate the reliability of the ACS in all the 120 babies with jaundice referred to the pediatric surgery department during the past 10 year period. The ACS described here is based on 5 clinical parameters--Age, Jaundice, Colour of urine and stool and Clinical examination of liver. The accuracy of the hepatobiliary scan (HBS) with respect to the diagnosis of EHBA in this series, as compared to per operative cholangiography (POC) which is considered as the gold standard to distinguish these conditions was only 68%. However, ACS showed a sensitivity of 91.5%, a specificity of 76.3%, positive predictive value of 89.2%, negative predictive value of 80.5% and an overall diagnostic accuracy of 86.6%. ACS is reliable to distinguish NH from EHBA at bedside.

Incarcerated diaphragmatic hernia secondary to plication for eventration of diaphragm.

Diaphragmatic plication is the procedure of choice for congenital diaphragmatic eventration. In the absence of complications, most newborns with eventration do well after plication and have normal long-term respiratory functions. However complications are rare. Recurrence, ipsilateral pneumothorax and rupture of the diaphragm have been reported in literature. We report a case of incarcerated diaphragmatic hernia in a two-year-old child who had undergone plication for eventration of the diaphragm at six months of age.

Congenital diaphragmatic hernia.

Over the last two decades there has been a constant improvement in the understanding of the pathophysiology of Congenital Diaphragmatic Hernia (CDH) and its management. However, the ideal treatment remains elusive. The earlier management strategy of immediate surgery is replaced by the principle of physiological stabilisation and delayed surgery. Conventional mechanical ventilatory techniques, with high pressures and hyperventilation to reverse ductal shunting and cause alkalinization, are being questioned because of the risks of barotrauma and consequent broncho-pulmonary dysplasia. It has also been shown that paralysis with pancuronium bromide for patients on conventional mechanical ventilation results in increased incidence of sensorineural hearing loss in childhood survivors of CDH. With the introduction of the concept of permissive hypercapnia and high frequency oscillation ventilation, the complications of pulmonary barotrauma are circumvented. Although ECMO therapy is invasive, yet has improved survival by about 15% independently, especially in critically ill infants who have the predictive mortality rate of more than 80%. Further insights into the pathophysiology of CDH and the introduction of less invasive therapeutic techniques in the form of high frequency oscillation ventilation, inhalation nitric oxide, surfactant, and perfluorocarbon liquid ventilation may even make the need for ECMO redundant.

Intestinal atresia.

Intestinal atresia accounts for about one third of all cases of neonatal intestinal obstruction. The survival rate has improved to 90% in most of the series with the operative mortality being < 1%. The survival rate improves with distal atresias. An increased mortality is observed in multiple atresias (57%), apple peel atresia (71%), and when atresia is associated with meconium ileus (65%), meconium peritonitis (50%) and gastroschisis (66%). Although appearance of echogenic bowel on prenatal ultrasonography is suggestive of GI, it is confirmed in only 27% cases. Prenatal ultrasonography is more reliable in detection of duodenal atresia than more distal lesions. Short bowel syndrome is the major impediment in the management of jejunoileal atresia. Although total parenteral nutrition (TPN) is the main adjunctive treatment, it delays intestinal adaptation and may cause cholestasia and subsequent liver damage. Graduated enteric feedings, use of growth hormone, glutamine and modified diets containing low fat, complex carbohydrates and protein supplements have been used in a adults with short bowel syndrome to successfully diminish TPN requirements and enhance nutrient absorption in nearly half of the patients. Utilization of growth factors to facilitate intestinal adaptation and advances in small bowel transplant may improve the long-term outcomes in future.

Neonatal Hirschsprung's disease.

Since the classic description of this disease by Harold Hirschsprung's. In 1886 just over a century ago, there has been a marked evolution in the diagnostic modalities, understanding of the pathophysiology, unraveling of genetic association and the treatment approach of this disease. Recent research on the molecular biology of the disease has bestowed us with a clearer understanding of the pathogenesis of the disease. Genetic deletions have been identified which explain the familial incidence of 3.6%-7.8%, and in some kindreds the inheritance rate approaches 50%. Improvement in the diagnostic approaches has evolved from full thickness rectal biopsy (under GA) to suction rectal biopsy. The development of the histochemical staining for acetylcholinesterase, allows rapid identification in the lamina propria of hypertrophied extrinsic nerve fibers which have proliferated in the absence of intrinsic ganglion cells. Frozen sections now permit screening for normal ganglia in the myenteric plexus from seromuscular biopsies during the definitive pull-through procedures or leveling colostomy. This service, however, requires significant infrastructure and an expert pathologist, not widely available. Hirschsprung's disease (HD) variants like intestinal neuronal dysplasia(IND), hypoganglionosis are better identified and treated with availability of histochemical staining. The three-stage procedure till date remains the gold standard; however, increasingly, the world over, two stage and even single stage procedures are being tried. In the era of minimally invasive surgery, perineal pull through procedure has been attempted with or without laparoscopic assistance.

Management of bladder exstrophy.

The exstrophy-epispadias complex is a severe anomaly of the lower urinary tract. While the surgical reconstruction of normal anatomy in patients with this disorder was once considered impossible, modern management has made possible a near-normal reconstruction of the bladder and lower abdominal wall. Early operation in the neonatal period along with closure of the pelvic ring has been shown to significantly improve results in terms of continence and achievement of adequate bladder capacity. With the possibility of prenatal diagnosis, it is important that the counseling obstetricians and neonatologists should understand the basis of the surgical management and the outcome that can be expected.

Surgery in tuberculosis.

Tuberculosis in children involves many organs and systems including lymphatics, lungs, CNS, GIT and genitourinary. Although the mainstay of treatment is medical, surgery has a definite role under specific circumstances. It is important to differentiate atypical mycobacterial infection wherein surgery is the primary modality of treatment. The lung is most commonly involved in tuberculosis and besides bronchoscopy, thoracic procedures range from tube thoracostomy to decortication and lung resection. Neurotuberculosis constitutes almost half the cases of extrapulmonary tuberculosis and tuberculous meningitis (TBM) is the commonest type of CNS involvement. Hydrocephalus is a late complication of TBM and shunt surgery is indicated when signs and symptoms of raised intracranial pressure persist despite adequate medical therapy. Abdominal tuberculosis could be peritoneal or gastrointestinal. Either form can complicate the other and each can present in acute, subacute or chronic form. Surgical therapy is reserved for complications like strictures, fistulae and GI bleed. Genitourinary tuberculosis constitutes 15-20% of all extrapulmonary disease and epididymitis is the most common manifestation in the males. Surgery is generally reserved for management of complications such as ureteral strictures, perinephric abscesses and nonfunctioning kidneys.

Surgical aspects of chronic constipation in children.

Chronic constipation accounts for 3% of paediatric visits. These patients can be broadly classified into three groups: (a) medical group (60-70%), (b) surgical group (20-25%), (c) refractory constipation group (5-10%). The first two groups can be managed by dietary adjustments and surgery respectively. The recognition and management of refractory constipation is a slower process and needs close scrutiny and perseverance before a definite therapeutic option could be decided.

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.

Gastroschisis and omphalocele.

The survival rate of patients with abdominal wall defects has gradually improved with the advances in the investigation and treatment modalities. The present paper reviews the results of various treatment modalities and also analyses the long term results in these patients. A meta-analysis was performed via a medline search of English written clinical studies containing the text words "abdominal wall defects", gastroschisis and 'omphalocele or exomphalos" from 1953 to 1998. The present consensus on operative management of abdominal wall defect is to provide primary closure, if it can be achieved without haemodynamic or respiratory compromise. Patients with primary closure on analysis were found to have better survival rates, reduced risk of sepsis and overall, a shorter hospital stay. However, resumptions of oral feeds, duration of total parenteral nutrition (usually lasting 10-15 days) and ventilatory support required postoperatively did not significantly differ in the primary and silo technique. Long term outcome of these patients is generally good, but they have high incidence of GER (40-50%) for which they should be on regular follow up.

Bilateral Wilms tumor: an eleven year experience.

Over the last 11 years, 101 patients of Wilms tumor were treated at our hospital. Of these, 7 had bilateral Wilms tumors (BWT). The first two patients were referred in terminal stages and died. Of the latter five, three have survived while one is on treatment. One patient succumbed to liver metastasis, he had stage III disease on one side and was irregular in his chemotherapy protocol. Remarkably, one of the survivors was cured even after developing vertebral metastasis, retroperitoneal recurrence and bilateral lung metastasis. Part of the success was due to "salvage chemotherapy regimens" using Cis-plantinum, ifosphamide and VP-16. Flow cytometric analysis of DNA ploidy performed on only three of the five patients showed that two tumors were aneuploid and one was diploid. Spectacular cure rates can be achieved in BWT by renal conservation surgeries and effective chemotherapy. The use of "salvage chemotherapeutic regimes" and the study of DNA ploidy is being highlighted.

Neurogenic bladder: urodynamic and surgical aspects.

Children with spina bifida often have vesico-urethral dysfunction manifesting either as upper urinary tract deterioration or voiding dysfunction, chiefly incontinence. Surgery of the back and presence of or increase in hydrocephalus may contribute to neuro-urologic worsening; secondary cord tethering and syrinx or hydromyelia may be additional factors coming into play later in life. Urodynamic assessment using simple modalities like uroflowmetry, external sphincter EMG, residual urine volume and cystometry provide data useful to classify patients according to detrusor and sphincter activity. Besides diagnosis, urodynamic studies are useful in guiding therapy of children with vesico-urethral dysfunction, and for their follow-up to detect sub-clinical deterioration. They also help to prognosticate risk of upper tract deterioration and the possible success of measures to contain incontinence. Urodynamic data in thirty one patients with spinal dysraphism who presented to us with urologic symptoms were analysed. Twenty-three children had hyper-reflexic bladders while in the other 8 the bladder was areflexic. 13 children showed upper tract dilatation. The leak point volume was significantly lower in this group of patients compared to those who did not show upper tract dilatation. Our results are comparable to earlier similar studies.

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.