Undescended testicle: An update on fertility in cryptorchid men.

Cryptorchidism or undescended testis is one of the most common anomalies encountered in paediatric urology and is estimated to affect 1 to 4 per cent of full term and upto 30 per cent of preterm male neonates. The associated problems of sub-fertility or infertility and malignant transformation have been recognized for long. Fertility is impaired after both unilateral and bilateral cryptorchidism. The reported paternity rates in adults are about two-third for unilateral undescended testis and less than one-third for bilateral disease. Over the last five decades, the concepts related to cryptorchidism have changed dramatically as knowledge about its effects has accrued from research conducted worldwide. The recommended age of orchidopexy has fallen progressively from adolescence to less than one year. The realization that the infantile testes are not in a state of 'suspended animation' and the recognition of the defect in the androgen dependent transformation of gonocytes into adult dark spermatogonia in cryptorchidism have been recognized as the primary cause of sub-fertility in these patients. This has paved the way for hormone therapy in an attempt to simulate the 'post-natal gonadotropin surge' or 'mini-puberty'. This review summarizes the current knowledge about the various factors affecting the fertility status in cryptorchidism with a particular focus on the derangements in the development and maturation of the germ cells and the role of surgery, hormone therapy and antioxidants in reversing these changes.

A highly efficient in vitro propagation protocol for elephant tusk cactus: Coryphantha elephantidens (Lem.) Lem.

BACKGROUND: Elephant tusk cactus Coryphantha elephantidens (Lem.) Lem. is an important attractive ornamental cactus. The plant produces offshoots from tubercles very rarely, and the seedlings exhibit slow growth and susceptibility to damping off. Slow growth and high demand in the cactus industry lead to finding an alternate fast propagation method. RESULTS: An innovative in vitro technique based on axillary bud proliferation has been developed for an ornamental cactus C. elephantidens (Lem.) Lem. Four different explant types formed multiple shoots on Murashige and Skoog (MS) medium. Of the two cytokinins, 6-Benzylaminopurine (BAP) and Kinetin (KN), BAP proved to be more effective for multiple shoot induction and shoot growth from different explant types. Longitudinally cut stem explants, when cultured on MS medium supplemented with 6.6 µM BAP give maximum axillary shoot proliferation (12.4 shoots). Type of explant significantly influenced the micropropagation rate. Type of carbon source used in the medium imparted a profound effect on shoot growth and dry weight. The maximum dry weight gain of the shoot was observed with 9% sucrose. CONCLUSION: Development of an efficient micropropagation protocol which can be used to produce more than 10,000 rooted plantlets in 150 days from a single longitudinally divided shoot explant.

Delayed presentation of strangulated congenital diaphragmatic hernia: learning from our experience.

BACKGROUND: Strangulation is very rare in congenital diaphragmatic hernia (CDH) of the Bochdaleck variety. Here, we share our experience with six cases of delayed presentation of strangulated CDH. The aim of this article is to provide information on how to diagnose and manage this situation using a systematic approach. MATERIALS AND METHODS: A retrospective review identified six cases of strangulated/obstructed CDH from 1998 to 2011. Demographic data, clinico-radiological findings, management and complications, along with final outcome were recorded. RESULTS: Small bowel gangrene was found in one patient, gastric perforation in three, transverse colon perforation in one and colonic obstruction in one patient. Video-assisted thoracoscopic surgery (VATS) was used in all but one patients for definitive diagnosis, diaphragmatic repair, pleural lavage and management of empyema. Laparotomy was needed for management of strangulated or perforated bowel. Three patients in this study survived. CONCLUSION: Clinicians should always consider a diagnosis of obstructed Bochdaleck hernia in children, because X-ray findings are not always typical or even normal in complicated CDH. VATS may be considered as both diagnostic and therapeutic. Preventive measures for empyema or early intervention in the evolving stage can significantly reduce morbidity.

Simultaneous occurrence of jejuno-jejunal and ileo-ileal intussusception in a child: a rare occurrence.

Intussusception is the most common cause of intestinal obstruction in infants and children. This condition is frequent in children and presents with the classic triad of cramping abdominal pain, bloody diarrhoea and a palpable tender mass. Small bowel intussusceptions are much less common, with jejuno-ileal and duodeno-jejunal intussusceptions being the rarest types of all. Multiple simultaneous intussusception is a peculiar variety of intussusception. The authors report the simultaneous occurrence of jejuno-jejunal and ileo-ileal intussusception in a patient. As this is an extremely uncommon entity, it is being reported with a brief review of the relevant literature.

Modified method of primary esophageal anastomosis with improved outcome in cases of esophageal atresia with tracheoesophageal fistula.

Survival rates for infants who have esophageal atresia (EA) with or without fistula (TEF) have improved dramatically in the past 50 years. Despite excellent long-term survival for patients with esophageal atresia with tracheoesophageal fistula (EA-TEF), many significant complications can occur. Anastomotic leak at the esophagoesophagostomy site is one such problem resulting in considerable morbidity and mortality in these patients. The methods of esophageal anastomosis for long period has remained the simple end to end anastomosis of esophageal ends with various modifications described from time to time. The present study aims to study the effect on the early postoperative complications, following horizontal mattress suture technique on the primary esophageal anastomosis in cases of EA-TEF. A total of 32 patients with EA-TEF, were operated by our technique during a period of 1 year (2007-2008). The results were compared with the patients (n = 66), who were operated by the traditional simple technique during the same period. Among those patients in whom the esophageal anastomosis was done by horizontal mattress suture, only one had major anastomotic leak, while two had minor anastomotic leaks, as compared to six and nine cases correspondingly in other patients in whom anastomosis was done by simple technique. There was single mortality. We propose that, the utilization of our technique of horizontal mattress suture in primary anastomosis of esophagus in cases of EA-TEF significantly reduces the risk of anastomotic leaks and subsequent morbidity and mortality.

Melanotic neuroectodermal tumor of infancy: 2 decades of clinical experience with 18 patients.

PURPOSE: The purpose of this article is to report our experience in the management of 18 patients with melanotic neuroectodermal tumor of infancy involving the maxillary alveolus. PATIENTS AND METHODS: All patients presented with hard nontender swelling involving the upper alveolus with facial deformity. Analysis included hematocrit, coagulation profile, serum creatinine, and screening for vanillyl mandelic acid and catecholamines. Imaging studies included x-ray of the maxilla and chest, ultrasound of the abdomen, computed tomography scan (1990 to 1999), and magnetic resonance imaging (after 1999). All surgeries were performed using endotracheal anesthesia, and complete gross excision of the tumor was achieved with coverage of the defect with mucoperiosteal flaps. All specimens were subjected to histopathology and immunohistochemistry. RESULTS: The expansion of the alveolus produced by the tumor improved in 4 to 6 months. Subsequent dentition was affected by the removal of involved tooth buds during the operation. All the patients are in regular follow-up (maximum 206 months) and there has been no local recurrence or distant metastasis. Mean follow-up time was 130.8 months (95% confidence interval, 168.8-210.6). Overall survival at 17 years was 85.6%. Median survival could not be established due to statistically insignificant sample size, while mean survival time was 189.7 months (95% confidence interval, 103.7-157.8). CONCLUSIONS: In the absence of metastatic disease, melanotic neuroectodermal tumors of infancy can be successfully managed by local excision.

Anorectal junction stenosis: diagnosis and management.

PURPOSE: The aim of this paper is to describe the management of 9 patients with anorectal junction stenosis and present the diagnostic features together with a single-stage surgical technique with reproducible results. MATERIALS AND METHODS: Nine patients with anorectal junction stenosis were seen over a period of 12 years. The children (aged from 2 months to 15 years) presented with constipation . Anorectal junction stenosis was diagnosed by rectal examination during which the tip of the finger was unable to pass beyond the stenosis in all cases. Contrast study showed the dilated rectosigmoid proximal to the stenosis. RESULTS: Six patients (who did not have significant rectal dilation) underwent single-stage surgery by posterior Y-V plasty which was curative. Two patients were operated through the posterior sagittal route with a covering colostomy done during the same session; the oldest child required resection of the megasigmoid with abdominoperineal pull-through. None of the six patients operated with Y-V plasty experienced any complications. One of the patients operated via the posterior sagittal route had a leak from the anorectal anastomosis requiring revision. The follow-up ranged from 6 months to 12 years. All patients are alive and well and there was no recurrence of stenosis in any case. CONCLUSION: Anorectal junction stenosis is a rare anorectal anomaly easily diagnosed by digital rectal examination. Single-stage surgery by posterior Y-V plasty is effective in curing the majority of these patients if significant rectosigmoid dilation is not present.

Evaluation of congenital diaphragmatic hernia in a tertiary health center of a developing country: management and survival.

AIM: The purpose was to evaluate the diagnosis and efficacy of management of congenital diaphragmatic hernia (CDH) in a tertiary health center of a developing country. METHODS: Forty-six children aged from 1 day to 7 years were studied. Parameters studied were age, sex, clinical features, and management. RESULTS: Fifty-six percent of patients presented in the neonatal period; however, none of them presented on the first day of life. The majority (91.3%) of patients had left-sided CDH. Respiratory distress was the most common clinical feature observed (91.3%). Chest X-ray confirmed the diagnosis in 82.6% of patients, and contrast study was needed in the remaining 17.4%. The survival rate was 87%. It was better in patients presenting late than those presenting in the early neonatal period. Stabilization in the preoperative period improved survival. Not using a chest tube had no adverse effect on survival. CONCLUSION: The relatively increased survival rate of CDH in a tertiary health center of a developing country is attributed to delayed arrival to the center. Respiratory infections compound the survival. More studies are needed before it can be safely said that not using a chest tube has no adverse outcome. Late presentation has been associated with varied manifestations, hence proper clinical evaluation, a high index of suspicion and adequate management, which includes imaging and surgery after stabilization, gives excellent results.

Quality of life and gender role behavior in disorders of sexual differentiation in India.

OBJECTIVE: Culture-specific tools to assess longterm psychosocial outcomes for patients with disorders of sexual differentiation are scant. We conducted a study to develop tools for evaluating gender role behavior and health related quality of life for Indian adolescent patients with intersex disorders. We also studied factors important to parents while deciding sex of rearing for their baby. METHODS: A 29-item gender role behavior questionnaire and an 18-item health related quality-of-life questionnaire were administered to 82 healthy controls, 13 patients with intersex disorders and 18 patients with type 1 diabetes mellitus. Internal consistency was checked by Cronbach's alpha and test-retest reliability using intra-class correlation coefficient. Responses of 28 parents to a questionnaire on factors affecting the decision of sex of rearing were recorded on a 5-point Likert scale in order of importance. RESULTS: Cronbach's alpha was 0.92 and 0.75, and intra-class correlation coefficient 0.76 and 0.75, for the gender role behavior and quality-of-life questionnaires respectively, indicating a high degree of internal consistency and stability. The mean composite scores for healthy girls on the gender role behavior questionnaire (82.5 +/- 8.7) differed significantly from that for healthy boys (53.2 +/- 7.1, p <0.001). Factors important to parents while making decisions for sex of rearing were appearance of the genitalia, medical advice, ability to bear children and economic independence. CONCLUSIONS: We have created valid tools to study gender role behavior and quality of life in adolescent patients with intersex disorders in India. We have also identified in a quantitative way the factors of greatest importance to parents while deciding sex of rearing. These results have direct utility in the management of patients with intersex disorders in India and other similar cultures.

Retroperitoneal teratomas in children.

OBJECTIVE: This paper aims to highlight the clinical features, investigations and treatment of retroperitoneal teratomas condition. METHODS: 12 patients (8 females and 4 males, age range-2 months to 14 yrs) of retroperitoneal teratoma admitted to the department of Pediatric Surgery, King George Medical University, Lucknow between 1980 and 2004 were studied. Investigations included hematology, plain X-ray of the abdomen, intravenous urography, ultrasound, computerised tomography (CT) of the abdomen (after 1990, 8 patients) and serum alpha-fetoprotein assay (after 1991, 6 patients, preoperatively). All patients underwent surgery. Serum alpha-fetoprotein assay was used during follow-up to detect recurrence. RESULTS: Majority of the tumors were left pararenal in location. In two patients there was bilateral involvement. In all except one, the tumor could be excised easily preserving the kidneys. In one child with a massive cystic tumor with bilateral involvement, the tumor was marsupialised in the first stage and excised subsequently. One child died postoperatively, the other 11 children are well and there has been no tumor recurrence on follow-up. CONCLUSIONS: Retroperitoneal teratomas are uncommon lesions in children mostly arising in close relation to the kidneys. The majority are benign but complete excision is necessary for cure. Even large tumors with bilateral involvement of the retroperitoneum can be excised while preserving adjacent organs. Serum alpha-fetoprotein assay is a reliable method of detecting recurrence.

Papillary renal cell carcinoma in a child.

AIM: To report a case of papillary renal cell carcinoma in a pediatric patient. METHOD: A seven-year-old boy complained of hematuria for one year. CT scan showed a mass in the middle zone of the right kidney. Radical nephrectomy was performed without incident. RESULT: Histology of the specimen showed papillary renal cell carcinoma. As the tumor was stage one, no chemotherapy was offered. At follow-up after 6 months the child was without recurrence or metastases. CONCLUSIONS: Papillary renal cell carcinoma is an uncommon kidney tumor in children and in its early stages it can be cured by nephrectomy alone.

Nocardiosis in patients of chronic idiopathic thrombocytopenic purpura on steroids.

We present two cases of chronic idiopathic thrombocytopenic purpura (ITP) on prolonged steroid therapy who developed subcutaneous and brain abscesses due to Nocardia asteroides. The special diagnostic and therapeutic challenges encountered in the patients because of severe thrombocytopenia are being highlighted.

Eosinophilic fasciitis.

Eosinophilic fasciitis presents with skin thickening involving the extremities, and sparing the hands. Raynaud's phenomenon is usually absent. It can be precipitated by undue exertion and is characterized by eosinophilia and infiltration of skin and subcutaneous tissue by mixed inflammatory infiltrate including eosinophils. We present a young man who developed eosinophilic fasciitis following severe gymnastic activity.

Low levels of anti-histone antibodies in north Indian children with juvenile rheumatoid arthritis.

BACKGROUND & OBJECTIVES: Early onset pauciarticular disease with uveitis is distinctly uncommon in Indian children with juvenile rheumatoid arthritis (JRA). The occurrence of anti-histone antibodies (AHA) in serum is strongly associated with presence of uveitis. There is a paucity of information from India on the levels of AHA in patients of JRA. In this study, an attempt was made to evaluate the levels of IgG and IgM antibodies to histones in children with JRA in north India. METHODS: Serum samples of 148 children with JRA (84 boys, 64 girls) were collected. Clinical details including onset, symptoms and course of the disease in each patient were recorded. Detailed eye examination including slit lamp examination was done in all patients at presentation and yearly thereafter to rule out uveitis. The presence of antihistone IgG and IgM antibodies was studied by ELISA. Antinuclear antibodies (ANA) were measured by indirect immunofluorescence using HEP-2 cells as substrate at a screening dilution of 1:40. RESULTS: Of the 148 children, 54 had pauciarticular (12 early onset and 42 late onset), 64 polyarticular and 30 systemic onset disease respectively. ANA were present in two children. AHA were raised in 15 (10%) children, of whom 10 had IgM antibodies, 3 had IgG and 2 had both isotypes. None of the children with early onset pauciarticular disease had uveitis, ANA or AHA. INTERPRETATION & CONCLUSION: The low occurrence of AHA and uveitis in our subset of patients with JRA is in contrast to that reported from Western countries. The low occurrence is unlikely due to technical reasons as the antigen that has been used consistently showed significant binding to serum from patients with systemic lupus erythematosus (SLE). This is in accordance with the rarity of early onset pauciarticular disease and chronic uveitis in these patients. More studies from other parts of the country are required to validate this observation.

Sacrococcygeal teratoma.

This retrospective study details our experience regarding 72 patients with sacrococcygeal teratoma treated over a period of 17 years. The sex incidence was nearly equal, but there was a high proportion of Altmann type IV tumors. A preliminary colostomy before combined abdominosacral excision of large type III and IV lesions reduced morbidity. Sixty-six percent of the patients presented beyond the neonatal period; 14 had been treated elsewhere for bowel/urinary obstruction. Imaging studies included radiography, abdominal ultrasound, computed tomography, and magnetic resonance imaging (after 1995). In 60 patients the tumor was excised via the sacral route, 11 had a preliminary colostomy, and 1 had a vesicostomy. Eight children (5 with malignant lesions) required abdominosacral excision. After 1990, serial estimation of serum alpha-fetoprotein (AFP) was used to monitor tumor recurrence. There were 34 male and 38 female patients (age range 3 days-12 years); 47 had benign tumors, of which 42 were excised through the sacral route. Three patients underwent a preliminary colostomy and abdominosacral excision of the tumor with subsequent colostomy closure. There were 4 deaths in this group; no recurrence was seen in the surviving children with benign tumors. Twenty-five patients had malignant teratomas. In 18 of these the tumor was excised via the sacral route and 5 underwent abdominal-sacral excision. Eight had a preliminary colostomy and chemotherapy followed by excision of the residual tumor and colostomy closure. None of the initial 14 patients with malignant lesions survived beyond 2 years. Of the latter 11 (who received cisplatinum-based chemotherapy), 10 were alive 1 year after surgery. One patient is currently on preoperative chemotherapy and another developed recurrence of the tumor. The overall follow-up ranged from 3 months to 8 years; there has been no complaint of functional neurological deficit in any of the patients. As intrapelvic tumors tend to have a delayed diagnosis, this can be avoided by performing a rectal examination. There should be no recurrence after excision of a benign teratoma. Cisplatinum-based chemotherapy has improved the survival of patients with malignant tumors.

Paediatric gastric teratoma.

AIM: This paper reviews our experience with patients presenting with gastric teratoma treated over an eighteen-year period. This rare paediatric tumour has an excellent prognosis after curative resection. METHODS: There were seven patients, all males. Investigations included a haematological profile, plain radiography and ultrasound of the abdomen. All patients were operated and the specimens subjected to histopathological examination. RESULTS: The age at presentation ranged from 1 month to 7 years. Two patients presented with melaena and the rest with progressively increasing abdominal mass. Our oldest patient had a massive tumour occupying nearly the whole abdomen. None of the children had metastases and none of the lesions had invaded adjacent structures. The tumours could be completely resected and all patients had an uneventful recovery. Histology of the tumours showed mature elements arising from the three germ layers in 6 children, one child had an immature teratoma. The average follow-up was 3 years (range 2 - 6 years). None of the patients has had a recurrence or any other problem. CONCLUSIONS: Gastric teratoma is a rare tumour but amenable to curative resection with an excellent prognosis.

Respuesta de la vacuna Mycobacterium habana en pacientes con lepra lepromatosa y sus convivientes. Un estudio piloto

Se llevó a cabo una vacunación a dosis única con Mycobacterium habana, con 31 casos de lepra lepromatosa que recibieron 1-2 mg (1'5 mg = 6'27 x 108 bacilos) y 36 convivientes con 1'5, 2'0, 2'5 mg de vacuna intradérmica. La duración del estudio fue de 18 semanas. La vacunación indujo conversión a la lepromina en el 100% en contactos lepromina positivos, estable durante las 15 semanas de duración del seguimiento. El incremento máximo en la reactividad a la lepromina se obtuvo con 1'5 mg de vacuna , probablemente la dosis máxima admisible. Además, la vacunación de los no BCG previa reveló un valor promedio superior de incremento de la lepromina. Los cambios en la vacunación local incluyen induración, ulceración, picazón dolorosa y linfodenopatías periféricas, todas remitieron espontáneamente a las 15 semanas. Los efectos secundarios sistemáticos resultaron ser pyrexia, ENL, ictericia con una frecuencia no superior a los observado con otras vacunas. Estos efectos secundarios sistémicos eran facilmente controlables y no se acompañaban de deterioro ocular o nervioso. Las investigaciones analíticas tipo perfil de seguridad, revelaron un incremento en el valor promedio de la cantidad de (hemoglobina) Hb%, RBC (hematíes) y PCV en los convivientes y de PCV en pacientes lepromatosos, pos-vacunación. También se observaron alteraciones en las funciones hepáticas en los pacientes con lepra lepromatosa. Por lo tanto, M. habana parece útil en estimular CMI específica contra M. leprae como evidencia la reactividad incrementada a la lepromina.

Response of Mycobacterium habana vaccine in patients with lepromatous leprosy and their household contacts. A pilot clinical study.

Single dose vaccination was carried out with Mycobacterium habana vaccine, 31 lepromatous leprosy cases receiving 1.5 mg (1.5 mg = 6.27 x 10(8) bacilli) and 36 household contacts randomly receiving 1.5, 2.0, 2.5 mg vaccine intradermally. Duration of study was 18 weeks. Vaccination induced lepromin conversion in 100% of lepromatous leprosy cases and lepromin negative household contacts and augmentation of lepromin reactivity in 100% of lepromin positive household contacts, which was stable for the 15 weeks duration of follow-up. The maximum augmentation in lepromin reactivity was obtained with 1.5 mg of vaccine, which is probably the supramaximal dose. Overall, post-vaccination, those without prior BCG vaccination scars showed higher mean values of lepromin augmentation. Local vaccination site changes included induration, ulceration, itching, pain and uncomplicated regional lymphadenopathy, all of which remitted spontaneously by 15 weeks. Systemic side-effects noted were pyrexia, ENL and jaundice, and were seen with no greater frequency than that reported in other vaccine trials. Overall, systemic side-effects were easily controlled and were not accompanied by clinically detectable nerve or ocular damage. The safety profile investigations revealed an increase in the mean values of Hb%, RBC count and PCV in household contacts and of PCV in lepromatous patients, post-vaccination. Alterations in the liver function tests were also observed in patients of lepromatous leprosy. Thus, M. habana vaccine appears to be useful in stimulating specific CMI against M. leprae as evidenced by increased lepromin reactivity.

Ovarian adenocarcinoma in premenarchal girls.

Ovarian neoplasms are unusual in the paediatric age group; the majority of them are of germ cell origin. Malignant epithelial tumours of the ovary occur infrequently in adolescent girls. Ovarian carcinoma in particular is extremely rare before puberty. The authors describe 3 cases of adenocarcinoma of the ovary in premenarchal girls and highlight the unique characteristics of this tumour in this age group.