Laparoscopy versus ultrasonography for the evaluation of Mullerian structures in children with complex disorders of sex development.

PURPOSE: The diagnosis of children with disorders of sex development (DSD) requires a karyotype, different biochemical and radiological investigations in the context of a multidisciplinary team. The aim of this study was to compare the diagnostic accuracy of laparoscopy (L) versus ultrasonography (US) in the assessment of children with complex DSD. METHODS: We retrospectively examined the theatre database searching for children with DSD who underwent laparoscopic surgery from 1999 to 2011. The medical and radiological records were reviewed. RESULTS: Eighteen patients were identified. Age at diagnosis ranged from birth to 14 years (mean 2.5 years). There were seven patients with 46XY dysgenetic testicular DSD (4 mosaic Turner, 3 mixed gonadal dysgenesis), seven patients with 46XY non-dysgenetic testicular DSD (4 persistent Mullerian duct syndrome, 2 complete androgen insensitivity syndrome, one unknown), two patients with ovotesticular DSD, one patient with 46XX DSD (congenital adrenal hyperplasia) and one patient with 46XY DSD complete sex reversal. Fifteen underwent ultrasonography prior to laparoscopy. Both modalities identified Mullerian structures in seven (47 %) patients, in one (7 %) patient US and L confirmed the absence of Mullerian structures, while in six (40 %) patients there was discordance, with US failing to visualize pelvic Mullerian structures. In the last patient with 46XY non-dysgenetic testicular DSD, the rectum was thought to be a dilated uterus on ultrasonography. CONCLUSIONS: Pelvic ultrasonography failed to identify Mullerian structures in 40 % of patients with complex DSD. On the contrary, laparoscopy allowed excellent visualization of pelvic structures and gonads in children with complex DSD.

Comparative evaluation of laparoscopic versus open nephrectomy in children.

BACKGROUND AND AIMS: To assess the benefits of laparoscopic nephrectomy in children as compared to open surgery and to ascertain whether or not the retroperitoneal technique offers any surgical advantage over the transperitoneal approach. METHODS AND RESULTS: This study gathered retrospective data on a consecutive series of 51 paediatric patients comparing; operative time, length of hospital stay and analgesic requirement. Results show that when laparoscopic surgery is compared with the open technique it took 25 mins longer to perform, there was a shorter post operative stay by 2.5 days, and a 50% reduction in morphine use. Patients who had the retroperitoneal technique were discharged from hospital within one day. CONCLUSIONS: Compared to previous studies, the findings in this study suggest that there are distinct benefits in laparoscopy for children. There is some evidence in favour of the retroperitoneal technique. Patients are in hospital for shorter periods and need less analgesia.

Physiological effects of pneumoperitoneum in laparoscopic pyloromyotomy.

A retrospective analysis comparing intraoperative physiological variables during laparoscopic and open pyloromyotomies was undertaken during an 18-month period at our institution. Fifty cases were examined (22 laparocopic and 28 open). Operative time, temperature change, end-tidal CO2, heart rate, and blood pressure were examined and compared. No significant differences were found in operative time, temperature change, heart rate, and blood pressure. As anticipated, in laparoscopic procedures there was a statistically significant increase in end-tidal CO2, although none of the end-tidal CO2 values rose above 6 kPa. It is concluded that laparoscopic pyloromyotomies undertaken in small infants with insufflation pressures of 8-10 mm Hg are without significant adverse physiological effects and are no slower to perform than open procedures.

Is the laparoscopic Palomo procedure for pediatric varicocele safe and effective? Nine years of unicentric experience.

BACKGROUND: Varicocele is a rare disorder in children that can lead to testicular atrophy and infertility; therefore, radical treatment is frequently required. Whatever treatment is chosen, postoperative complications are fairly common (hydrocele, recurrence, persistence, and testicular atrophy). Laparoscopic varicocelectomy (the laparoscopic Palomo procedure) is one of the surgical options that has recently gained popularity. The aim of this study is to assess the safety and effectiveness of laparoscopic Palomo varicocelectomy by describing a series of patients operated on during a 9 year period at the Royal Hospital for Sick Children in Edinburgh. METHODS: This is a retrospective unicentric study including patients operated on between June 1995 and June 2004. All patients preoperatively underwent ultrasound scan of the testicles (color Doppler) and the abdomen. Indications for surgery included symptoms, high-grade varicocele (grade II and III), and testicular atrophy. Pneumoperitoneum was created using carbon dioxide insufflation with intraabdominal pressure up to 12 mmHg. Three 5 mm ports were inserted. The first port was inserted just below the umbilicus (telescope) under direct vision, and the others were inserted at the left flank and in the suprapubic region. All the enlarged spermatic and vas vessels were ligated or clipped. Outcomes and possible intraoperative, postoperative, or long-term complications are described. RESULTS: Forty-one patients were included in the study. Ninety percent of symptomatic patients improved significantly postoperatively, and 62% of patients with preoperative testicular atrophy showed postoperative catch-up growth of the involved testis. Nevertheless, hydrocele represents the most frequent postoperative complication in this series of patients. Approximately 15% of the patients required some sort of further surgical intervention (12% because of postoperative hydrocele occurrence). CONCLUSIONS: The laparoscopic Palomo procedure is a safe and effective surgical option for the treatment of pediatric varicocele, although it carries a fairly high risk of postoperative hydrocele. Postoperative hydrocele seems to be related to some sort of lymphatic obstruction, therefore lymphatic sparing procedures that can be accomplished laparoscopically should be reconsidered. Nevertheless, their feasibility and effectiveness need to be more carefully assessed.

European multicenter survey on the laparoscopic treatment of gastroesophageal reflux in patients aged less than 12 months with supraesophageal symptoms.

BACKGROUND: This multicenter survey includes neonates and infants who underwent surgery for primary gastroesophageal reflux (GER) who presented with supraesophageal symptoms of unknown origin with a minimum of 12 months postoperative follow-up. METHODS: A total of 726 patients underwent GER surgery in 10 European Centers in the period 1998-2002. Respiratory symptoms were present in 204 patients (28%); 135 patients (17%) had surgery under 1 year of age, and 46 of them (6.3%) because of respiratory symptoms. Surgery was performed without any previous medical treatment in 10 cases (21%). The type of procedure included 37 complete 360 degrees wraps (80%) (Nissen, 12, and Rossetti, 25) and nine partial wraps (20%) (Thal five, Lortat Jacob one, Toupet one, others two). Gastrostomy was associated in 17 cases (37%) (6 PEG and 11 modified Stamm). No gastric emptying procedures were recorded. RESULTS: No major intraoperative complications were reported. Six patients developed complications (13%) and a redo operation was performed in three (6.5%). Respiratory outcome after antireflux surgery was good in 35 patients (76%) and fair with significantly improved respiratory symptoms in 11 (24%). CONCLUSIONS: This multicenter survey underlines that GER has to be suspected and aggressively treated in infants with difficult-to-treat supraesophageal symptoms, and also in high-risk cases, in order to prevent major complications.

Changing trends in a decade of circumcision in Scotland.

PURPOSE: There has been a move toward conservative management of foreskin problems. The aim of this study was to examine overall trends for circumcision in Scotland to see if there has been a decrease in the number of operations performed. METHODS: Retrospective analysis looking at the number of circumcisions performed each year over a 10-year period (1990 to 2000), in the 0 to 13-year age group. The principal indication for circumcision in each case was ascertained as were the number of preputioplasties performed over the corresponding period. RESULTS: A total of 15,605 circumcisions were performed during the 10-year period; 10,888 (69.8%) for phimosis, 2,724 (17.5%) for nonmedical/religious reasons, and 1993 (12.8%) for all other indications. There was a 33.7% decrease in total number of circumcisions performed between the first and second halves of the study period. The fall in operations performed is almost solely attributable (94.5%) to a reduction in number of procedures carried out for phimosis. There was a corresponding increase in preputioplasties performed over the study period. CONCLUSIONS: There has been a steady decrease in circumcision rates in Scotland. This is in keeping with greater appreciation of pathologic phimosis as distinguished from a healthy nonretractile foreskin, which does not require circumcision.

Risk-adapted treatment for childhood hepatoblastoma. final report of the second study of the International Society of Paediatric Oncology--SIOPEL 2.

SIOPEL 2 was a pilot study designed to test the efficacy and toxicity of two chemotherapy (CT) regimens, one for patients with hepatoblastoma (HB) confined to the liver and involving no more than three hepatic sectors ('standard-risk (SR) HB'), and one for those with HB extending into all four sectors and/or with lung metastases or intra-abdominal extra hepatic spread 'high-risk (HR) HB'. SR-HB patients were treated with four courses of cisplatin (CDDP), at a dose of 80 mg/m(2) every 14 days, delayed surgery, and then two more similar CDDP courses. HR-HB patients were given CDDP alternating every 14 days with carboplatin (CARBO), 500 mg/m(2), and doxorubicin (DOXO), 60 mg/m(2). Two courses of CARBO/DOXO and one of CDDP were given postoperatively. Between October 1995 and May 1998, 77 SR-HB (10 of whom were actually treated with the HR protocol) and 58 HR-HB patients were registered and all 135 could be evaluated. Response rates for the entire SR-HB and HR-HB groups were 90% (95% CI 80-96%) and 78% (95% CI 65-87%), and resection rates were 97% (95% CI 87-99%) and 67% (95% CI 54-79%) including several children undergoing liver transplantation. For SR-HB patients, 3-year overall and progression-free survivals were 91% (+/-7%) and 89% (+/-7%) and for the HR-HB group 53% (+/-13%) and 48% (+/-13%), respectively. The short-term toxicity of these regimens was acceptable, with no toxic deaths. A treatment strategy based on CDDP monotherapy and surgery thus appears effective in SR-HB but, despite CT intensification, only half of the HR-HB patients are long-term survivors. For SR-HB patients, the efficacy of CDDP monotherapy and the CDDP/DOXO ('PLADO') combination are now being compared in a prospective randomised trial (SIOPEL 3).

Laparoscopic Nissen fundoplication in children: 2-5-year follow-up.

PURPOSE: This study aims to document the current status of a cohort of children who underwent laparoscopic fundoplication at a single centre between 1996 and 1998. METHODS: Parents were contacted and a questionnaire regarding preoperative and current symptoms completed. Case notes were reviewed for results of postoperative investigations. RESULTS: Forty-five laparoscopic Nissen fundoplications were performed. The median age was 5 years. Twenty-eight children were neurologically impaired. Five died of underlying medical problems during follow-up. Two were lost to follow-up, leaving 38 parents interviewed. Median follow-up was 36 months. Twenty-five children were asymptomatic, and 13 reported upper gastrointestinal symptoms. In ten, symptoms were less severe than preoperatively. Nine of the 13 children were taking acid suppressing drugs. No children reported problems with dysphagia. Twelve of the 13 symptomatic children had investigations for recurrent reflux. In no case was there evidence of reflux or wrap disruption. One of the children who died had been demonstrated to have recurrent reflux on barium swallow. CONCLUSIONS: Sixty-six percent of patients reported complete relief, and a further 26% reported considerable improvement of their symptoms. There was a high degree of parental satisfaction with the outcome of the operation. The results suggest that laparoscopic fundoplication is a durable procedure with documented recurrent reflux in only 2% of children at a median follow-up of 3 years.

Congenital colonic atresia: should primary anastomosis always be the goal?

We report 4 cases of the rare condition, congenital colonic atresia, presenting over 9 years. Two patients had dilated loops of bowel noted on routine ante-natal ultrasound. Three had primary anastomosis for lesions in transverse or distal descending colon and one had a staged procedure with colostomy formation. All had type III atresia. One patient had an early leak following primary colo-colic anastomosis for atresia extending from the hepatic flexure to the sigmoid colon. If it is desired to preserve the proximal colon in a right sided lesion with significant loss of colonic length then primary anastomosis may not be safe and we would advocate a staged procedure. Otherwise we would support current moves towards primary anastomosis in this condition. We also urge early investigation in all patients who exhibit intestinal dilatation on antenatal ultrasound.

Urinary ascites in infancy: varied etiologies.

Three cases of urinary ascites are presented, each with a different underlying aetiology. The age and modes of presentation also varied and management strategies were accordingly tailored to each patient's clinical requirements. All 3 patients survived and subsequently were discharged with good renal function. Although a rare condition, infants with urinary ascites can present as clinical emergencies in need of prompt resuscitation with subsequent drainage of the urine and decompression of the urinary tract. The ultimate management regime will vary and depend upon site of urinary extravasation and underlying aetiology.

An unusual presentation of neonatal adrenal hemorrhage.

Case report of a neonate presenting within 1 h of birth with right side scrotal swelling and a dusky skin discoloration thought clinically to be a hydrocele or testicular torsion. Ultrasound examination demonstrated bilateral hydroceles, more prominent on the right, scrotal oedema and a perinephric loculated fluid collection secondary to right adrenal hemorrhage. We review the ultrasound appearance of the normal and haemorrhagic neonatal adrenal and discuss the differential diagnosis of both neonatal adrenal lesions and scrotal swellings. The role and benefits of ultrasound in the primary diagnosis and management of neonatal adrenal hemorrhage is emphasized.

Hepatocellular carcinoma in children: results of the first prospective study of the International Society of Pediatric Oncology group.

PURPOSE: To improve survival and reduce operative morbidity and mortality in children with primary epithelial liver tumors by using preoperative chemotherapy, as well as to collect information on the epidemiology, natural history, and prognostic factors. PATIENTS AND METHODS: Forty children with hepatocellular carcinoma (HCC) were registered onto the Group for Epithelial Liver Tumors International Society of Pediatric Oncology's first study from January 1990 to February 1994. The outcome could be analyzed in 39 of those patients. Disease was often advanced at the time of diagnosis; metastases were identified in 31% of the children and extrahepatic tumor extension, vascular invasion, or both in 39%. Multifocal tumors were common (56%). Thirty-three percent of tumors were associated with hepatic cirrhosis. All but two patients received preoperative chemotherapy (cisplatin and doxorubicin). RESULTS: Partial response was observed in 18 (49%) of 37 patients; there was no response or progression in the remainder. Complete tumor resection was achieved in 14 patients (36%). Twenty patients (51%) never became operable. Overall survival at 5 years was 28%, and event-free survival was 17%. Most deaths resulted from tumor progression (26 of 28). Presence of metastases and pretreatment extent of disease system grouping at diagnosis had an adverse influence on overall survival in multivariate analysis. CONCLUSION: Survival for pediatric HCC patients is significantly inferior to that for children with hepatoblastoma. Complete tumor excision remains the only realistic chance of cure, although it is often prevented by advanced disease. The presence of metastases is the most potent predictor of poor prognosis. A prospective worldwide cooperation in the field of pediatric HCC should be encouraged to look for novel therapeutic concepts.

An audit of post-pyloromyotomy feeding regimens.

A retrospective audit was carried out to assess patient outcome in relation to the timing of feeding after pyloromyotomy for hypertrophic pyloric stenosis. Fifty-two patients received the first feed within 8 hours of surgery and 49 patients were fed between 13 and 20 hours post-operatively. Seventy-seven per cent of patients in the early group vomited compared to 53% in the late group. Sixty-one per cent of patients in the early group produced large vomits compared to 29% in the late feeding group. However, time to establish feeding and post-operative stay were comparable in both groups. We recommend feeding to be delayed for 12 hours after pyloromyotomy unless the patient is distressed because of hunger in which case the parents are warned of the increased risk of vomiting.

Cholecystokinin administration in congenital diaphragmatic hernia--a case report and review of the literature.

Deterioration in the respiratory function of a newborn infant with a repaired diaphragmatic hernia and respiratory insufficiency followed administration of cholecystokinin for cholestatic jaundice. The possible mode of action is discussed and a vasoactive/bronchoactive effect is proposed.

Totally implantable central venous access devices for paediatric oncology patients.

BACKGROUND: Totally implantable central venous access devices (ports) have been available for over 10 years but have not achieved widespread use in paediatric oncology patients. We reviewed our experience with these devices over 9 years to assess their safety and acceptability. PROCEDURE: We conducted a retrospective review of insertion technique and reasons for removal of all ports placed in paediatric oncology patients in this hospital between 1989 and 1996, with follow-up until 1998. Acceptability of both ports and external catheters was assessed by a questionnaire in a subgroup of families attending the oncology clinic. RESULTS: One hundred forty-nine ports were inserted during the study period. The median catheter life was 399 days (4-1,406), with a total of 69,342 catheter days. Sixty-nine percent of ports were removed electively at the end of treatment; 8% required removal because of infection and 5% because of blockage. No ports were accidentally dislodged or damaged. Children experienced significantly less restriction of activity with a port compared to an external catheter and greatly preferred the cosmetic appearance. The need for needle insertion to access the port was not seen as a disadvantage by most families. CONCLUSIONS: Ports can provide satisfactory central venous access for the majority of paediatric oncology patients, with a low risk of line-related complications and a high degree of acceptability to children and their parents.

Real and apparent mortality from congenital diaphragmatic hernia.

Some 50 cases of congenital diaphragmatic hernia (CDH) born in the authors' regional referral area over the 14 years from 1980 to 1993 were reviewed, contrasting 7 years when management included preoperative ventilatory stabilization with the preceding 7 when urgent surgery was performed. Six children experienced no respiratory distress and suffered no mortality. For infants with respiratory distress in the first 6 h of life, ventilatory stabilization improved survival rates of those who reached the surgical centre from 45 per cent between 1980 and 1986 to 59 per cent between 1987 and 1993. A larger proportion of the total number of patients, however, continued to die without reaching the surgical centre. The improvement in survival rate based on the true incidence of CDH was from 28 per cent in the first period to 38 per cent in the second. The apparent poor survival rate of patients born in central obstetric units compared with those born in peripheral units (37 versus 75 per cent) can be attributed to patient selection; a larger number of children born in central units were transferred for surgery (70 versus 57 per cent). There is no evidence that paediatricians have altered their referral practice to include prolonged ventilation outside the surgical unit since delayed surgery was advocated. Assessment of the impact of altering the management of CDH cannot be made without knowing the number of patients who die before transfer to a neonatal surgical unit. Any serious attempt to reduce the mortality rate of CDH must be directed to neonates who are not presently referred to the surgical service.

Extrarenal rhabdoid tumour presenting as a congenital subcutaneous mass.

Extrarenal malignant rhabdoid tumour is a rare neoplasm of childhood. A congenital example has only been reported once previously, presenting as a facial mass. We report a second congenital case with the tumour over the right shoulder in which postoperative investigations revealed multiple hepatic metastases proving fatal at 51 days, with adjuvent therapy felt inappropriate.