Evaluation of ovarian reserve in young females with non-iatrogenic ovarian insufficiency to establish criteria for ovarian tissue cryopreservation.

RESEARCH QUESTION: Can ovarian reserve parameters predict the outcome of ovarian tissue cryopreservation (OTCP) in patients ≤18 years with non-iatrogenic premature ovarian insufficiency (POI)? DESIGN: Retrospective cohort analysis carried out in a single tertiary hospital between August 2010 and January 2020. Thirty-seven patients ≤18 years with non-iatrogenic POI (27 with Turner syndrome, six with POI of unknown aetiology, three with galactosemia and one with blepharophimosis, ptosis, epicanthus inversus syndrome) were included. Three parameters were used to evaluate ovarian reserve: anti-Müllerian hormone (AMH), follicle-stimulating hormone (FSH) and transabdominal antral follicle count. Fertility preservation (most commonly OTCP) was offered if ovarian reserve was diminished and one or more parameters was positive. Follicles were counted in ovarian samples obtained at the time of OTCP. RESULTS: Ovarian reserve was diminished in 34 patients and 19 of them had one or more positive parameter. Fourteen (11 aged ≥12 years and 3 aged <12) underwent OTCP, one (14 years old) underwent ovarian stimulation and oocyte cryopreservation and four declined fertility preservation. Follicles were detected in 11 of 14 patients who underwent OTCP with one or more positive parameters (79%), and in all those (100%) who had two or three positive parameters. The median number of follicles was 27 (range 5-64) and 48 (range 21-75) in patients ≥12 years and those <12 years, respectively. CONCLUSION: This study shows that if OTCP is performed in patients with one or more positive parameters of ovarian activity, a 79% positive predictive value is achieved for the detection of follicles. The incorporation of this criterion for OTCP will minimize the risk of harvesting ovarian tissue with a low number of follicles.

Oesophageal atresia: sonographic signs may prenatally predict surgical complexity.

OBJECTIVE: Oesophageal atresia (OA) is a major anomaly of varying severity. The complexity of surgical correction highly depends on the gap length of missing oesophagus and the presence of a distal fistula. The aim of this study was to identify antenatal sonographic findings associated with presence of a distal fistula and type of surgical repair METHODS: Prenatal medical records of neonates postnatally diagnosed with OA were reviewed. Sonographic signs of OA (small/absent stomach, polyhydramnios, oesophageal pouch) and the trimester at sign detection were recorded and compared between (1) OA with and without a distal fistula and (2) early one-step versus delayed two-step anastomosis. Multivariate analysis was performed. RESULTS: Overall, 80 cases of OA were included. Absence of a distal fistula was significantly associated with higher rates of small/absent stomach (100% vs 28.6%, P<0.0001), oesophageal pouch (100% vs 24.3%, P<0.0001) and severe polyhydramnios (66.7% vs 22.9%, P=0.006), compared with OA with a distal fistula.Cases requiring a delayed two-step repair had higher rates of small/absent stomach (84.2% vs 16.7%, P>0.0001), severe polyhydramnios (47.4% vs 16.7%, P=0.008) and oesophageal pouch (73.7% vs 18.5%, P<0.0001), compared with those corrected in an early one-step anastomosis.Multivariate logistic regression found small/absent stomach and pouch to be significantly and independently associated with a delayed two-step anastomosis. CONCLUSION: OA without a distal fistula is associated with higher rates of prenatal sonographic signs. Both small/absent stomach and a pouch are independently associated with a delayed two-step anastomosis. These findings may help improve antenatal parental counselling regarding the anticipated surgical repair.

Huge Mediastinal Lipoblastoma in a Nine Year-old Boy Successfully Removed Surgically.

A nine year-old boy presented with symptoms of dyspnoea. The chest radiograph and computed tomography scan revealed a large mediastinal tumour. A decision to operate on the patient was made. A huge 2.45 kg mediastinal lipoblastoma was successfully removed from the boy's chest. At three years no evidence of recurrence was found.

The hepatorenal reflex contributes to the induction of oliguria during pneumoperitoneum in the rat.

BACKGROUND: Hepatic blood flow is known to decrease during pneumoperitoneum. Studies have shown that such changes affect kidney urinary output through the sympathetic pathway known as the hepatorenal reflex. This study investigated the potential role of the hepatorenal reflex in pneumoperitoneum-induced oliguria. The authors hypothesized that oliguria detectable during pneumoperitoneum is caused by activation of the hepatorenal reflex. METHODS: Denervation of the sympathetic nervous structure was performed in 15 rats by applying 1 ml of 90 % aqueous phenol solution circumferentially to the portal vein and vena cava area at their entrance to the liver. The same was applied to only the peritoneum in 15 nondenervated rats. After 2 weeks, the rats were divided into three subgroups (5 rats per subgroup) that were exposed respectively to carbon dioxide-induced pneumoperitoneum at 0, 10, and 15 mmHg for 2 h. Statistical analysis was performed using Student's t test and analyses of variance. RESULTS: Denervation did not affect the preinsufflation parameters. The denervated and the nondenervated 0-mmHg subgroups presented with similar parameters. The postinsufflation mean urine output was significantly lower in the nondenervated than in the denervated 10- and 15-mmHg subgroups (p = 0.0097). The denervated rats had a final creatinine clearance 29 % lower than the preinsufflation value (p = 0.83), whereas the nondenervated animals presented a 79 % drop in creatinine clearance (p = 0.02). CONCLUSION: The study findings indicate that the hepatorenal reflex plays an important role in the pathophysiology of oliguria that occurs during pneumoperitoneum in the rat.

[Laparoscopic sleeve gastrectomy (LSG) in adolescents with morbid obesity].

BACKGROUND: Morbid obesity has become a significant health problem for the pediatric population. The medical impact of obesity in youth is determinant. Conservative methods for weight loss are disappointing and therefore, bariatric surgery should be considered. AIMS: To present the experience of laparoscopic sleeve gastrectomy in adolescents with morbid obesity, treated by the pediatric multi-disciplinary obesity clinic. METHODS: All patients (n=7, all female) participated in a weight loss program for at least 6 months without success. At referral, the mean age was 16.2 years (range 13.8 - 18 years), mean body mass index (BMI in kg/m2) was 44.4 (range 38.9-55.2). All suffered from various co-morbidities of obesity: type 2 diabetes, insulin treated (n=1), hypertension (n=5), fatty liver (n=2), obstructive sleep apnea (n=2) and pseudotumor cerebri (n=1). RESULTS: There were no intra- or postoperative complications. After a mean follow-up of 15.1 months (range 5-25 months), all patients but one had reduced BMI (mean BMI of 32.55). In all subjects who lost weight, remission or improvement of the co-morbidities was noted. CONCLUSION: In this study, with a mean follow-up of 15.1 months, LSG was proven to be a safe and effective option of bariatric surgery in adolescents, resulting in a significant weight loss and remission or improvement of co-morbidities. We suggest that LSG might be considered as a single intervention for morbid obesity in adolescents. Long-term studies are needed to evaluate the efficacy of LSG and other bariatric surgeries in adolescents.

A foregut cyst presenting as an umbilical mass in a newborn.

We described for the first time a foregut cyst presenting as an umbilical mass in a female infant. The diagnosis was made microscopically. This case illustrates, based on varied histologic findings, the complexity in classifying such cysts, in view of existing terminology.

Retrospective study of the surgical management and outcome of nonrhabdomyosarcoma soft tissue sarcomas of the groin and axilla in children.

PURPOSE: The incidence of pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) of the groin and axilla is unknown, and the optimal surgical approach to these patients is unclear. METHODS: We conducted a retrospective study of patients treated at St Jude Children's Research Hospital from January 1962 to March 2007 for NRSTSs of the groin and axilla. Demographic variables, tumor pathology, clinical management, and outcome were reviewed. RESULTS: Of the 300 patients treated for NRSTSs, only 10 had tumors of the axilla or groin (6 of whom had synovial sarcoma). Surgical interventions included wide resection of the tumor (n = 7), marginal resection (n = 1), subtotal resection (n = 1), and biopsy only (n = 1). Six patients underwent lymph node sampling; all were negative for tumor. Short- and long-term surgical complications were rare. Four patients received adjuvant chemotherapy (n = 3) and/or radiotherapy (n = 2). At a median follow-up of 8.5 years, 7 of the 10 were surviving free of disease. Two of these patients died of tumor progression (1 with metastases at diagnosis and 1 with an unresectable tumor at diagnosis), and one patient who was free of NRSTS died of secondary breast carcinoma. CONCLUSIONS: Pediatric NRSTSs of the axilla and groin are rare, but outcomes are similar to those of other patients with NRSTS. Wide local excision of the tumor with preservation of good limb function should be the surgical goal and may be sufficient therapy in some cases.

Should contralateral exploratory thoracotomy be advocated for children with osteosarcoma and early unilateral pulmonary metastases?

PURPOSE: Children presenting with osteosarcoma and pulmonary metastases have poor survival rates. The standard approach to treating unilateral metastases is ipsilateral thoracotomy with complete resection of the metastases whenever possible. We analyzed whether contralateral exploratory thoracotomy is beneficial in these patients. METHODS: We reviewed the records of all osteosarcoma patients presenting with or developing early pulmonary metastases (within 24 months of diagnosis) at the St Jude Children's Research Hospital (Memphis, Tenn) between June 1980 and September 2005. Demographics, imaging results, treatment protocols, surgical procedures, and recurrence, survival, and timeline data were assessed. RESULTS: Of the 109 patients, initial pulmonary involvement was radiographically identified as unilateral in 81 (74%) and bilateral in 28 (26%) patients. Of the patients presenting with unilateral pulmonary metastases, 2 years later, 13 (16%) had recurrence in the ipsilateral and 19 (23%) in the contralateral lung. In 2 (2.4%) patients, metastases recurred bilaterally. There was no evidence of statistically significant difference between the incidence of recurrence in the ipsilateral and contralateral lung (P = .18). CONCLUSIONS: Children with osteosarcoma and unilateral pulmonary metastases had similar incidence of recurrence in the ipsilateral and contralateral lung. Their survival rates were also not significantly different. Therefore, contralateral exploratory thoracotomy in patients presenting with unilateral pulmonary metastases will probably not improve survival.

Twelve years of minimally invasive surgery in children and adolescents: a single center experience.

BACKGROUND/PURPOSE: Minimally invasive surgery plays an important role in the daily practice of pediatric surgeons. However, there have been few reports of large series of patients. The aim of this paper is to report our experience over the past 12 years with a broad range of pediatric laparoscopic procedures. METHODS: We performed a retrospective analysis of 1084 minimally invasive procedures conducted in the department of pediatric surgery from June 1992 to March 2004. RESULTS: The mean age of the patients was 14 years (range, 1-19). Seven hundred forty-six operations (69%) were thoracoscopic sympathectomy for hyperhydrosis. Other common operations were high ligation of spermatic vessels (n=98), cholecystectomy (n=65), exploration of nonpalpable testes (n=53), appendectomy (n=55), female adnexal surgery (n=24), and splenectomy (n=13). Thirty miscellaneous procedures included Nissen fundoplication, liver biopsies, diagnostic laparoscopy, Morgani and Bochdalek hernia repair, Swenson procedures, V-P shunt placement, splenic and hepatic cyst removal, thorcoscopy for pleural empyema, treatment of gastric ulcer perforation, and nephrectomy. We reviewed the indications for surgery, the surgical technique, and the outcome for each procedure. All patients in this series were well at follow-up and there was no long-term morbidity. CONCLUSION: Although laparoscopic procedures have gained an integral place in pediatric surgery and are relatively safe, advanced laparoscopic procedures should be developed, practiced and evaluated in dedicated surgical units to ensure a broad base of experience on which to base future decisions and guidelines.