Massive ovarian edema in a prepubertal girl without signs of virilization or early puberty.

Massive ovarian edema is a rare cause of ovarian enlargement in young women. This article describes the case in a nine-year-old girl without signs of virilization or early puberty. The patient underwent laparotomy and a right oophorectomy was performed. The final pathology was massive ovarian edema. Awareness of this rare and benign lesion in young women and pediatric patients may allow a conservative management avoiding oophorectomy in some cases.

Left pneumonectomy and expandable prosthesis placement for pleuropulmonary blastoma in a infant: one year follow-up.

Pneumonectomy is a rare procedure in pediatric age. In contrast to adults, children that undergo this intervention are prone to postoperative mediastinal shift, which leads to bronchial stretching resulting in severe respiratory failure. This postpneumonectomy syndrome can be corrected by inserting a prosthesis in the empty side of the chest. The authors present the case of a 9-month-old infant affected with a pleuropulmonary blastoma who underwent left pneumonectomy and prosthesis placement in the same operation.

Necessity for surgery in children with gastrooesophageal reflux and supraoesophageal symptoms.

BACKGROUND/PURPOSE: The majority of gastrooesophageal reflux (GER) manifestations in children are supraoesophageal, and "spitting/posseting" is "the tip of the iceberg" because most reflux episodes are not regurgitated. Aim of the present study was to prospectively evaluate the incidence of gastrooesophageal reflux and the incidence of antireflux surgery in patients with difficult-to-treat respiratory symptoms. PATIENTS AND METHODS: Five hundred and ninety-five children with difficult-to-treat respiratory symptoms were prospectively enrolled in a blind study looking for the correlation between clinical presentation (asthma or non-asthma), oesophageal pH monitoring, X-ray barium meal, broncho-alveolar lavage, necessity for surgery, and outcome. RESULTS: pH monitoring was anomalous in 47% of patients with asthma (group A) and in 43% of those who did not have asthma as main symptom (group B). Overall, 48 patients finally underwent anti-reflux surgery (8%) as anti-reflux medical treatment did not ensure stable benefits. No major surgical complications were experienced. Postoperatively, respiratory symptoms improved strongly (Visick 1) in 69% of cases, moderately (Visick 2) in 27%, while clinical worsening (Visick 4) was observed in 4%. CONCLUSIONS: The results of this study stress the importance of symptoms, clinical response to anti-reflux medical treatment and broncho-alveolar lavage compared to classical pH parameters in the decision-making process for patients with difficult-to-treat supraoesophageal symptoms. To date no single tool alone has proved to be diagnostic in these patients. Fundoplication is recommended only when a relationship between supraoesophageal symptoms and gastrooesophageal reflux is strongly suspected.

Anesthesiologic aspects of laparoscopic fundoplication for gastroesophageal reflux in children with chronic respiratory and gastroenterological symptoms.

INTRODUCTION: During the past three decades laparoscopy has significantly improved. As fundoplication extensively benefits by the great advantages of the minimally invasive approach, many surgeons chose laparoscopic fundoplication for the treatment of gastroesophageal reflux in adults and children as well. Pneumothorax, cardiovascular collapse, hypoxia, and hypercarbia are some of the anesthesiologist's principal fears during carbon dioxide insufflation. Thus, monitoring cardiovascular and respiratory status is mandatory to early detect any complication and to maintain a proper balance during pneumoperitoneum. MATERIALS AND METHODS: At Gaslini Children's Hospital we performed a prospective nonrandomized study aimed at describing the main cardiorespiratory changes produced by pneumoperitoneum in 33 pediatric patients operated on by laparoscopic fundoplication between January 2000 and June 2001. Patients were divided into two groups; namely, group A and group B. Group A included 14 patients with chronic respiratory symptoms, and group B included 19 children who preoperatively mainly emphasized gastrointestinal symptoms. We monitored intraoperative cardiorespiratory status, timed length of surgery, and described intraoperative complications. RESULTS: No significant cardiovascular changes occurred during carbon dioxide insufflation. Partial oxygen saturation remained still in all the patients. End tidal CO2, meanly higher in group A children, increased in all the patients after pneumoperitoneum creation, but never exceeded 45 mmHg. Similarly, peak inspiratory pressure increased in all the patients, but was always maintained within acceptable values. Finally, group B patients required a harder and slower surgery, whose length seems to be negatively influenced by age. No intraoperative complication occurred. CONCLUSIONS: Carbon dioxide insufflation does not impair cardiovascular function, if intraabdominal pressure is maintained lower than 10 mmHg nor does it interfere with gas exchanges. Pneumoperitoneum slightly reduces ventilatory function, mainly in respiratory patients with various degrees of underlying bronchopulmonary impairments, but this effect is easily correctable. Thus, laparoscopic fundoplication is feasible and safe in both respiratory and gastroenterological patients, although surgery is easier and faster if periesophagitis is less evident.

Italian multicenter survey on laparoscopic treatment of gastro-esophageal reflux disease in children.

BACKGROUND: Skepticism is still present today about the laparoscopic treatment of gastro-esophageal reflux (GER) in children. We present the prospective experience and short-term results of eight Italian pediatric surgical units. METHODS: We included all the children with complicated GER, operated after January 1998 by single surgeons from eight different centers. Diagnostic aspects, type of fundoplication, and complications were considered. All the patients were followed for a minimum period of 6 months in order to detect complications or recurrences. RESULTS: 288 children were prospectively included. Mean age was 4.8 years (3 m-14 y). Nissen fundoplication was done in 25%, floppy Nissen in 63%, Toupet in 1.7%, and anterior procedures (Lortat Jacob, Thal) in 10%. Gastrostomy was associated, if neurological impairment or feeding disorders were present. Mean follow-up was 15 months and reoperation was necessary in 3.8% of cases. CONCLUSIONS: This experience underlines that minimal invasive access surgery in children is safe and that the laparoscopic approach is considered in eight centers the golden standard for surgical repair of gastro-esophageal reflux disease maintaining the same indications and techniques of the open approach.

Lichen sclerosus et atrophicus in children with phimosis and hypospadias.

This prospective study was designed to evaluate the incidence of lichen sclerosus et atrophicus (LSA) in a pediatric population with hypospadias and phimosis in order to discuss the indications for circumcision and utilization of preputial skin for urethral surgery. All 115 boys, 55 with congenital phimosis, 45 with acquired phimosis, 13 with hypospadias, and 2 with recurrent chronic balanitis, underwent full-thickness biopsies of the foreskin that were examined by a single pathologist. Of the patients with acquired phimosis, 88% showed inflammatory features in the foreskin; 60% had LSA. Of the patients with congenital phimosis, 82% showed inflammatory disease in the prepuce; 30% had LSA. Of the patients operated upon for hypospadias, 61% showed histologic findings of chronic inflammation of the foreskin and LSA was evident in 15%. The high incidence of LSA in the prepuce of patients with phimosis suggests that circumcision should be performed to correct this disease. The frequent presence of chronic inflammation is a possible cause of stenosis when the foreskin is used to perform a urethroplasty in patients with hypospadias.

Laparoscopic vs open approach for the treatment of gastroesophageal reflux in children.

BACKGROUND: The laparoscopic approach has become increasingly popular for fundoplication over the last few years; however many surgeons are skeptical about its real advantages. METHODS: We conducted a prospective comparative study of children operated on for gastroesophageal reflux (GER). Exclusion criteria included age <1 YEAR AND >14 years, previous surgery on the esophagus or stomach, and neurologic impairment. We compared two groups of patients who met the same inclusion/exclusion criteria. One group was treated via a laparotomic approach between January 1993 and December 1997; the other was treated via a laparoscopic approach between September 1998 and December 2000. A 360 degrees wrap was performed in each group. RESULTS: Group 1 (laparotomic approach) included 17 patients; mean operative time was 100 min and postoperative time was 7 days. Group 2 comprised 49 children operated on via a laparoscopic approach; mean operative time was 78 min and postoperative time was 48 hours. No major complications were encountered in either group. In postoperative period, two patients in group 1 had complications. One had a prolonged bout of gastroplegia, which required nasogastric drainage, and then recovered spontaneously after 20 days; the other had stenosis of the wrap, which required dilation. No relapses occurred during a follow-up of 6 months. Long-term follow-up data are not presented. Comparative analysis of the short-term functional results indicated that there were no differences between the two groups. CONCLUSION: This study confirms that the minimally invasive approach is safe and effective for the treatment of primary gastroesophageal reflux disease in children.

Pleuropulmonary blastoma.

We report a rare case of pleuropulmonary blastoma arising in a 3-year-old boy and involving the middle and upper lobes of the right lung. Radical resection of the mass was achieved by a bilobectomy. Microscopic examination of the histologic sections showed the typical findings of blastemal and mesenchymal areas with focal zones of rhabdomyoblastic and liposarcomatous differentiation. Monthly cycles of chemotherapy consisting of ifosfamide, vincristine and actinomycin D were given for 10 months after surgical resection. Our patient is presently alive and disease-free two years after diagnosis. As complete tumour ablation is essential to prevent local recurrence and allow any chance of survival, the favourable outcome of our patient is probably due to the radical resection of the neoplasm.

Medium-term results after cholecystectomy in patients younger than 10 years.

BACKGROUND: Gallbladder stones are a well-known and widely studied problem in children. Hematological disorders are the most common diseases that can cause cholelithiasis. However, in the last few years, the proportion of children with idiopathic cholelithiasis has increased 50%. Herein, we present a prospective study on laparoscopic cholecystectomy in a selected group of patients aged < 10 years. METHODS: Fifty-eight patients aged < 10 years underwent laparoscopic cholecystectomy for stones in the period 1992-99. The female/male ratio was 1.5, the mean age was 8 years (range, 2-10), and the mean weight was 30 kg. In all patients, parenteral nutrition, fasting state, and prolonged use of antibiotics had been suspended for > or = 6 months, and conservative treatment had been tried for > or = 12 months in the absence of symptoms of stone migration. All the patients were followed up after surgery: clinically at 1,6,12, and 36 months and by ultrasound at 1, 12, and 36 months. Liver function and hematological tests were performed in case of symptoms or if hemolytic disorders were the cause of stones. RESULTS: The mean operative time was 63 min (range, 30-120) in children undergoing cholecystectomy alone and 150 min in children undergoing associated splenectomy. There were no major complications or reoperations. Minor complications included bleeding from accessory cystic artery (n = 3) and insufflation of the omentum (n = 2). One case was converted to an open procedure due to technical problems. All the children were followed up and no complications were observed. CONCLUSION: We consider the laparoscopic approach the gold standard for cholecystectomy in children. This procedure does not have a complication rate any higher than open cholecystectomy, and patient follow-up is as good as that of open surgery. Previous abdominal surgery is not a contraindication to laparoscopy.

Primary anastomosis in esophageal atresia type I without a gap.

This paper reports the case of an infant born with type I esophageal atresia (EA) associated with duodenal atresia (DA). The critical condition of the patient necessitated an exploratory laparotomy, which revealed severe dilatation of the stomach and duodenum. The routine procedure for repairing type I EA is a delayed primary anastomosis after 10 weeks of age because of the long gap between the two esophageal segments. In our case, due to the concomitant DA, the lower pouch was long enough to allow primary neonatal anastomosis. A radiograph taken with a Hegar dilator in the lower segment via the gastrostomy confirmed this suspicion, and the baby underwent a thoracotomy and primary anastomosis between the esophageal pouches. The authors propose the possibility of primary esophageal anastomosis in similar cases.

[Constipation and chronic intestinal pseudoobstruction as a clinical expression of intestinal neuronal dysplasia (IND)]. / Stipsi e pseudo-ostruzione intestinale cronica come espressione clinica di displasia neuronale intestinale (IND).

BACKGROUND: Intestinal neuronal dysplasia (IND) belongs to the group of dysganglionosis. It may occur as part of a syndrome of early chronic constipation, neonatal intestinal occlusion, chronic intestinal pseudo obstruction. The aim of this study was to report the cases examined by the Istituto G. Gaslini in Genoa and to discuss the numerous aspects of this disease which are still unclear. METHODS: 787 children were included in the study and underwent biopsy between 1984 and 1997. Rectal biopsies were obtained by suction or in some cases during surgery and were treated using enzymohistochemical techniques, such as acetylcholinesterase, rapid acetylcholinesterase and alpha-naphthylesterase. RESULTS: 574 children were found to be suffering from innervative alterations: 348 (60.6%) presented isolated Hirschsprung's disease, IND was found in 83 (14.5%), in 8 of the latter in association with other dysganglionosis. IND was accompanied by other diseases in 40 cases (48.2%). Over the past three years (since October 1994) a total of 164 dysganglionosis have been diagnosed, including 55 cases of aganglia. During this period IND was the most frequently observed alteration and affected 61 children. CONCLUSIONS: Rectal biopsy is the essential diagnostic test for the diagnosis of intestinal dysganglionosis. Biopsies are performed in outpatient clinics without sedation, and do not represent an invasive procedure for the young patients. Radiological and manometric examinations cannot provide reliable data for the diagnosis of IND. In our experience, the incidence of IND over the past few years has increased and its diagnosis is essential for correct treatment which is not surgical in the majority of cases. The real incidence of IND and its pathogenesis still need to be clarified.

[Congenital deformities of the chest wall. Surgical treatment]. / Le malformazioni congenite della parete toracica. Trattamento chirurgico.

BACKGROUND AND AIMS: Malformations of the front chest wall are congenital defects that have been reported since the seventeenth century and which include the clinical syndromes of funnel chest, pigeon chest and Poland's syndrome. Although they have been reported for such a long time, their pathogenesis is in many ways still unknown and the theories proposed up until now reveal uncertain and unsatisfactory findings. Attempts to gather precise information regarding their real incidence are equally ineffective given that frequently those cases which are not particularly severe are not referred for specialist care. These malformations usually involve severe psychological problems caused by the deformity and, in more severe cases, may lead to alterations in normal cardiac and respiratory function which are above all evident during intense physical effort. METHODS: The authors critically review the cases of chest malformation corrected by the Department of Pediatric Surgery at the G. Gaslini Institute during the period from 1986 to date. They describe the surgical techniques used, justifying their choice and discussing any postoperative complications. RESULTS: This experience shows that there is a clear preference for less invasive surgery for the correction of funnel chest compared to the technique proposed by Ravitch in which the sternum was totally mobilised following its extensive detachment from the other bone and muscular structures of the thoracic cage, with a high risk of damaging the internal mammary artery. The operation that was felt to be most suitable for correcting this defect was that described by Wesselhoett and De Luca in 1982. It is easier to perform and less invasive: it eliminates the detachment phase by inserting a support in titanium alloy through the sternal frame. Autologous grafts taken from the bottom contralateral ribs to the defect were used to treat Poland's syndrome in line with the technique suggested by Ravitch, thus achieving good stability of the whole chest; in view of the young age of the patients undergoing correction, it was not thought appropriate to proceed with the cosmetic reconstruction of the pectoral muscles using a peduncled flap of latissimus dorsi. A total of 70 patients were operated in this way; the cosmetic results were very satisfactory, whereas complications were limited to 5 cases of intraoperative pneumothorax, 5 cases of serohematic subcutaneous collection which were treated conservatively, and 1 case of hypertrophic scarring.

[Clinical effects of inspiratory muscle training in patients with chronic airflow limitation]. / Efectos clínicos del entrenamiento muscular inspiratorio en pacientes con limitación crónica del flujo aéreo.

The clinical role of inspiratory muscle training (IMT) in chronic obstructive pulmonary disease (COPD) has not been established, because data on its clinical effect is scarce and controversial. To further investigate these aspects we studied 20 COPD patients (FEV1 37 +/- 3% P) who were randomly and double blindly trained for 30 minutes a day during 10 weeks using a threshold inspiratory trainer with either 30% (group 1) or 10% (group 2) of PIMax as a training load. The training load was crossed after each patient completed 10 weeks of training. Effects were assessed through changes in PIMax, dyspnea through the transition dyspnea index (ITD) and the respiratory effort with Borg's score. Walking capacity was measured with the six minutes walking distance test (6WD) and depression symptoms with Beck's score. Daily life activities were also assessed. Results showed that after 10 weeks of IMT, PIMax increased in both groups (p < 0.05), dyspnea improved in group 1 as compared to group 2 (p < 0.04), 6WD increased significantly in patients of group 1, who also complained of less dyspnea (p < 0.05). Depression scores fell significantly in group 2. Daily activities improved more in group 1. After the crossover patients in group 1 disclosed a significant deterioration in PIMax whereas group 2 disclosed significant improvements in PIMax, dyspnea and 6WD. We conclude that IMT using a threshold device with 30% PIMax is a useful procedure for the treatment of severe COPD patients.

Evaluation of the cochleovestibular function in patients treated with carboplatin for ovarian carcinoma.

Carboplatin (JM8), a new cisdiamminodichloroplatin (CDDP) analogue, has exhibited the same antitumor as CDDP. In our pilot study, aiming at optimizing the dosage of this drug in a combination regimen with other chemotherapics, we planned to evaluate the existence of drug related damage on cochleo-vestibular structures. The study was performed on 9 patients suffering from advanced ovarian carcinoma. The results obtained in our study showed that patients undergoing carboplatin chemotherapy do not require an audiometric check-up. In order to assess definitely the existence of vestibular toxicity of this drug, larger studies planned to test JM8 in a high-dosage regimen are desirable.