[Gastric atresia, diaphragmatic hernia, and gastroesophageal reflux: a rare malformation requiring a multiple approach]. / Atresia del corpo gastrico, ernia diaframmatica laterale sinistra, reflusso gastro-esofageo. Malformazione rara e complessa trattata in più stadi con tecnica mista.

The first cases of atresia or web in the pyloric and prepyloric regions were described in the literature since 1937. To date, only one case of atresia at the junction between the fundus and the body of the stomach has been reported. We want to describe a complex case with incomplete atresia between fundus and gastric body, with left lateral diaphragmatic hernia, treated in several stages by endoscopic, open surgery and minimally invasive surgery

Anorectal malformations (ARM): quality of life assessed in the functional, urologic and neurologic short and long term follow-up.

UNLABELLED: Imperforate anus represents a wide spectrum of anorectal malformations associated with urologic, neurologic and orthopedic malformations. The outcome from the various corrective measures has improved due to new surgical techniques as well as to a better understanding of the pathology. Other factors which influence the overall outcome include the degree of patient acceptance, family support as well as the ability of the health care structure to support the patient's needs on a clinical, functional and psychologic level. AIM OF THE STUDY: Assess (with the new techniques available) the quality of life in the adult patient with ARM and compare it to that experienced by a younger patient; also we wish to determine the correlation between the observed abnormalities with the functional, neurologic and urologic outcome. MATERIALS & METHODS: Sixty-six patients were subjected to PSARP (36 M; 30 F). Six presented with cloaca and 60 with ARM (23 high and 37 low). All patients underwent the same workup to include L/S MRI diagnostics, evaluation for incontinence (urinary and bowel), a urology screening, and if required, a subsequent urodynamic study with rehabilitation and/or bowel management. All answered questionnaires (AIMAR: Italian parent's association of ARM) in order to assess their satisfaction with the current health condition, with the information received and with the treatment and follow-up sessions. The patients were classified into one of two groups. Group A, totaled 33 patients (4 cloacae) with an age range between 2 and 12 years who were operated after 1995. The second, group B, was made up of 33 patients who had been surgically treated before 1995 (age range 15-41 years), had followed the study protocol and had also a neuropsychiatry consult. RESULTS: Overall fecal continence was 69% and of this number 37% were clean without constipation. Twe2nty-one patients (32%) suffered from some form of constipation. Constipation was the most common functional disorder observed in patients who have undergone PSARP. The highest incidence of constipation was found in the ARM (low type), a favorable prognostic group with 43% constipation. Patient with "high" defects and a cloaca had a lower incidence of constipation (18%). Of the 59 patients evaluated, 85% were urinary continent and 15% were incontinent. All of the incontinent patients were in the unfavorable prognostic group of malformations. Urodynamic studies showed 7 neurogenic bladders (NB) and 2 patients with a neurovescical dysfunction (NVD). Of the 50 "dry" patients. 20 had voiding disturbances due to a voiding dysfunction, in the absence of neurologic abnormalities, and presented occasional daytime or nighttime wetting. There was no correlation between the level of the anatomic defect and the urodynamic patterns in the group. Abnormal MRI findings were observed in thirty out of fifty-two patients evaluated. The MRI findings were classified as follows. Severe abnormalities: 7 patients (13%) presented with a combination of skeletal (sacral/lumbar) and spinal cord anomalies. Only spinal cord abnormalities: 12 patients (21%). Only skeletal abnormalities: 11 (19%) patients. Patients were divided into high, low and cloacal malformations. A high degree of statistical correlation was noted between the patients belonging to the cloacae and high defect groups and the abnormal MRI findings. No significant correlation was found between the low defect group and dysrafism, abnormal MRI results and the severity of the malformation. The incidence of Tethered Cord (TC) in our limited number of patients was limited in our study (9% in the high and 7% in the low defect group) when compared to the current literature. Furthermore there was no statistically conclusive evidence that TC by itself affects the urinary or fecal control in our patients. Our recommendation is nevertheless to obtain an MRI study in all patients with ARM. CONCLUSION: All patients 17 and older reported a "good quality of life". Four are married, two with children. Aclose working relationship with the medical personnel is not only necessary but is also well received by the family particularly when younger patients are involved. The adult patient easily adapts even when information is initially scarce. He quickly reaches autonomy with personalized solutions but prefers a longer follow-up time during which, specialized medical facilities will play an important role in the treatment of ARM. Our findings illustrate the importance of both global disease-specific functioning and perceived psychosocial competencies for enhancing the QL of these patients.

Polypoid lesion of the gallbladder in childhood: case report and literature review.

Gallbladder polypoid lesions are rare in the pediatric patient and sometimes represent an incidental finding. A 13 year old male was referred to the Padua Hospital Pediatric Department for an obesity. A routine abdominal ultrasound (US) detected a gallbladder polypoid lesion 6 mm in diameter, initially considered a gallbladder adenoma. Investigation did not detect any other biliary tract abnormality. After seven months, the asymptomatic patient underwent a follow-up US which revealed the disappearance of the polypoid mass. The following concerns are raised: what is the size of the polypoid mass that should be considered for surgery? How does the presence of symptoms worsen the diagnosis and lead to preferring a surgical approach (cholecystectomy) over an echographic follow-up?

[Serious dog bite lesions in childhood: presentation of 3 cases and literature review]. / Lesioni gravi da morso di cane in età pediatrica: descrizione di 3 casi e revisione della letteratura.

Animal bite lesions in the pediatric patient are mainly accounted for by dog bites in 80% to 90% of the cases. They often present a favorable prognosis but serious lesions do account for 5% to 20% of the total incidence. We will present three particular clinical cases which required urgent surgical treatment as well as a review of the current literature to include both medical and surgical treatment methods for this kind of lesion.

Resection of primary tumor at diagnosis in stage IV-S neuroblastoma: does it affect the clinical course?

PURPOSE: To determine whether resection of primary tumor has a favorable influence on outcome of infants (age 0 to 11 months) with stage IV-S neuroblastoma. PATIENTS AND METHODS: Between March 1976 and December 1993, 97 infants with previously untreated neuroblastoma diagnosed in 21 Italian institutions were classified as having stage IV-S disease. Seventy percent were younger than 4 months. Adrenal was the primary tumor site in 64 of 85 patients with a recognizable primary tumor. Liver was the organ most often infiltrated by the tumor (82 patients), followed by bone marrow and skin. RESULTS: The overall survival (OS) rate at 5 years in 80% and event-free survival (EFS) rate 68%. In 24 infants, the effect of resection of primary tumor could not be evaluated because of rapidly fatal disease progression (n = 8), absence of a primary tumor (n = 12), or partial resection (n = 4). Of 73 assessable patients, 26 underwent primary tumor resection at diagnosis: one died of surgical complications, one relapsed locally and died, and two others relapsed (one of these two locally) and survived, for a 5-year OS rate of 92% and EFS rate of 84%. Of the remaining 47 patients who did not undergo primary tumor resection at diagnosis 11 suffered unfavorable events, of whom five died, for an OS rate of 89% and EFS rate of 75% (no significant difference from previous group). Disease recurred at the primary tumor site in only one five who died, and in only one of six survivors of progression or relapse; in these patients, the primary tumor, located in the mediastinum, was successfully resected. CONCLUSION: Infants who underwent resection of the primary tumor at diagnosis had no better outcome than those in whom the decision was made not to operate.

Sugiura procedure in the surgical treatment of bleeding esophageal varices in children: long-term results.

Recurrent bleeding, the high occurrence of encephalopathy, and the impairment of hepatic function in the successful cases of portasystemic shunts have led to increasing dissatisfaction with these procedures in recent years. Between March 1974 and November 1990 we have operated on 15 children for bleeding esophageal varices using the Sugiura procedure (esophageal transection with paraesophagogastric devascularization). In two cases the entire procedure was performed via the thoracic approach. The spleen was left in place in five cases. We have had no mortality. Operative complications included bleeding in the early postoperative period in two children and partial leakage from the esophageal suture in two others. Follow-up was from 4 years 3 months to 16 years 8 months with an average of 10 years 4 months. Long-term results have been gratifying in 12 patients (80%) with disappearance of the varices and no evidence of recurrent bleeding. Three children (20% of the patients) had recurrent bleeding 4 1/2 years, 4 years 2 months, and 2 1/2 years after the surgical procedure. In all cases there was no evidence of esophageal stenosis, gastroesophageal reflux, or hiatal hernia, nor signs of encephalopathy nor impairment of hepatic function. In our opinion the Sugiura procedure is a valid procedure in the surgical treatment of esophageal varices bleeding in the pediatric age with a high rate of success (80%) and without late complications.

Management of giant omphalocele by progressive external compression: case report.

The management of giant omphalocele remains a major surgical challenge. A staged approach is mandatory to achieve an uncomplicated reduction. The case of a child conservatively and successfully treated by progressive external compression of the herniated organs and viscera using an elastic bandaging is described here. The complete integration of the omphalocele content and the closure of the abdominal wall were obtained in 9 days. No ventilatory support was required. The procedure is easy, safe, effective and inexpensive. Large base and intact amniotic sac are prerequisites for feasibility.

Laparoscopic surgery for adolescent varicocele: preliminary report on 80 patients.

Laparoscopic clipping and division of the internal spermatic vessels was performed without complications in 80 adolescents to treat varicocele. In four cases the procedure had to be converted to open surgery. Two of the cases were converted because of technical errors during CO2 insufflation and in two cases because dissection of the vessels was impossible owing to abnormal adhesion of sigmoid colon. At 6 months' follow-up there was only one relapse. Only one hydrocele was noted postoperatively, and testicular size did not decrease in any patient. The authors believe that laparoscopic varicocelectomy is a simple, safe, and effective surgical procedure, which could be proposed as an alternative to open surgical or percutaneous embolization approaches. However, much more experience is needed to obtain a definitive conclusion.

Ureteral reimplantation in children with neurogenic bladder.

The treatment of urologic complications from myelomeningocele and especially of vesico-renal reflux is a controversial problem. A series of 26 reimplanted ureters in 17 children, with good results in more than 85%, is reported. Ureteroneocystostomy, carried out with a few technical innovation, may represent a useful method for the treatment of vesico-renal reflux and obstruction of the uretero-vesical junction in neurogenic bladder associated with myelomeningocele. This surgical approach leads to the disappearance of the reflux, decrease of dilatation of the upper urinary tract and preservation of renal function in most cases; moreover, infection can be more easily controlled. Ureteral reimplantation should be preceded by periodic urethral dilatation, external transurethral sphincterotomy, and pharmacologic regulation in order to attempt to decrease urethral resistance. After successful surgery, it is possible to try to reeducate the bladder. Reimplantation should be preferred to permanent urinary diversion even if there is gross reflux.

Pneumatosis cystoides intestinalis: a rare cause of nonsurgical pneumoperitoneum in an infant.

The authors describe a 3-year-old boy with pneumatosis cystoides intestinalis (PCI) and associated duodenal stenosis who came to them with abdominal pain, significant abdominal distension, large gastric residue, and pneumoperitoneum. PCI is a rare condition in children characterized by the presence of multiple gas-filled cysts within the wall of some part of gastrointestinal tract that may break and cause pneumoperitoneum without an intestinal perforation. Even if in most cases the standard treatment is surgical, to keep in mind this rare condition could be useful to adopt a most rational treatment approach and avoid unnecessary operation in case of "benign" pneumoperitoneum.

Embryonal rhabdomyosarcoma of the lung arising in cystic adenomatoid malformation: case report and review of the literature.

Rhabdomyosarcoma (RMS) of the lung is a very rare lesion, but the association with cystic adenomatoid malformation (CCAM) is unlikely to be a coincidence. Although the etiologic factors predisposing infants and children to pulmonary neoplasms are unknown, pulmonary developmental abnormalities may play a pathogenetic role. A case of embryonal pulmonary RMS is described, which was discovered within a congenital CCAM in a 22-month-old child. The hypothesis regarding histogenesis of this neoplasm are also briefly discussed. Because of the risk of malignant change, early removal of the congenital cystic lesions of the lung is advisable.

Coexisting pelviureteral and vesicoureteral junction obstruction in children.

OBJECTIVE: To address reasons for diagnostic failure in children with coexisting pelviureteral and vesicoureteral junction obstruction. PATIENTS AND METHODS: A total of 11 children with coexisting pelviureteral and vesicoureteral junction obstruction are reported. This anomaly was recognised at presentation in only 5 (45%) of cases. In the remainder, correct diagnosis was made after initial pyeloplasty and a significant delay occurred in 3 children. In all 11 patients treatment consisted of pyeloplasty and subsequent reimplantation. RESULTS: A satisfactory postoperative course with improvement of urinary tract dilatation was noted in all 11 patients, while 8 of 11 patients showed preserved renal function at late follow-up. CONCLUSION: According to the few available data, our data confirm the difficulty of diagnosing coexisting pelviureteral and vesicoureteral junction obstruction in children. However, prompt recognition and relief of urinary obstruction in these patients is mandatory in order to prevent deterioration of renal function.

Fatal cardiac tamponade as a late complication of central venous catheterization: a case report.

Central venous catheterization is a reliable technique in neonatal surgery. Nevertheless, the rate of mechanical catheter-related complications remains high. We report a neonate with gastroschisis in which the successful placement of a central venous catheter was followed later by a cardiac tamponade with a fatal outcome. This complication occurred without perforation of the cardiac wall. A similar finding has been reported in only one other pediatric patient. Vigilant observation is required in any neonate with a central venous line in place to prevent the occurrence of this life-threatening event.

Successful use of transureteroureterostomy in children: a clinical study.

The purpose of this review of patients was to look at the current role of transureteroureterostomy in children. In addition, this study should give an indication of the long-term safety of transureteroureterostomy in children with neurogenic bladder. From 1972 on, 70 subjects between the ages of 2 and 13 underwent transureteroureterostomy. In 68 patients (97 %), transureteroureterostomy was used as a salvage renal procedure after unsuccessful ureteroneocytostomy attempts at other institutions. In 2 patients (3 %), transureteroureterostomy was used as a diversion procedure. The postoperative course was uneventful except for 1 case of early anastomotic occlusion successfully treated by application of a double-J stent. No patient required surgical revision of the ureteral anastomosis. However, 4 patients with neurogenic bladder from myelomeningocele developed sequelae. Of these, 1 presented with stenosis at the end of the recipient ureter, 2 had occurrence of vesicoureteral reflux in nonreimplanted recipient ureter, 1 developed calculi in the donor pelvis. This study supports the view that transureteroureterostomy is highly useful for the management of children with a wide spectrum of urological anomalies. However, long-term monitoring of patients with neurogenic bladder who have undergone this procedure is essential to achieve a long-term good outcome.

Adolescent varicocele: operative anatomy and tricks for successful correction.

Conventional varicocelectomy is often complicated by high recurrence rate, ranging from 6 to 25% and the persistence usually is due to residual venous communications. We report our experience of 21 years in varicocele correction using 3 different operative techniques on 389 adolescents. In the first 12 years (120 patients) a typical ivanissevich operation was carried out with a recurrence rate of 18%. In the following 4 years (63 patients) a preoperative venography was always carried out and a surgical procedure via an inguinal approach was performed with high retroperitoneal and inguinal ligations of the internal spermatic vein(s) and cremasteric vein(s) as indicated by venography; the recurrence rate was still 8%. In the last 5 years we have been using a personally modified technique characterized by: 1) unique access to all venous areas both in the retroperitoneum and in the inguinal canal with a systematic ligation of the internal and external spermatic veins and the deferential vein when dilated; 2) intraoperative identification of the residual collaterals after venous ligations by injecting some milliliters of a blue-methylene solution. This procedure allowed a varicocele recurrence in only 2 out of 206 consecutive patients (1%). Our results suggest that it may be possible to lessen significantly the postoperative recurrence rate using some simple operative tricks.

Labial skin-flap vaginoplasty using tissue expanders

In severe vaginal malformations, when the distance between the upper vaginal pouch and perineum is too long (6 cm or more), reconstruction of the vagina can be performed by colonic interposition or by long cutaneous flaps obtained by the tissue expansion technique. Two female adolescents were treated using expanded labial skin flaps. Dissection and anastomosis between the vaginal remnant and cutaneous tube was performed by the transtrigonal approach. Results were satisfactory at 2.5-year follow-up. In our opinion, expanded labial skin-flap vaginoplasty has three main advantages: (1) it permits the construction of a large, soft, well-vascularized neovagina using non-hair-bearing labial skin; (2) it obviates postoperative dilations and prevents delayed strictures; and (3) a transtrigonal approach permits an easy vaginal dissection and a careful, tension-free anastomosis.

[Correction of anterior or distal hypospadia using the Mathieu-Righini technic]. / La correzione dell'ipospadia anteriore o distale con la tecnica di Mathieu-Righini.

In a overall series of 1621 hypospadias operated on, the Authors describe the technical aspects and report the precise indications and results in 141 cases of anterior and middle penile hypospadias using the one-stage Mathieu-Righini repair. This technique has resulted in remarkably few complications and very nice aesthetic results. There was a remarkably low (3.5%) rate of fistula and no urethral stenosis without necessity of postoperative urethral dilatations. The penis looks "normal" in appearance as well as in function.

[Urinary incontinence in the neuropathic bladder in children. Treatment using an artificial urinary sphincter]. / L'incontinenza urinaria nella vescica neuropatica del bambino. Trattamento con applicazione di sfintere urinario artificiale.

The neurogenic urinary incontinence can be successfully managed by implantation of an artificial urinary sphincter. The most important requirement for implantation is the recognition that the patient has failed to respond to well-accepted conservative measures or is an unsuitable candidate for such conservative measures. It is necessary to rule out the presence of detrusor hyperreflexia or to show that, if it exists, hyperreflexia can be easily pharmacologically controlled. At the same time the bladder's capability for storage of urine must be "adequate" and the vesical compliance normal or only slightly reduced. The bladder emptying should be unobstructed, without residual urine. The patient must be psychologically motivated and able to utilize the artificial sphincter intelligently. The upper urinary tract should be either normal or stable, preferable with no evidence of vesicorenal reflux. Finally, according to the authors, the patient should be more than 11 years old. In the author's opinion these are the ideal candidates for sphincter implantation. The authors operated on 25 patients with neurogenic urinary incontinence, aged from 13 to 19; 23 were males and 2 females. All the male patient had preoperative transurethral external sphincterotomy. In the first 2 cases we applied model AS 792; both these sphincters were substituted with the last model AS 800. In the other 23 cases model AS 800 was used. The cuff was always placed at the bladder neck and only in 1 case, with a previous operation on the neck, at the urethral bulb. Obviously in this late case, the lower pressure balloon was chosen. In all cases primary deactivation was performed; the sphincter was activated after about 3 weeks.(ABSTRACT TRUNCATED AT 250 WORDS)

[A flowmetric study after a Mathieu-Righini intervention in medial-distal hypospadias]. / Studio flussometrico dopo intervento di Mathieu-Righini nelle ipospadie medio-distali.

Uroflowmetric long-term data (mean follow-up 4.5 years) of 64 nonselected patients successfully treated for mid-distal hypospadias with Mathieu-Righini procedure were analyzed. None of our patients needed meatal dilation after primary procedure. Four patients (6.3%) presenting a plateau pattern and a maximum flow rate less than -2 standard deviations were considered to have meatal obstruction. These patients were successfully treated with meatoplasty. We conclude that functional characteristics of the neourethra are quite equivalent to a normal urethra in most of boys and adolescents after meatal based flap urethroplasty. On the other hand, in order to prevent the possibility of unknown strictures postoperative uroflowmetric controls are strongly recommended.

[Progress in the surgical treatment of congenital and acquired broncho-pulmonary pathology]. / I progressi nel trattamento chirurgico della patologia bronco-polmonare congenita ed acquisita.

Surgery is the cornerstone in the management of congenital bronchopulmonary diseases. This term include a wide spectrum of malformative anomalies subdivided clinically into cystic and solid lesions. Bronchogenic cyst (BC), cystic adenomatoid malformation (CAM) and congenital lobar overinflation (CLO) are congenital cystic lesions. Pulmonary sequestration (PS) and pulmonary arteriovenous malformation (PAM) are congenital solid lesions. As a group, congenital bronchopulmonary diseases require removal in order to reduce potentially life-threatening infection and respiratory distress. Bronchopulmonary infections and neoplasms constitutes acquired bronchopulmonary diseases. Improvement in antibiotic therapy has reduced the place of surgery in the current treatment of bronchopulmonary infections. At present, the failure of medical therapy and the appearance of complications are the most common indications for operative treatment. Although these problems are not as common now, at times they required surgical intervention. Finally bronchopulmonary neoplasms are unusual in the pediatric age group. As a result, experience with the diagnosis, management and prognosis is limited. However, the complete surgical resection offers the best chance for survival in the large majority of cases.