[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?

[Non-invasive urodynamic approach to the diagnosis, treatment and follow-up of voiding disorders in pediatric patients]. / Approccio urodinamico non invasivo nella diagnosi, trattamento e follow-up dei disturbi minzionali in età pediatrica.

The authors reports their experience on 248 patients affected by minctional disorders isolated or related to UTI (upper tract infections) and VUR (vesico-ureteral reflux). All the patients were assessed throught a predominantly non invasive diagnostic approach which included: pediatric urologic examination with aimed anamnestic and clinical freaming, functional examination of the lower urinary tract using uroflowmetry + EMG of the perineal plane muscles (UR + EMG), kidney and bladder ultrasound. This methodology has permitted a widening of the indications in the study of vesical function as well as limiting the selected cases (hight UTI, uncertain diagnosis) of mini-invasive examination, such as flow pressure study, minctional cystourethrogram (MC) and or renal scintigraphy. An MNR of the lumbo-sacral medulla (cord) and a neurological and/or neurosurgery evaluation were only carried out were there was a suspected occult neurological pathology. The clinical sintomatogy was as follows: approximately 70% of the patients suffered from partial diurnal incontinence, 42% were affected by secondary nocturnal enuresis while 58.6% suffered from recurrent UTI. In those patients with UTI, 11% (16 patients, 24 ureteral units) suffered from associated VUR while 3.5% suffered from either congenital or acquired urethral stenosis. From the urodynamic examination, we determined the presence of detrusorial instability in 158 patients (64%) and lazy bladder or vescical hypotonia in 84 patients (34%). The suggested therapy foresees the use of: hospital home-based uroriabilitation (minctional biofeedback), endoscopic therapy (sub-ureteral bulking, urethral dilatation) and corrective VUR surgery (only in those cases that did not respond to medical treatment). The percentage of total recovery in patients with detrusorial instability was 80%, the minctional biofeedback both associated and unassociated with drug therapy lead to complete recovery in 66% of patients with lazy and uncordinated bladder, wile 26% showed improvement and 7 patients (8.4%) did not respond. All the patients with VUR (24 reflux units) recovered; and endoscopic therapy and or surgery (12 reflux units) was carried out in some of this patients.

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.

Experience with an elective vacuum assisted cardiopulmonary bypass in the surgical treatment of renal neoplasms extending into the right atrium.

PURPOSE: We evaluate the results of an elective cardiopulmonary bypass conceived to minimize the surgical risk related to its use with temporary circulatory arrest and deep hypothermia in the treatment of patients with renal tumor extending into the right atrium. MATERIALS AND METHODS: From July 1996 to December 2000, 19 patients with renal neoplasm and venous involvement were admitted to our department. Three patients 4, 57 and 58 years old with a right (2) and left (1) renal tumor extending into the right atrium underwent radical nephrectomy and tumor thrombus removal using a normothermic cardiopulmonary bypass. The bypass circuit was connected with a vacuum assisted venous drainage giving a negative pressure of 20 to 40 mm. Hg. Neither circulatory arrest nor hypothermia was used. Tumor thrombus was extracted through a longitudinal "cavotomy" and removed along with the kidney. RESULTS: Total cardiopulmonary bypass time was 14, 19 and 22 minutes, respectively. No intraoperative or postoperative complications due to surgical technique occurred. No significant bleeding was observed at the time of cavotomy and all neoplastic tissue was removed. Pathological examination documented renal cell carcinoma in 2 cases and Wilms tumor in 1. All the patients are alive 30, 42 and 15 months, respectively, after the operation. CONCLUSIONS: Normothermic cardiopulmonary bypass with vacuum assisted venous drainage makes circulatory arrest and hypothermia unnecessary and avoids the potential complications associated with these procedures. With respect to veno-venous shunts this technique guarantees complete surgical control of the thrombus and avoids the need for extensive dissection of the retrohepatic vena cava and Pringle maneuver.

Reoperative orchiopexy: surgical aspects and functional outcome.

Over a 14-year period, a total of 40 patients underwent surgical revision after an unsuccessful orchiopexy attempt. Overall, 34 reorchiopexies (1 bilateral) and 7 orchiectomies were performed. All reorchiopexies were carried out using extensive mobilization of the testis and spermatic cord. This approach was sufficient for scrotal placement of the testis in 23 instances; however, it was necessary to transpose the spermatic vessels medially in the remaining 11 (incision of the transversalis fascia in 5, incision of the transversalis fascia and division of the inferior epigastric vessels in 6). Only 1 prepuberal boy developed testicular atrophy (2%). Functional long-term assessment was done in 20 postpuberal patients using testicular ultrasound and sperm analysis. The reoperated cryptorchid testes were significantly smaller than the controls (P < 0.005), but decreased fertility was only noted in 3 patients (18.7%). We conclude that testicular volume in adulthood is not directly related to fertility, so that accurate reorchiopexy seems to be the best way to preserve fertility in this special group of cryptorchid patients.

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.

Relevance of infection in children with urachal cysts.

OBJECTIVE: To assess the role of infection in the management of children with urachal cysts. METHODS: A retrospective study on 10 children with urachal cysts operated on over an 11-year period (from 1987 to 1998) was performed. Uncomplicated urachal cysts were found in 2 children who underwent primary cyst removal. The remaining 8 were admitted with severe sepsis due to the presence of a urachal abscess; they were managed by a staged approach including percutaneous drainage and delayed cyst removal. The diagnosis of urachal cyst was readily made by ultrasound in all the 10 patients (100%). In 1 patient with urachal abscess, computed tomography provided additional information. RESULTS: The postoperative course was uneventful in 9 of 10 children (90%). A 5-year-old female patient developed peritonitis following urachal abscess rupture into the peritoneal cavity, which resulted in additional surgery and prolonged hospitalization. CONCLUSIONS: (1) Ultrasound is an excellent diagnostic tool for patients with urachal cysts. (2) A renal screening ultrasound must be included in the preoperative work-up. (3) A thorough urological assessment is indicated in patients with abnormal renal ultrasound of recurrent urinary infections. (4) At present, a staged surgical procedure still remains the most effective surgical option in children with urachal cyst.

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.

[Fecal incontinence in pediatric age: diagnostic-therapeutic protocol and possible surgical procedures]. / Incontinenza fecale in età pediatrica: protocollo diagnostico-terapeutico e prospettive chirurgiche.

Fecal incontinence or soiling always endangers the personality development of a children. For very severe cases, the patient will be limited on his educational and professional training as well as on his sexal life. The outlook of severe cases, the chances of completing school, the choice of professional training and of sexual life in the future will be limited. A corrective procedure for anorectal incontinence depends on the exact investigation of all factors contributing to continence. The aim of this paper is to analyze the diagnostic aspects concerning continence in order to choose for each patient the most appropriate procedure for the treatment of all disturbance of stool control. A long term review of 312 patient with MAR, Myelomeningocele and severe stipsis with soiling was undertaken to determine the level of continence achieved. The choice between conservative measures and surgical procedures depends on clinical and manometric findings.

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.

Endoscopic and minimal open surgical incision of ureteroceles.

Ureterocele decompression by endoscopic or open incision was the primary treatment in 59 children with 63 ureteroceles. Of these, 18 were intravesical and 45 were ectopic. The endoscopic or open incision adequately decompressed all intravesical ureteroceles and 37 of 45 ectopic ureteroceles (82%). After incision, the secondary operation rate was 17% for intravesical and 62% for ectopic ureteroceles. In our opinion, endoscopic or open incision must be considered a definitive treatment in the large majority of intravesical ureteroceles and is also valid in ectopic ureteroceles. In these cases, the early decompression obtained by this approach produced significant functional recovery, fewer urinary tract infections, and facilitated subsequent surgery in those patients requiring complete surgical reconstruction.

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.

Juxta-adrenal malignant schwannoma.

A case of malignant schwannoma occurring in an 11-year-old boy is described. To our knowledge, this is the third case of malignant juxta-adrenal schwannoma reported in the literature. It was misdiagnosed at the onset of the disease as a post-traumatic renal hematoma. Angiography revealed that the tumor derived its blood supply from collateral vessels of the aorta, splenic, and left gastric arteries. An analysis of the diagnosis, clinical course, and prognostic factors of this rare tumor is performed.

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.

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.

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.