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Sclerosing stromal tumor (SST) is a rare ovarian tumor arising from the sex cord-stromal cells that occurs mainly in young adults during the second and third decades of life and rarely in pediatric and adolescent populations. The objective of this study is to report three illustrative cases of SST in young girls who had undergone surgery at our clinic in or after 2009, and to perform a literature review of this rare ovarian tumor. A retrospective chart review of female patients aged <18 years with a diagnosis of SST treated in a tertiary pediatric hospital was performed. Furthermore, a 10-year review of the SST literature was completed. Three cases of SST at our institution were outlined. After reviewing the literature, 18 SST cases were identified. The mean age at diagnosis was 13.4 years, and the reported clinical presentations were abdominal or pelvic pain and menstrual irregularity. Seven patients had abnormal hormone tests or CA-125 levels. In approximately 30% of cases, conservative surgery was performed, preserving residual ovarian tissue. In conclusion, some preoperative findings may help in suggesting the presence of SST. However, definitive diagnosis can only be made by histopathological examination. It is important to consider this tumor because, given its benign behavior, a conservative approach is preferred, particularly in this age group.

Background: OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly or Herlyn-Werner-Wunderlich syndrome) is a rare Müllerian malformation. Usually, symptoms begin with worsening dysmenorrhea in post-menarche adolescents. The management in pre-menarche period is controversial and has only recently been subject of study., Aims: To review the experience of a pediatric tertiary center and to propose an age-specific management protocol for patients diagnosed before menarche., Methods: A retrospective cohort study (review of medical records - period 2009-2021)., Results: Twenty-eight patients were diagnosed (mean age 11.9 years), seven (25%) before menarche, one (3%) perinatally. One patient had Floating-Harbor syndrome. Twenty-three patients had ipsilateral renal agenesis, while five had a multicystic-dysplastic kidney. The contralateral kidney showed hypertrophy in 25 patients, pelvicalyceal ectasia in 8 and dysplasia in 1. Twenty-four patients were symptomatic. Three of the seven diagnosed prior to menarche had symptoms. All post-menarche diagnosed patients were symptomatic. Twenty-six patients underwent surgery (one-stage drainage, vaginal septal resection, and vaginoplasty). Asymptomatic pre-menarche patients were followed-up until surgery after menarche onset. No patient underwent surgery prior to menarche solely for OHVIRA diagnosis. At follow-up (median 3.5 years, 3 lost to follow-up), eighteen patients were asymptomatic, one developed endometriosis, one had impaired renal function, two needed reoperations., Conclusions: Pre-menarche OHVIRA patients, without symptoms, should undergo regular follow-up until the onset of menarche. Surgery must be considered in post-menarche or symptomatic patients. Post-operative, long-term follow-up is required, evaluating both renal and gynecological issues., Level-Of-Evidence: IV., Competing Interests: Declaration of Competing Interest The authors have no financial relationships, competing interests or conflicts of interest to disclose. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Anogenital condylomata acuminata are induced by human papillomavirus (HPV) and they rarely manifest in immunocompetent children. Therapeutic options depend on patient's age and general conditions and extension of the lesions. However, management is still a challenge and recurrences are frequent. Cryotherapy, laser, and surgical treatments in children are painful and frequently require general anesthesia. Imiquimod is a topical immune response modifier and constitutes a noninvasive alternative for the treatment of anogenital condylomata acuminata. Here, we report an infant admitted to our hospital with a giant vegetative papillomatous lesion on the perianal region surrounded by small satellites papules. PCR for HPV confirmed the clinical diagnosis of giant condylomata acuminata due to HPV type 6. The child has been successfully treated with topical 5% imiquimod cream without side effects. Although topical imiquimod is not licensed for pediatric age, this report highlights the potential benefits of its use in selected pediatric cases., (© 2020 Wiley Periodicals LLC.)

Purpose: The food industry is a well-established and complex industry. New entrants attempting to penetrate it via the commercialization of a new technological innovation could face high uncertainty and constraints. The capability to innovate through collaboration and to identify suitable strategies and innovative business models (BMs) can be particularly important for bringing a technological innovation to this market. However, although the potential for these capabilities has been advocated, we still lack a complete understanding of how new ventures could support the technology commercialization process via the development of BMs. The paper aims to discuss these issues., Design/methodology/approach: To address this gap, this paper builds a conceptual framework that knits together the different bodies of extant literature (i.e. entrepreneurship, strategy and innovation) to analyze the BM innovation processes associated with the exploitation of emerging technologies; determines the suitability of the framework using data from the exploratory case study of IT IS 3D - a firm which has started to exploit 3D printing in the food industry; and improves the initial conceptual framework with the findings that emerged in the case study., Findings: From this analysis it emerged that: companies could use more than one BM at a time; hence, BM innovation processes could co-exist and be run in parallel; the facing of high uncertainty might lead firms to choose a closed and/or a familiar BM, while explorative strategies could be pursued with open BMs; significant changes in strategies during the technology commercialization process are not necessarily reflected in a radical change in the BM; and firms could deliberately adopt interim strategies and BMs as means to identify the more suitable ones to reach the market., Originality/value: This case study illustrates how firms could innovate the processes of their BM development to face the uncertainties linked with the entry into a mature and highly conservative industry (food).

Background: Ovarian torsion (OT) is a serious condition, and delay in surgical intervention may result in loss of the ovary. Children and adolescents who have suffered from ovarian torsion may be at risk for asynchronous torsion of the contralateral ovary., Study Objective: Three cases of asynchronous bilateral ovarian torsion were reported to analyse clinical history of three patients, to review the current literature, and to draw a conclusion for future treatment., Design: Case reports and review of the literature., Result: When a prepubertal girl presents with an ovarian torsion, several considerations have to be taken in account in order to preserve her future fertility; in particular, the pediatric surgeon/gynecologist has to preserve as much as possible the twisted ovary in addition to considering the fate of the contralateral ovary., Summary and Conclusions: Pelvic pain in a young girl has always raised the clinical suspect of an ovarian torsion; the possibility of asynchronous bilateral ovarian torsion is rare, but it is described in the literature and has catastrophic consequences; this condition has to be known and treated in the proper way by pediatric surgeons as well as by gynecologists in order to maximize the future fertility of the young patients.

Statement of Problem: Metal ions released into the oral cavity from dental prosthesis alloys may damage the cellular metabolism or proliferation and cause hypersensitivity or allergies. The oral cavity environment is particularly prone to corrosion due to saliva, microorganisms, and pH variations., Purpose: The purpose of this in vitro study was to evaluate the ion release of chromium, cobalt, and iron from the Co-Cr alloys used for traditionally cast and computer-aided design/computer-aided manufacturing dental devices after interaction with oral bacteria and different pH conditions., Material and Methods: All specimens were prepared from currently available alloys, polished, and immersed in 3 different pH media (artificial saliva [pH 2.3] and 6.5% and 0.9% saline solution [pH 7.1]). Specimens were also incubated in the presence of the bacterium Eikenella corrodens. Solutions were analyzed with an atomic absorption spectrometer after 15 and 30 days in the chemical corrosion test and 30 days in the biocorrosion test to detect ions released in different solutions. An ANOVA test was used to evaluate statistically significant differences among the percentages of metal corrosion ion release values., Results: The greatest amount of element release was seen after 30 days: 4.964 ppm of casting alloy, 2.642 ppm of milling alloy, and 2.351 ppm of laser metal sintering., Conclusions: With the exception of casting alloy under acidic conditions, no significant differences were found, even after exposure to bacteria., (Copyright © 2015 Editorial Council for the Journal of Prosthetic Dentistry. Published by Elsevier Inc. All rights reserved.)

Introduction: Discrepant data exist regarding the incidence and severity of clinical problems related to intra-hospital transport of brain-injured patients and no consensus exists whether modern-day intra-hospital transport represents a safe or potentially problematic environment for neurointensive care unit (NICU) patients., Methods: We examined the incidence of clinical complications and physiological derangements that occurred in 160 neurologically injured patients (90 males, 70 females, mean age 57 ± 17 years) who underwent intra-hospital transport (288 cases, 237 scheduled, 51 unscheduled) for computed tomography scans., Results: Our findings indicate that (1) at least one significant complication (predominantly hemodynamic) occurred in over one-third (36%) of all transports (p = n.s scheduled vs. unscheduled) necessitating the deployment of interventions designed to treat changes in arterial pressure (2) despite the presence of trained medical personnel and availability of specialized equipment, intra-cranial pressure was not adequately monitored during transports (especially in patients with intra-cranial hypertension prior to transport) (3) intra-hospital transfer was associated with minor but statistically significant clinical changes, including a reduction in arterial partial pressure of oxygen (Pa(O(2)))/inspired oxygen fraction (Fi(O(2))) (only in the scheduled transport population), decreased arterial lactate levels (scheduled transport population), lowered body temperature (scheduled transport population), and increased arterial partial pressure of carbon dioxide (Pa(CO(2))) (scheduled transport population)., Conclusions: Intra-hospital transport of brain-injured NICU patients may present some hazards even if performed by skilled personnel with specialized equipment. In Trauma Centers such as ours, an improvement in the frequency of neuromonitoring [intra-cranial pressure (ICP) and end-tidal CO2 (ET(CO(2)))] during transport is recommended.

Background: Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. The diagnosis is generally made only if the suspicion of this genitourinary syndrome is raised., Objective: To highlight the imaging diagnostic clues in this rare condition., Materials and Methods: We report on 11 adolescents with this condition., Results: Sonography mostly allowed the correct diagnosis by showing uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney. MRI provided more detailed information regarding uterine morphology, the continuity with each vaginal channel (obstructed and nonobstructed), and the bloody nature of the contents., Conclusion: Early and accurate diagnosis of this syndrome is important so that adequate and prompt surgical therapy (excision of the vaginal septum) can provide relief of pain and prevent further complications. It is also advisable to look for an obstructed Müllerian system whenever a multicystic dysplastic kidney or the absence of a kidney is discovered in a fetus, or girl postnatally.

Isolated torsion of the fallopian tube is a rare clinical entity, especially in adolescents and at menarche. The diagnosis is essentially made at laparoscopy or at laparotomy because of nonspecific clinical signs. We present a case of isolated tubal torsion in a 12-year-old girl a few days after menarche, highlighting the sonographic and MR findings. Both techniques demonstrated the enlarged and tortuous fallopian tube with normal ovaries and uterus, but MR was also able to characterize contained blood and absent vascular supply. Although this condition is uncommon it should be considered as a cause of acute pelvic pain in adolescents because of the possibility of salvage surgery with early diagnosis. Sonography and MRI have a complementary role in this diagnosis.

Purpose: Maintenance of spermatogenesis in hypogonadotropic hypogonadal patients is possible and is an objective to be actively pursued. Gonadotropin releasing hormone and gonadotropin/ testosterone therapy can develop and maintain sex characteristics and spermatogenesis such that even small gonads should be preserved. An empty scrotum, like any other congenital or acquired condition compromising the size of the genitalia, can produce serious psychosexual problems and have an extremely negative effect on normal social life., Materials and Methods: In 2 adolescents affected by Kallmann's syndrome with testes of infantile volume 1 gonad was transferred to the contralateral hemiscrotum and replaced by an adult testicular prosthesis., Results: There were no postoperative complications. The cosmetic results were considered satisfactory by the patients and their parents., Conclusions: When possible and when there is no risk of physiological repercussions, every effort should be made to normalize the appearance of the genital area for strong psychological reasons.

The diagnostic accuracy of laparoscopy for impalpable testis is well recognized. However, in some cases, laparoscopic findings may be misleading, and a viable gonad may be missed with significant medico-legal implications. From January 1993 to December 2000, 202 patients with 219 impalpable testes were evaluated. In 95 cases, the gonad was immediately visualized, and in 5, the presence of a testis was documented by inserting the scope into the processus vaginalis. In the 119 remaining cases, no gonad was seen while entering the abdomen with the laparoscope. All patients with documented vas and vessels exiting the inguinal ring were surgically explored. Ten testes were found, 8 ectopic, with significant changes in shape and position, and 2 were canalicular. In the absence of hormone stimulation, no testes were found while exploring patients with cord structures coursing a closed inguinal ring and with contralateral hypertrophy. In 1 patient with absent vas and vessels, the testis was found at the lower renal pole while removing a dysplastic kidney. Despite technical refinements and an increase in clinical practice, a small percentage of viable testes may be missed with laparoscopic findings consistent with absent/vanished inguinal testis. Therefore, inguinal exploration is mandatory in all these cases.

Purpose: Neurovesical dysfunction (NVD) is common in children with anorectal malformation (ARM). NVD is mainly related to tethered cord or iatrogenic injury but how to investigate it is still debated. We evaluate the usefulness of routine magnetic resonance imaging (MRI) and urodynamics (UDS) for ARM., Materials and Methods: A total of 89 children were screened for sacral, spinal or urological anomalies using sacrum x-ray, MRI, renal and spinal ultrasound, uroflowmetry and/or 4-hour voiding observation. UDS was performed in 60 patients with suspected NVD. Mean +/- SD followup was 9.8 +/- 5.2 years., Results: Of the 89 patients 29 presented with urinary tract anomalies. The prevalence of sacral (53 cases) and spinal cord (54) anomalies was no different between patients with low, intermediate and high ARM. Spinal cord tethering was present in 13 patients with a normal sacrum x-ray. NVD was found in 31 of the 89 patients (hyperreflexia 21 and hypo-areflexia 10), and was associated with sacral and spinal anomalies in 23, occult spinal dysraphism without bone lesion in 3 and sacral anomalies in 5. The incidence of NVD was 40% of cases with low and 51% with high ARM., Conclusions: Because tethered cord occurs in children without sacral anomalies as well as in those with low ARM, we recommend evaluation of all patients using MRI. When MRI is positive UDS should be performed. We agree with a previous suggestion to evaluate all males with rectourethral fistula and females with cloaca malformations. Finally we recommend a noninvasive evaluation for all other children and UDS when neurogenic dysfunction is suspected.

Gastrointestinal motility disorders are frequently found in several pathologies. The aim of this study was to assess, by means of electrogastrography, the presence of gastrointestinal motility abnormalities in children affected by Crohn's disease (CD) or Chronic Intestinal Pseudo-Obstruction (CIPO). Patients and Methods. We studied 34 subjects; 20 control subjects (M = 15, mean age = 10 +/- 3.5 yrs), 8 patients (M = 4, mean age = 18 +/- 7 yrs) with Crohn's disease in a quiescent phase and 6 patients (M = 6, mean age = 10 +/- 3.5 yrs) with Chronic Intestinal Pseudo-Obstruction. Results. Analysis of gastric electrical activity (GEA) parameters demonstrated that in the control group physiological post-prandial changes are represented by an increase of 3 Cycles Per Minute (3 CPM) activity, Period Dominant Power (PDP) and Period Dominant Frequency (PDF) and by the reduction of bradygastria. Crohn patients showed an insignificant increase of 3 CPM and PDP; CIPO patients showed an abnormal variation of 3 CPM, PDP and post-prandial bradygastria. Moreover, CD patients showed a significant difference in post-prandial values of PDP compared to normal subjects. CIPO patients revealed a significant difference in the values of either preprandial PDF with tachygastria or the post-prandial value of 3 CPM, compared to normal subjects. Conclusions. EEG is a non-invasive method to study gut motility related to GEA alterations present in CIPO as well as in CD patients.

Purpose: Despite the introduction of more refined surgical techniques, the optimal treatment of the most severe forms of hypospadias remains to be determined. Single stage procedures, whether with the use of flaps or grafts, have long been regarded as the best approach, although the complication rate is nonnegligible with all procedures., Materials and Methods: We report the use of a 2-stage repair with preputial graft interposition and subsequent tubularization of the urethral plate applied it to all severe cases of hypospadias with significant chordee or small glans., Results: Both stages of the procedure were completed in 34 patients. Complications in 8 cases (23.5%) included 4 glans disruption in 4, coronal grove fistula in 2, urethral diverticulum in 1 and urethral stenosis due to balanitis xerotica obliterans in 1. Two pinhole fistulas also occurred which closed spontaneously. No complete disruptions or postoperative hematomas/bleeding was noted. Cosmetic and functional outcome after a minimum followup of 1 month was optimal in all cases with a normally located "slit" meatus and straight penile shaft., Conclusions: Although the controversy between use of grafts and flaps will probably continue forever, we believe that our 2-stage approach should be considered as a valid alternative for the most severe forms of hypospadias. Long-term results appear to outnumber the necessity of a learning curve for appropriate graft manipulation.

The first case of pediatric paratesticular pilomatricoma is reported. Differential diagnosis with other more common lesions in such a site is the main issue. Conservative surgery is the treatment of choice. Follow-up is recommended, since most pilomatricomata are benign, but rarely malignant transformation may occur.

Purpose: There is general agreement on treatment for varicocele in pediatric patients. Randomized prospective studies have shown that anatomical and functional lesions may be corrected. Due to the impossibility of seminal examination patients with moderate to large varicocele or ipsilateral testicular hypertrophy, characterized by a change in testicular consistency or symptoms, should undergo surgical correction. The best therapeutic approach is still under discussion., Materials and Methods: At 2 centers 2 therapeutic approaches to varicocele treatment in pediatric patients were compared, namely the Palomo repair and antegrade sclerotherapy according to Tauber. The 89 patients from the same geographical area elected 1 procedure after an explanation. From the medical records we retrospectively evaluated operative time, postoperative analgesics, postoperative fever onset, complications, convalescence, recurrence and postoperative hydrocele., Results: After Palomo repair in 45 patients there were 2 recurrences (4.4%) and 2 postoperative hydroceles (4.4%). Of 44 antegrade sclerotherapy cases 1 was converted to Palomo repair, there was no hydrocele formation and recurrence developed in 2 (4.5%). Testicular atrophy was not observed in any patient regardless of the method used. The cost of the procedure was lower in the sclerotherapy group., Conclusions: These data suggest that the failure rate was similar in both groups. The principal advantages of sclerotherapy are simplicity, decreased cost and lack of hydrocele formation.

Abdominoscrotal hydrocele (ASH) consists of two large sacs, both abdominal and scrotal, connecting with the inguinal channel. The diagnosis is made only by ultrasound scan. Surgical treatment is mandatory since no spontaneous resolution has been reported. A new surgical procedure used successfully to treat 11 patients with ASH is described.

Short-bowel syndrome (SBS) either in adults or in children is considered as an indication to small-bowel transplantation (SBTx), particularly in its most severe form with a residual bowel length below 20 cm. Among factors likely to worsen the prognosis, more recent reports also indicate the number of surgical interventions, early onset sepsis and early development of liver disease. We report six cases of ultra-short-bowel syndrome followed from birth to verify the importance of various prognostic factors. In our case series, the male sex is predominating (5:1). Intestinal resection was indicated in 3 patients for multiple intestinal atresias, in 2 for volvulus and in 1 for necrotizing enterocolitis. The length of intestine remaining was invariably less than 20 cm and 2 patients had a preserved ileocecal valve. In most cases, more than 50% of the colon remained. The number of abdominal operations ranged from 1 to 4. In almost all cases (5 of 6), sepsis and hepatopathy developed early. Our experience suggests that rather than depending on the length of intestine remaining or the presence of the ileocecal valve, the prognosis of patients with the extreme-short-bowel syndrome depends on recurrent neonatal onset sepsis and early onset liver impairment. In addition, our case review shows that the extreme-short-bowel syndrome is not necessarily an indication for bowel transplantation.

Background: It is difficult to give guidelines when approaching gastroesophageal disease in neurologically impaired children. Indication for surgery has been increasing over recent years, but there is no consensus on the surgical technique of choice. Nothing has been written specifically comparing the results of different procedures in these patients, so far., Study Design: We retrospectively compare the short- and long-term results of two different types of fundoplication in a series of children operated on for documented gastroesophageal reflux disease at our institution., Results: One group (group A) of 27 patients, operated on between 1977 and 1993, underwent Nissen fundoplication, the other (group B), formed of 20 patients all of whom were operated on between 1993 and 1995, underwent Thai fundoplication. We compared the results in terms of positive outcome (recovery) and negative outcome (minor and major complication), computing the relative odds of group A versus group B in terms of risk of complication, and we compared the mean operative time and the length of hospital stay by means of a student's t-test analysis., Conclusions: Our results show that there is no statistical difference between the two procedures in terms of relative risk of complication and success rate. The duration of surgery and hospital stay were significantly shorter in group B. The Thal procedure can, therefore, be proposed as first choice in the management of these patients.

Methods: From 1983 to 1996, 31 children with caustic esophageal strictures were seen at Bambino Gesù Children's Hospital; they were all treated conservatively except for two cases complicated by tracheoesophageal fistula. The remaining 29 patients were divided into three groups depending on the treatment, which was modified over the years. Group A (1983 to 1987) consisted of seven patients treated by periodic dilatations; group B (1988 to 1992) consisted of 10 children treated by 40 days of esophageal stenting plus dexamethasone, 0.5 mg/kg/d plus ranitidine plus no oral feeding for 7 to 10 days; group C (1993 to 1996) consisted of 12 cases treated by 40 days of esophageal stenting plus dexamethasone, 1 mg/kg/d plus omeprazole plus early oral feeding resumption., Results: No differences were observed between the three groups of patients with regard to the mean age and to the ingested substance, whereas a significant difference (P = .007) was observed in the mean length of the stricture between group A and C (3.4+/-1.3 and 5.6+/-1.6 cm, respectively). In all but one of the patients (96.5%) complete healing of the stenosis was achieved by conservative treatment, with definitive relief of dysphagia. One patient in group C did not improve after a repeated stenting procedure and was surgically treated. However, in group A, resolution of the stricture was obtained after an average of 19.9+/-14.8 dilatations in a mean period of 25.3+/-17.2 months. In group B, a mean of 12+/-11.3 dilatations were required in a mean period of treatment of 14.1+/-10.6 months. In patients in group C, a mean of 3.5+/-3.2 dilatations were necessary in a mean of 5.8+/-4.8 months. A statistically significant difference was observed both with regard to the number of dilatations and to the duration of treatment, between group A and group C (P = .002) and group B and C (P = .03)., Conclusion: Esophageal replacement should be considered only in cases complicated by tracheoesophageal fistula or in the rare patients who do not respond to repeated esophageal stenting.

The long-term follow-up of patients operated on for congenital diaphragmatic hernia (CDH) at birth has been extensively evaluated, both clinically and with respect to respiratory function. However, little is known about the sports practice and stress performance of these subjects. Fifteen of 107 patients operated on for CDH underwent exercise stress testing with a stepwise increase in workload. A questionnaire was provided, which requested information on sports practice and lifestyle. Maximal oxygen consumption [Vo2 max] was measured along with dynamic lung volumes. Clinical examination included a whole-body assessment (height, weight, skinfolds) and vital parameters (heart rate and blood pressure). Fifteen healthy children who practiced regular physical activity (2 to 4 hours/week) served as controls. All the CDH patients experienced a good lifestyle, but only 8 of them were participating in sports. Exercise duration and Vo2 max were significantly lower for the CDH patients, and were lowest for the sedentary patients. Therefore, the reduced Vo2 max of these otherwise healthy children most likely represents a lower degree of physical fitness rather than decreased respiratory function. Fitness is an expression of well-being; thus, there is evidence that these patients could safely participate in competitive motor activities.

Spinal dysraphism (SD) has been found associated with functional abnormalities of anorectal anomalies (ARA). The incidence of SD in these children is probably underestimated and a complete neuroradiological investigation of the lower urinary tract function has not been carried out routinely. In a 2 years time frame we performed urodynamic (UDS) evaluations on 14 patients (8 males and 6 females) with ARA who showed SD at Magnetic Resonance Imaging (MRI) studies. We divided them into 2 groups by age: group A (5 to 18 months) and group B (3 to 12 years). The UDS findings were classified as upper (UMN) and lower motor neuron (LMN) lesion. Out of the 9 children included in group A, 5 showed normal urodynamic pattern, while 4 had pathological UDS findings suggesting UMN lesion. Among the older (group B) children only one had normal and 4 had pathological findings: 2 hyperreflexia suggesting UMN lesion and 2 external sphincter denervation suggesting LMN lesion. These data support the hypothesis that the neurovesical dysfunction found in children with anorectal anomalies results from a possible association with spinal dysraphism. An early morphological evaluation of the spinal cord is mandatory in all children with ARA prior to definitive surgical correction of the malformation. The low incidence of pathological UDS findings in small children if compared to older ones suggest that SD, although present, may be asymptomatic.

Ulcerative colitis is seen with increasing frequency in paediatric age and its diagnosis is made more difficult by atypical cases. Sixty-five patients with UC were seen at our institute and all of them underwent medical treatment. In all patients the disease extended to the whole colon (pancolitis). Eleven patients (average age 9 yrs) underwent surgical correction by Endorectal Pull Through (EPT) 8 straight and 3 with ileal reservoir. One straight EPT had to be converted to Brooke ileostomy because of unacceptable stool frequency. In the rest of the patients the disease is well controlled with medical treatment. After 2 years of follow up surgical complications, continence, stool frequency and quality of life were evaluated: results indicate that surgical complications rate is the same as in other reported series; furthermore, continence and stool frequency are good with all surgical techniques eve though straight pull-through may require a period of adaptation the length of which varies considerably. Our results confirm that children with pancolitis and severe symptoms should be offered prolonged medical treatment prior to undertake surgical correction.

The authors report their experience in the treatment of the chronic constipation in paediatric age. During the last 3 years (1991-1994), 230 children presenting chronic constipation have been studied at the Strumental Gastroenterologic Department of Children Hospital Bambino Gesù; 19 of them (8%), 10 male and 9 female, were studied with anorectal manometry, defecography and Intestinal Transit test. All the patients had a overtone (high squeeze) of the anal sphincter and the medical treatment was unsuccessful. They underwent sphincteromyectomy by posterior approach. In 17 patients it has been obtained a notable improvement of the symptomatology with regularization of the evacuations. The simplicity of the surgical technique, the absence of complications and the obtained results confirm the validity of sphinteromyectomy in the therapy of the chronic constipation in paediatric age. Interoperating anorectal manometry proved to be essential both in modulating sphincteromyectomy and in its eventual complications.

The maximal physical activity capacity of children operated on for tracheoesophageal fistula (TEF) has not been clearly defined. Eight patients (average age, 12 years) successfully operated on for TEF at birth underwent maximal exercise stress testing on a treadmill, according to the Bruce protocol, to test physical work capacity. Heart rate (HR), oxygen consumption (VO2), and pulmonary ventilation (VE) were measured by a portable lightweight telemetric device. Nine healthy children served as controls. Exercise duration was significantly lower for TEF subjects than for controls (11.6 +/- 1.7 minutes v 15.1 +/- 2.3 minutes; P < .01). Mean HR at rest and during exercise did not differ between the groups. All children reached the maximum HR according to their age; however, for the majority of TEF patients, this occurred at an earlier stage than in the controls. No differences were seen in mean VO2 at rest and on exertion between TEF and control children. However, maximal VO2, as measured at the end of exercise, was significantly different when normalized per kilogram of body weight (VO2/kg = 52.3 +/- 5.8 v 33.3 +/- 6.6; P < .005). The physical rehabilitation of TEF children usually takes into account only respiratory and nutritional factors. However, complete assessment of their cardiac and respiratory function, at rest and on exertion, also should be performed, because this may show that some patients have reduced motor performance; evidence is now accumulating that these children can safely participate in the same physical activities of their healthy peers.

Though the concept of caudal regression, suggested in 1961 by Duhamel, could explain the association between anorectal anomalies (ARA) and spinal dysraphism (SD), its real incidence may be underestimated and its clinical significance is debatable. From 1988 to 1993, 111 patients with ARA were treated at the authors' institution. Associated anomalies were present in 36% of cases, with the exception of vesicoureteral reflux, which was considered functional rather than anatomical. In view of the late (1 to 2 years after surgical treatment) onset of vesical dysfunction and/or orthopaedic symptoms in some of these patients, a screening protocol was started in 1991, using magnetic resonance imaging (MRI) in all patients with ARA. Fifty patients, 29 boys and 21 girls, underwent a spinal cord MRI, with pathological findings in 25 cases (50%), 13 boys and 12 girls. The authors did not find any significant difference in incidence with respect to high, low, or cloacal malformations. A thickened filum, with or without fibrolipoma, was the most frequent finding, but even tethered cord, syringomyelia, and sac morphological alterations were present. MRI was also able to detect osteoarticular and/or muscular anomalies. Even when a urodynamic study and a neurological and orthopaedic workup were performed in 20 patients undergoing MRI, the clinical significance of these findings remained unclear. However, accurate follow-up of these patients is mandatory in order to detect early neurological symptoms, because currently it is not advisable to refer for neurosurgery all the patients with ARA presenting with anomalies of the spinal cord.

The urological malformations associated with anorectal anomalies (ARA) are not only anatomical, but also functional, the latter being related to congenital neurovesical dysfunction (NVD). The true incidence of spinal dysraphism (SD) in these children is still unclear and is probably underestimated. The concept of caudal regression could explain its association with the anorectal anomalies. Because of awareness of the late onset of neurovesical dysfunction and/or orthopaedic symptoms in some of our patients, in 1991 we started to screen with magnetic resonance imaging (MRI) the spinal cord of all patients with ARA. Eighteen (44%) out of 41 patients without neurological or orthopaedic symptoms and 7 (78%) of 9 children with neurological or orthopaedics symptoms screened by MRI showed pathological findings. The overall incidence of spinal dysraphism in ARA was 50%, without any great difference with respect to the type of the anomaly (high, low, cloacal). The pathological MRI findings encountered were: fibrolipoma (with or without a thickened filum terminale), tethering of the cord and syringomyelia, and sac anomalies. In order to check the onset of NVD in these children, we performed urodynamic studies with external sphincter electromyography in 24. Grouped by age: 14 were between 5 and 18 months and ten were 4 to 13 years old. Ten patients (71%) out of the first group and 3 (30%) out of the second had a normal urodynamic pattern. A total of 11 children had pathological findings; of these, 4 infants had a hyperreflexic bladder (one with detrusor-sphincter dyssynergia) suggesting upper motor neuron (UMN) lesion. Of the 7 older patients, two had UMN and 3 lower motor neuron (LMN) lesion.(ABSTRACT TRUNCATED AT 250 WORDS)