Long-term urological outcome of cloaca patients with multidisciplinary management.

PURPOSE: Urological management of Cloacal Malformation (CM) focuses on preserving renal function and continence. Study aim was to analyze urinary and intestinal outcomes in CM patients, considering the length of common channel (CC) and presence of occult spinal dysraphism (OSD). METHODS: Retrospective review of CM treated at our institution by a multidisciplinary team from 1999 to 2020. Patients with follow-up < 2.5 years were excluded. Length of CC, renal function, urinary and bowel outcomes, presence of associated anomalies (especially OSD) were evaluated. RESULTS: Twenty patients were included, median age at follow-up: 8 years (4-15). A long CC > 3 cm was described in 11 (55%). Chronic kidney disease was found in 3 patients. Urinary continence was achieved in 8/20 patients, dryness (with intermittent catheterization) in 9/20. Fecal continence was obtained in 3/20, cleanliness in 14 (under bowel regimen). OSD was present in 10 patients (higher prevalence in long-CC, 73%). Among OSD, 1 patient reached fecal continence, 7 were clean; 2 achieved urinary continence, while 6 were dry. CONCLUSIONS: Length of CC and OSD may affect urinary and fecal continence. An early counseling can improve outcome at long-term follow-up. Multidisciplinary management with patient centralization in high grade institutions is recommended to achieve better results.

Short versus mid-long-term outcome of transanal irrigation in children with spina bifida and anorectal malformations.

PURPOSE: We assessed short- and mid-long-term clinical efficacy of transanal irrigation (TAI) and its effect on the quality of life of children with spina bifida (SB) and anorectal malformations (ARM). METHODS: Seventy-four pediatric patients (age 6-17 years) with SB and ARM with neurogenic bowel dysfunction were enrolled for a prospective and multicentric study. Patients were evaluated before the beginning of TAI (T0), after 3 months (T1) and after at least 2 years (range 24-32 months) (T2) using a questionnaire assessing bowel function, the Bristol scale, and two validated questionnaires on quality of life: the CHQ-PF50 questionnaire for the parents of patients aged 6-11 years and the SF36 questionnaires for patients aged between 12 and 18 years. RESULTS: Seventy-two patients completed TAI program in T1, and 67 continued into T2. Bowel outcomes (constipation and fecal incontinence) improved in both the SB and the ARM groups in the short and mid-long term. In both groups at T1 and T2, parents and children reported an improvement in quality of life and there was a significant increase of stool form types 4 and 5 as described by the Bristol scale. Common adverse effects during the study were similar at T1 and T2 without serious complications. CONCLUSIONS: We observed a sustained improvement in bowel management and quality of life in SB and ARM children during the study, more significant in the short term than in mid-long term. To maintain success rates in the mid-long term and to reduce the dropout rate, we propose patient training and careful follow-ups.

Peristeen(®) transanal irrigation in paediatric patients with anorectal malformations and spinal cord lesions: a multicentre Italian study.

AIM: In paediatric and adult patients with neurogenic bowel, transanal irrigation (TAI) of the colon has gained popularity due to the introduction of a specifically designed device. The aim of this pilot study was to present the results of TAI using the Peristeen(®) TAI system in a group of paediatric patients with anorectal malformation (ARM) and congenital or acquired spinal cord lesions (SCLs). METHOD: Eight Italian paediatric surgery and spina bifida centres participated in the study. The inclusion criteria were age between 6 and 17 years, weight above 20 kg and unsatisfactory bowel management. Patients with chronic inflammatory bowel disease, mental disability and surgery within the previous 3 months were excluded. At the beginning of treatment (T0) and after 3 months (T1) the Bristol scale, a questionnaire assessing bowel function, and two questionnaires on quality of life (QoL) for patients aged 6-11 years (CHQ-pf50) and 12-17 years (SF36) were administered. RESULTS: Eighty-three patients were enrolled, and seventy-eight completed the study (41 ARMs, 37 SCLs). At T1, constipation was reduced in ARMs from 69% to 25.6% and in SCLs from 92.7% to 41.5%, faecal incontinence in ARMs from 50% to 18.6% and in SCLs from 39% to 9.8% and flatus incontinence in ARMs from 20.9% to 9.8% and in SCLs from 31.7% to 10%. At T0, the Bristol Stool Scale types were 1-2 in 45% of ARMs and 77.5% of SCL patients, whereas at T1 types 1-2 were recorded in only 2.5% of SCL patients. QoL improved in both groups. In the younger group, a significant improvement in QoL was recorded in ARM patients for eight of nine variables and in SCL patients for seven of nine variables. CONCLUSION: This study showed that Peristeen TAI resulted in a significant time reduction in colonic cleansing, increased independence from the carer and improved QoL in paediatric patients with ARMs and SCLs.

Epidydimo-orchitis and anorectal malformations: when and in whom?

PURPOSE: Epididymo-orchitis (EO) is infrequently reported in anorectal malformation (ARM) cases. Therefore, it is difficult to assess its risk factors. METHODS: A total of 110 male patients who were operated on for ARM at the same Institution over a period of 13 years were contacted. Association was assessed between EO and the following: spinal dysraphism (SD), symptomatic VUR (VUR), and bowel management (BM) requiring enemas. The data were analyzed with the Chi-square test. RESULTS: A total of 89 patients were contacted. Ten cases of EO were found, and all occurred in patients with recto-urethral (RU) fistula after reconstruction. The patients' age at first episode ranged between 4 and 11 years. RU fistula patients experiencing EO (Group A, 10 patients) were compared with those without EO (Group B, 33 patients). VUR occurred in 9/10 cases in Group A and in 13/33 cases in group B (Chi-square 7.8658, p = 0.005038). SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). A total of 8/10 cases in Group A and 12/33 cases in Group B were on BM (Chi-square 5.87, p = 0.0015). CONCLUSIONS: EO occurs in approximately in 20 % of male cases with ARM, and recto-urinary communication and should be considered the primary diagnosis in the presence of testicular pain. This could avoid unnecessary surgical exploration, and the family should be counseled about this subject.

Consensus review of best practice of transanal irrigation in adults.

STUDY DESIGN: Review article. OBJECTIVES: To provide a consensus expert review of the treatment modality for transanal irrigation (TAI). METHODS: A consensus group of specialists from a range of nations and disciplines who have experience in prescribing and monitoring patients using TAI worked together assimilating both the emerging literature and rapidly accruing clinical expertise. Consensus was reached by a round table discussion process, with individual members leading the article write-up in the sections where they had particular expertise. RESULTS: Detailed trouble-shooting tips and an algorithm of care to assist professionals with patient selection, management and follow-up was developed. CONCLUSION: This expert review provides a practical adjunct to training for the emerging therapeutic area of TAI. Careful patient selection, directly supervised training and sustained follow-up are key to optimise outcomes with the technique. Adopting a tailored, stepped approach to care is important in the heterogeneous patient groups to whom TAI may be applied. SPONSORSHIP: The review was financially supported by Coloplast A/S.

Acute oligohydramnios: antenatal expression of VURD syndrome?

OBJECTIVE: Oligohydramnios (OA) is nowadays regarded as one of the best markers of renal function (RF) impairment in bladder outlet obstruction (BOO) detected in utero. As such, its onset is usually early and progressive because of decline in fetal urine production. A series of acute OA complicating pregnancies with BOO has never been reported. METHODS: Over a 7-year period, 5 fetuses with in utero suspicion of BOO exhibited an abrupt decrease of amniotic fluid after the 30th week of gestation. RESULTS: All fetuses were delivered by cesarean section: diagnosis was posterior urethral valves in 3 cases, urethral atresia in 1, and prune-belly syndrome in 1. Urologic work-up demonstrated a unilateral vesicoureteral reflux dysplasia (VURD syndrome) in all 5 fetuses. RF at 1 year was normal in 4 fetuses and impaired in 1. CONCLUSIONS: Besides obstetrical reasons, OA may also have acute onset occurring in the presence of anomalies of the urinary tract; although diagnosis is almost always BOO, functional and anatomical characteristics of the urinary tract are those of VURD syndrome with a non-functioning, refluxing renal unit. The associated acute OA/VURD syndrome may represent a milder expression of a pop-off mechanism advocated in this syndrome with a more favorable prognosis than progressive OA detected early in pregnancy.

RANTES stimulates cell-mediated transmission of HIV-1 infection.

We wished to determine the effects of the beta-chemokine RANTES in an established system of cell-mediated transmission of HIV-1, that is, normal human umbilical vein endothelial cells (HUVEC) nonproductively infected with HIV-1, cocultivated with CD4+ T cells to rescue productive infection. The results indicate that the addition of RANTES to HUVEC, either before or after HIV-1 infection, stimulates HIV-1 rescue by CD4+ T cells. However, viral DNA is not increased in HUVEC, suggesting that the stimulation exerted by RANTES could be mediated by events following HUVEC infection. The mechanisms of increase seem to be related to the rescue phase, involving membrane interaction of abortively infected HUVEC with permissive T cells. In fact, a strong upregulation and polarization of intercellular adhesion molecule-1 (ICAM-1) is induced in HUVEC by RANTES, and antibodies against ICAM-1 inhibit HIV-1 rescue by T cells. These results indicate that RANTES, similarly to other inflammatory cytokines, may favor HIV-1 spreading and crossing of blood-tissue barriers by indirect mechanisms involving membrane interactions between nonproductively infected and permissive cells.

Recurrent intussusception of the sigmoid colon caused by a transanal protruding sessile tubulo-villous malignant polyp.

We report a case of a 35 year-old woman with a large malignant sessile tubulo-villous polyp of the proximal end of a dolico-sigmoid colon causing intussusception and transanal prolapsing. The diagnosis presented some difficulties being the intussusception intermittent. The clinical feature had been initially misdiagnosed for a very large bleeding polyp (5 cm in size), protruding out of the anus with a long pedicle in the rectum. The histology of multiple bioptic samples of the lesion revealed malignancy. Conventional radiologic studies (plain x-rays, barium enema) after metal clips had been placed on the head of the polyp before it rose up the colon, led to the correct diagnosis without, nevertheless, documenting intussusception. An elective surgical resection of the dolico-sigmoid colon allowed the correct diagnosis and the curative treatment of the colonic intussusception and the malignancy.

Cysts of the adrenal gland: revision of a 15-year experience.

Ten specimens of adrenal cyst resected during the period 1984-1999 were re-examined. Thorough examination of multiple sections and the use of immunohistochemistry allowed to change the recorded diagnosis in 8 cases: three epithelial cysts and five pseudocysts were redefined as endothelial cysts. All 10 cysts were of the endothelial type. A synthetic review of the current knowledge about the pathogenesis, the classification and the clinical aspects of this rare disease is presented.

Desmoplastic melanoma.

Desmoplastic melanoma is an uncommonly encountered variant of malignant melanoma. Three histological subtypes exist: desmoplastic, neurotropic, and neural transforming. Desmoplastic melanoma commonly presents in conjunction with existing melanocytic lesions or as an amelanotic firm nodule. Local recurrences are common. Thirty patients over a 6-year period were treated at our institution for desmoplastic melanoma. All lesions were treated with local excision. Local recurrence occurred in seven patients (23%) and was treated by aggressive re-excision in each instance. Clinical regional metastasis (lymph nodal basins) were detected in two patients (6%). Distant metastasis (lung) developed in two patients (6%). Twenty-three patients (76%) were found to have desmoplastic subtype, whereas five (17%) had neurotropic subtype. Six patients (20%) had associated pigmented melanotic lesions. Average length of follow-up has been 18 months. Overall survival is 96 per cent. Presentations and histologic diagnosis can sometimes be difficult and misleading. Treatment is aggressive local excision with follow-up necessary to detect resectable recurrent lesions.

Preliminary report of electrogastrography in pediatric gastroresection: can it be predictive of alteration of gastric motility?

BACKGROUND/PURPOSE: Gastric resection is an infrequent surgical procedure in childhood. However, the use of the stomach for bladder augmentation and substitution is well documented. Partial gastrectomy performed in gastrocystoplasty (GCP) involves the greater curvature of the stomach, the same area in which gastric pace-maker cells are known to be placed. The aim of this study was to assess, by electrogastrography (EGG), if subtotal gastric resection can alter gastric motility in children submitted to partial gastrectomy for GCP. METHODS: Gastric electrical activity (GEA) was evaluated in 25 children using EGG: 10 patients (4 boys, 6 girls; mean age, 11.6 years) previously submitted to GCP, and 15 normal subjects (12 boys, 3 girls; mean age, 8.62 +/- 2.77 years) as controls. All patients were submitted to cutaneous EGG; recording GEA for 30 minutes before and after a standard test meal. The percentage of 3 cycles per minute (3CPM), bradygastria, tachygastria, DFIC (dominant frequency instability coefficient), DPIC (dominant power instability coefficient), PDP (period dominant power), PDF (period dominant frequency) were recorded and analyzed using Wilcoxon matched-pair test. Data were considered statistically significant if P <.05. RESULTS: Normal subjects as well as operated patients showed a statistically significant difference in bradygastria (P =.05), PDP and PDF (P =.05) percentage, comparing pre versus postprandial period. In the normal group, 3CPM (P =.0012) and DFIC (P =.0008) were statistically different between the pre- and postprandial period. Patients who underwent GCP did not show any statistically significant difference in 3CPM and DFIC pre- and postprandial. CONCLUSIONS: In normal subjects, GEA showed a complete variation after the meal, whereas in operated patients GEA was impaired and only partially modified after the meal. This observation suggests that in patients with gastric resection, adaptation of the stomach to food ingestion is present but incomplete with respect to normal subjects; it can be caused by surgical removal of the pace-maker cells of the greater curvature. For this reason a follow-up analysis of gastric function is recommended for all patients undergoing GCP.

Continuous (6 hour) urodynamic monitoring in children with neuropathic bladder.

In adults the development of modern equipment for ambulatory monitoring permits long-term evaluation of the lower urinary tract which is more accurate than standard urodynamics (SUD). In children continuous urodynamic monitoring (CUM) has been used infrequently and therefore standardisation of the method has not been previously achieved, nor have the techniques and difficulties of performing 24-hour monitoring been solved. The aim of this study was to identify a technical method of CUM in children which was feasible and to verify its usefulness. For this purpose, we reviewed our preliminary experiences of CUM in children with neuropathic bladder. From March to November 1995 we made an outpatient study of the neuropathic bladders of 11 myelodysplastic children aged 1 to 18 years (average age 10.2 years). 7 of them were males and 4 females. All underwent SUD. With the children resting in bed, a 6-hour CUM (Lectromed MPR-2) was performed using a 4 Fr. microtip intravesical catheter (suprapubic in 3 infants and transurethral in 8 children) and an intrarectal catheter. The parents and/or a nurse monitored the fluid intake and micturition events and recorded the data in a diary. Based on the CUM experience in infants with non-neurogenic bladder dysfunction, the 3 suprapubic studies were done after 12 hours of urethral drainage in order to prevent leakage of urine. In all of the patients we were able to study 2 to 4 bladder fillings during a period of 6 to 8 hours (average 6.5 hours) observation. In 9 of the 11 children the CUM pattern was comparable to the SUD one but in the 2 remaining patients CUM showed uninhibited contractions. Higher uninhibited voiding contraction pressures were recorded in hyper-reflexic bladders. Our preliminary results show that it is feasible to perform CUM in children and that it has advantages over standard cystometry in the investigation of children with neuropathic bladder even if it is carried out for short term (6 hours).

Long-term urological follow-up of occult spinal dysraphism in children.

From 1980 to 1996 we followed up 65 children with occult spinal dysraphism (OSD) out of 85 observed in our urodynamic laboratory. The average age at diagnosis was 4.7 years (range: 1-21 years). In 19 cases (22%) the OSD was associated with anorectal anomalies (ARA). Urinary incontinence or voiding anomalies with urinary tract infection were the presenting symptom in 34 children (49%). At diagnosis all children underwent complete neuro-urological and urodynamic evaluation. Thirty-eight patients underwent neurosurgical correction. Vesicoureteral reflux (VUR) was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required surgery; 2 needed bladder augmentation because of upper tract and renal function deterioration. Urinary incontinence was treated mainly by intermittent catheterization and anticholinergic drugs, but endoscopic treatment was performed in 3 children. At long-term follow-up (2 to 14 years), socially acceptable continence was achieved in 78% of 57 children (8 could not be assessed because they were less than 4 years old). Upper urinary tract deterioration occurred in 15% and renal failure in 7.5%.

Prevalence of hypercontractility in male and female infants with vesico-ureteral reflux.

PURPOSE: To establish whether infants with vesico-ureteral reflux (VUR) have bladder dysfunction, with difference in gender, age at presentation and severity. PATIENTS AND METHODS: 37 infants (24 male and 13 female) aged 2 to 24 months with II degrees to V degrees degree VUR underwent cystometry. Of those, 10 underwent natural filling cystometry. We considered: instability and maximal voiding detrusor pressure (VDP) to be "high" when it exceeded 90 cm H2O. We defined hypercontractility as high VDP and/or instability. RESULTS: The prevalence of hypercontractility was 75% (18/24) in male and 46% (6/13) in female infants (p<0.004). High VDP was found in 50% (12/24) of male and 7% (1/13) of female patients (p < 0.001); no significant difference was found between male (25%) and female ones (38%) with instability alone. The mean VDP was significantly higher in male than in female infants (p < 0.001), in patients < 1 year of age than in older ones (p<0.001) and in severe than in moderate reflux (p<0.006). The mean voiding detrusor pressure of male infants was higher in severe (108+/-46cm H2O) and bilateral (101.3+/-44cm H2O) than in moderate (76+/-24 cm H2O) and unilateral (73.7+/-24 cm H2O) and in infants < 1 year of age (101.7+/-42 cm H2O) than in older ones (70.2+/-21 cm H2O). Natural filling cystometry confirmed the results of standard urodynamic studies. CONCLUSIONS: Bladder dysfunction is confirmed also in infants with reflux, particularly in male younger patients, and it differs in gender. The pathogenesis of congenital reflux is not always a feature of malformation of the vesico-ureteral junction; therefore, patients with bladder dysfunction must be identified early.

Early detection of bladder dysfunction following posterior urethral valves ablation.

Urinary incontinence following Posterior Urethral Valves (PUV) ablation has been attributed in the past to sphincter injury, but it is nowadays accepted that bladder dysfunction (BD) plays a determinant role. In order to assess BD evolution, we have evaluated, from 1982 to 1994, 48 boys with PUV by urodynamics (UD) studies. Age of the patients ranged from 10 months to 15 years. A total of 65 examinations were performed. We considered four groups: 0-3 years; 4-7 years; 8-12 years and > 12 years. Uninhibited detrusor contractions (instability), end filling pressure (EFP), bladder capacity and voiding detrusor pressure were evaluated in order to assess the presence of BD distinguishing it in: hypercontractility, hypocontractility and low-compliant bladder. The results collected confirm a high percentage of BD in PUV boys (71%) and the evolution of hypercontractility versus hypocontractility in 60% of patients considered.

Neurovesical dysfunction due to spinal dysraphism in anorectal anomalies.

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.

Occult spinal dysraphism: neurogenic voiding dysfunction and long-term urologic follow-up

From 1976 to 1994, we followed 55 children with occult spinal dysraphism (OSD). The average age at diagnosis was 4.5 years (range: 24 days - 21 years). In 13 cases the OSD was associated with anorectal anomalies. Urologic symptoms were present at diagnosis in 24 children (43%), but urinary incontinence affected all patients in the evolution of the OSD. At diagnosis, all children underwent complete neurourologic and urodynamic evaluation. Nine required early neurosurgical correction, before 3 years of age. During follow-up, intermittent clean catheterization was started in all patients. Vesicoureteral reflux was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required bladder augmentation because of upper-tract and renal-function deterioration. Endoscopic treatment for urinary incontinence was performed in 3 children. At long-term follow-up (6 to 18 years), socially acceptable continence was achieved in 78% of the children; renal failure occurred in 8. The long-term results were analyzed in order to compare the evolution of urinary continence and renal function in children with OSD with or without neurosurgery.

[Experience in the prophylactic use of ceftriaxone in pediatric surgery]. / Esperienze sull'uso profilattico del ceftriaxone in chirurgia pediatrica.

Authors report on their experience about ceftriaxone in surgical prophylaxis in childhood. 30 patients have been treated, suffering from surgical diseases, either thoracic or abdominal ones, including malignancies. Ceftriaxone was administered once a day; patients received ceftriaxone only once preoperatively: Results have been very satisfactory, the only two complications having been found in two severely immunocompromised patients.

[Indications for the surgical treatment of cavernous angioma of the liver in adults. Report of a case]. / Considerazioni sulle indicazioni al trattamento chirurgico dell'angioma cavernoso del fegato nell'adulto. A proposito di un caso.

The authors report a case of giant cavernous hemangioma of the liver, almost entirely extrahepatic, bulging from the inferior surface of the right lobe into the abdomen, treated surgically on account of worsening symptoms referable to continuing growth. A thorough search of the literature allows a critical review of the surgical indication in this benign pathology, rarely complicated by significant clinical events. Surgery is indicated, in lack of relevant complications, only when significant symptoms, continuing or worsening, are undoubtedly referable to the presence of the angioma.