Talking about sexuality with your own child. The perspective of the parents of children born with arm.

PURPOSE: Evaluate whether parents have ever discussed sexuality with their children with anorectal malformations (ARM), which sexuality issues they think should be addressed and who, in their opinion, should address these issues. METHODS: Parents from the Italian Parents' and Patients' Association for Anorectal Malformation participated in meetings organized by the Association together with local Pediatric Surgical Units and were asked to fill in a questionnaire. RESULTS: 103 parents participated. Overall, 66% of parents had never talked about sex with their children. Children's age was marginally correlated with occasions to talk about sexuality (r = .202, p = 0.53) indicating that the older were the children, the more the parents talked about sexuality. The majority of parents reported that their children should have the possibility to talk about sex with them (72%), psychologists (57%), gynecologists/andrologists (47%), pediatric surgeons (33.5%), surgeons specialized in ARM (39.8%), friends (28%), nurses (24.7%) and teachers (20.4%), respectively. The most important topic they thought their children should address was handling serene sexuality, although the most common topic effectively discussed with them was the conception. CONCLUSION: Psychologists, gynecologists/andrologists, and pediatric surgeons are seen as key resources for talking about sexuality. A great number of parents express the wish that their children had more opportunities to discuss sexual topics with pediatric surgeons.

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.

Tethered cord in patients affected by anorectal malformations: a survey from the ARM-Net Consortium.

PURPOSE: The goal of this study was to determine the degree of consensus in the management of spinal cord tethering (TC) in patients with anorectal malformation (ARM) in a large cohort of European pediatric centers. METHODS: A survey was sent to pediatric surgeons (one per center) members of the ARM-Net Consortium. RESULTS: Twenty-four (86%) from ten different countries completed the survey. Overall prevalence of TC was: 21% unknown, 46% below 15, and 29% between 15 and 30%. Ninety-six agreed on screening all patients for TC regardless the type of ARM and 79% start screening at birth. Responses varied in TC definition and diagnostic tools. Fifty percent of respondents prefer ultrasound (US), 21% indicate either US or magnetic resonance (MRI) based on a pre-defined risk of presenting TC, and 21% perform both. Discrepancy exists in complementary test: 82% carry out urodynamic studies (UDS) and only 37% perform somatosensory-evoked potentials (SSEP). Prophylactic untethering is performed in only two centers (8%). CONCLUSIONS: Survey results support TC screening in all patients with ARM and conservative management of TC. There is discrepancy in the definition of TC, screening tools, and complementary test. Protocols should be developed to avoid such variability in management.

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.

European consensus meeting of ARM-Net members concerning diagnosis and early management of newborns with anorectal malformations.

The ARM-Net (anorectal malformation network) consortium held a consensus meeting in which the classification of ARM and preoperative workup were evaluated with the aim of improving monitoring of treatment and outcome. The Krickenbeck classification of ARM and preoperative workup suggested by Levitt and Peña, used as a template, were discussed, and a collaborative consensus was achieved. The Krickenbeck classification is appropriate in describing ARM for clinical use. The preoperative workup was slightly modified. In males with a visible fistula, no cross-table lateral X-ray is needed and an anoplasty or (mini-) posterior sagittal anorectoplasty can directly be performed. In females with a small vestibular fistula (Hegar size <5 mm), a primary repair or colostomy is recommended; the repair may be delayed if the fistula admits a Hegar size >5 mm, and in the meantime, gentle painless dilatations can be performed. In both male and female perineal fistula and either a low birth weight (<2,000 g) or severe associated congenital anomalies, prolonged preoperative painless dilatations might be indicated to decrease perioperative morbidity caused by general anesthesia. The Krickenbeck classification is appropriate in describing ARM for clinical use. Some minor modifications to the preoperative workup by Levitt and Peña have been introduced in order to refine terminology and establish a comprehensive preoperative workup.

Towards the perfect ARM center: the European Union's criteria for centers of expertise and their implementation in the member states. A report from the ARM-Net.

BACKGROUND: Pediatric surgeons and patient organisations agree that fewer centers for anorectal malformations with larger patient numbers are essential to reach better treatment. The European Union transacts a political process which aims to realize such centers of expertise for a multitude of rare diseases. All the centers on a specific rare disease should constitute an ERN on that disease. ARM-Net members in different countries report on first experiences with the implementation of national directives, identifying opportunities and risks of this process. METHODS: Relevant details from the official European legislation were analyzed. A survey among the pediatric surgeons of the multidisciplinary ARM-Net consortium about national implementation was conducted. RESULTS: European legislation calls for multidisciplinary centers treating children with rare diseases, and proposes a multitude of quality criteria. The member states are called to allocate sufficient funding and to execute robust governance and oversight, applying clear methods for evaluation. Participation of the patient organisations is mandatory. The national implementations all over Europe differ a lot in respect of extent and timeframe. CONCLUSIONS: Establishing Centers of Expertise and a ERN for anorectal malformations offers great opportunities for patient care and research. Pediatric surgeons should be actively engaged in this process.

Extrarenal testicular Wilms' tumor in a 3-year-old child.

We report an extremely rare case of extrarenal testicular Wilms' tumor in a 3-year-old boy with intrabdominal undescended left testis. The patient was admitted because of pain and vomiting, with evidence of a huge abdominal mass. At surgery a large tumor arising from the intrabdominal testis was found. Histology showed the classical triphasic Wilms' tumor elements: epithelial, mesenchymal and blastemal areas. Extrarenal Wilms' tumors account for only 3% of all Wilms' tumors and just -100 cases have been reported in literature. Testicular origin is anecdotic. We present histomorphological, histogenetic, clinical, diagnostic, prognostic and therapeutic features of this rare tumor.

Intravaginal testicular torsion in newborns. To fix or not to fix the contralateral testis?

Scrotal swelling suggesting testicular torsion is a rare urological emergency which requires a clinical urgent evaluation and most of the times must be managed surgically. In newborns it can occur in the postnatal period, usually within the twenty-eighth day of life, or more frequently in utero, during the descent of the testis into the scrotum. Usually its poor fixedness allows the testis an abnormal mobility inside the scrotum, configuring the framework of extravaginal torsion. On the contrary during the perinatal period a twist that takes place inside the tunica vaginalis, known as intravaginal torsion, is extremely uncommon and only few cases are well documented in the literature. Authors present a rare case of intravaginal testicular torsion occurred in perinatal period. In this situation only the early surgical exploration of the scrotum may allow the rescue of the gonad, although in rare cases. Timing of surgical treatment and need for contralateral testicular fixation remain controversial. However since the anatomical defect of the tunica vaginalis can be bilateral the surgical fixation even of the contralateral testis is important, now or later, in order to prevent any future torsion of this gonad. The authors also present a brief review of recent literature on the subject.

Labeling male anorectal malformations: objective evaluation of radiologic imaging before surgery.

Purpose: Prone cross-table lateral x-ray (CTLxR) and colostogram aid surgical planning for anorectal malformations (ARMs) without perineal fistulas. We suggest objective imaging tools to classify ARMs. Methods: Three observers prospectively evaluated CTLxR and colostograms of male ARM patients (2012-2022) without perineal fistulas. The level of the rectal pouch was estimated with pubococcygeal (PC) and ischiatic (I) lines. On CTLxR, we described the "pigeon sign", defined as the rectal pouch ending with a beak-like image, suspicious for a rectourinary fistula. ARM was defined as rectobulbar when the rectal pouch was below the I line, rectoprostatic when between PC and I lines, and rectovesical when above the PC line. Concordance was assessed with Fleiss' kappa. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the "pigeon sign" were calculated. Results: Thirteen patients were included in this study. The interobserver agreement on CTLxR was 69.2% (k = 0.54) on pouch ending, 84.6% (k = 0.69) on the "pigeon sign", and 76.9% (k = 0.69) on diagnosis; concordance between observers and intraoperative diagnosis was 66.6% (k = 0.56). The "pigeon sign" had 75% sensitivity, 100% specificity, 100% PPV, and 50% NPV. The interobserver agreement on colostograms was 84.6% (k = 0.77) on pouch ending and 89.7% (k = 0.86) on diagnosis; concordance between observers and intraoperative diagnosis was 92.3% (k = 0.90). Conclusion: PC and I lines and the "pigeon sign" are useful tools in examining CTLxR and colostograms. Adequate CTLxR interpretation may modify surgical strategy.

Anorectal malformations and neurospinal dysraphism: is this association a major risk for continence?

BACKGROUND/PURPOSE: Anorectal malformations (ARM) are a spectrum of defects ranging from the very minor ones, with an excellent functional prognosis, to those that are more complex, often associated with other anomalies, difficult to manage and with a poor functional outcome. A significant number of these children suffer from fecal and urinary incontinence despite major advances in the management of ARM patients have significantly improved the quality of life. The role of sacrovertebral anomalies/dysraphism (SD) and neurospinal cord anomalies/dysraphism (ND) associated with ARM on the continence of these patients is still controversial. The authors made a review of their experience in a period of 5 years, focusing on the role of neurospinal cord anomalies in patients with ARM. MATERIALS: At colorectal clinic of our department of pediatric surgery 215 patients who underwent a procedure of posterior sagittal anorectoplasty for ARM are followed-up in a multidisciplinary clinic. Among them 60 patients with either SD or ND were documented. In 37 patients the anomaly involved the spinal cord (ND). 12 of these 37 patients underwent neurosurgical treatment and 25 were managed conservatively. Data collected from their follow-up were analyzed and compared, focusing on their bowel and urinary continence. RESULTS: All 37 patients acquired regular bowel movements with an appropriate bowel management according to Peña's protocols. Urinary incontinence required clean intermittent catheterization in four cases. None of the patients who did not receive neurosurgical treatment developed acute complications due to the progression of the neurospinal anomaly, like acute urinary retention, orthopedic and motility problems or acute hydrocephalus. From literature review we were unable to find good evidence that the presence of ND worsens the functional prognosis of patients with ARM. We were also unable to find convincing evidence to support the practice of prophylactic neurosurgical procedures. CONCLUSIONS: The present study supports the theory that for ARM patients the prognosis in terms of continence depends mainly on the type of malformation and is not complicated by the association with ND. In our series neurosurgical treatments did not have any effect in improving the continence of ARM patients and a conservative management of ND did not expose the patients to the sequelae of progressive deterioration, reported elsewhere, requiring rescue neurosurgery. We believe that the correct practice of pediatric surgeons following-up ARM patients is a protocol which includes appropriate investigations to detect the presence of a SD or ND and, once these entities are detected, it is mandatory to manage the patient with a multidisciplinary team, where a conservative non-operative management is initially justified and advocated in the absence of neurosensorymotor symptoms.

Completely isolated enteric duplications cysts: a survey of four cases.

Enteric duplication cysts are uncommon congenital abnormalities with epithelial lining. They are cystic or tubular structures intimately attached to a portion of the gastrointestinal tract; they are usually located on the mesenteric site of the digestive tract sharing common blood supply. Isolated cystic duplications are an extremely rare variant with their own blood supply: in literature only five cases have been reported. We present our four cases series of this uncommon anomaly.

The impact of perioperative care on complications and short term outcome in ARM type rectovestibular fistula: An ARM-Net consortium study.

BACKGROUND: The impact of perioperative care interventions on postreconstructive complications and short-term colorectal outcome in patients with anorectal malformation (ARM) type rectovestibular fistula is unknown. METHODS: An ARM-Net consortium multicenter retrospective cohort study was performed including 165 patients with a rectovestibular fistula. Patient characteristics, perioperative care interventions, timing of reconstruction, postreconstructive complications and the colorectal outcome at one year of follow-up were registered. RESULTS: Overall complications were seen in 26.8% of the patients, of which 41% were regarded major. Differences in presence of enterostomy, timing of reconstruction, mechanical bowel preparation, antibiotic prophylaxis and postoperative feeding regimen had no impact on the occurrence of overall complications. However, mechanical bowel preparation, antibiotic prophylaxis ≥48 h and postoperative nil by mouth showed a significant reduction in major complications. The lowest rate of major complications was found in the group having these three interventions combined (5.9%). Multivariate analyses did not show independent significant results of any of the perioperative care interventions owing to center-specific combinations. At one year follow-up, half of the patients experienced constipation and this was significantly higher among those with preoperative mechanical bowel preparation. CONCLUSIONS: Differences in perioperative care interventions do not seem to impact the incidence of overall complications in a large cohort of European rectovestibular fistula-patients. Mechanical bowel preparation, antibiotic prophylaxis ≥48 h, and postoperative nil by mouth showed the least major complications. Independency could not be established owing to center-specific combinations of interventions. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: III.

Ductility improvement due to martensite α' decomposition in porous Ti-6Al-4V parts produced by selective laser melting for orthopedic implants.

Ti-6Al-4V parts obtained by selective laser melting typically have an acicular α' martensitic microstructure whose ductility is low. Thus, post-heat treatments are useful for increasing ductility. In this work, the effects of sub-ß-transus heat treatments on the mechanical properties of Ti-6Al-4V parts with porous structures are correlated with martensite α' phase decomposition. The precipitation of ß phase and the gradual transformation of α' into α phase by the diffusion of excess vanadium from α' to ß phase are proposed to be the main events of martensite α' phase decomposition in parts fabricated by selective laser melting. The heat treatment performed at 650°C for 1h produced no microstructural changes, but the samples treated for at the same temperature 2h showed a fine precipitation of ß phase along the α' needle boundaries. The heat treatment performed at 800°C for 1 or 2h produced a fine α+ß microstructure, in which ß phase are present as particles fewer in number and larger in size, when compared with the ones present in the sample heat-treated at 650°C for 2h. Heat-treatment of the parts at 800°C for 2h proved to be the best condition, which improved the ductility of the samples while only slightly reducing their strength.

[Intestinal intussusception: easy diagnosis in the 21st Century? Outcome of five years experiences]. / Invaginazione intestinale: una diagnosi facile nel 21 degrees secolo? Outcome degli ultimi 5 anni.

UNLABELLED: Intussusception is an entity well codify in pediatric surgery, with guidelines well defined since years in terms of imaging and procedures. MATERIAL: From January '99 to Dec. '03, 25 children were admitted to the Department of Pediatric Surgery of the Children's Hospital of Milan. All the aspects related to the pathology have been evaluated in the study. RESULTS: 25 patients have been studied. 23/25 presented severe pain, 19/25 normal bowel movements in the last 3 hours and in 9/25 a mass were detected at clinical examination.19/25 were submitted to laparotomy after failure of reduction through barium enema, and in 17/19 a manual reduction were performed. Only 2 patients required a bowel resection with primary anastomosis. The analysis of the delay of diagnosis, the delta-T between beginning of the symptoms and first medical evaluation was of 16,23 h. (3-72), and the delta-T between the first evaluation and diagnosis was 18,7 h. The interval between diagnosis and surgery was of 4,3. CONCLUSION: Authors believe that intussusception still represent a challenge for medical and surgical emergency team, supported by the data in literature. Imaging procedures must not be constricted in case of minimal suspicions of intestinal intussusception.

A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

Solid and cystic tumor of the pancreas--case report.

Pancreatic tumors are rarely present in childhood. The authors present a case of papillary-cystic tumor in a 13-year-old girl, treated by partial pancreatoduodenectomy, with preservation of the pylorus. The histologic pattern was of a papillary cystic tumor without evident atypical nuclei. One year after operation, the girl is well without any finding of disease.

[Intestinal resection in children: personal experience and suggested follow-up. Preliminary results]. / Resezioni intestinali in età pediatrica: esperienza personale e proposta di follow-up. Risultati preliminari.

In the paediatric field cases of patients subjected to massive intestinal resection (M.I.R.) are more and more frequent; this survival of individuals with a small intestine shorter than 30 centimeters is not to be considered exceptional. Without going as far as such extreme limits of short residual intestine, intestinal resections contributed to create a new pathology represented by those patients who, once they have been subjected to particularly extended intestinal resections, need a particularly responsible nutritional therapy, sometimes with very long hospitalizations. The present study is to appraise the nutritional parameters and the residual absorption capacity of a group of patients in pediatric age subjected to intestinal resection, in order to draw up a record enabling to monitor, as time goes on, the intestinal functionality of those patients and to observe as early as possible the consequences of an alteration of intestinal physiology while creating the premisis for appropriate measures of nutritional supplementing.

[Massive intestinal resection in the newborn: nutritional and feeding problems]. / Resezioni intestinali massive nel neonato: problemi di nutrizione e di alimentazione.

Authors present their experience about nutritional treatment of short bowel syndrome in newborns. Their results seem to demonstrate that right approach to this problem is to start enteral nutrition as soon as possible, using mainly human milk and polimeric nutritional solutions. There are several experimental evidences that this kind of nutritional approach promote intestinal adaptation following massive intestinal resections.

[Spontaneous regression of an adrenal mass detected in utero]. / Risoluzione spontanea di massa surrenalica diagnosticata in utero.

The case of a left adrenal mass detected by ultrasonography in a male fetus at the 37th week of gestation is presented. After an uneventful delivery at the 38th gw, the newborn was sonographically monitored, and during the follow-up clinical evaluations were performed, in order to make a differential diagnosis between an adrenal hemorrhage and a congenital neuroblastoma. VMA and HVA were within normal levels, and MIGB scan was negative. A MR of the the abdomen performed at the age of one month was suggestive for an adrenal hemorrhage in involution. We therefore decided to keep monitoring the mass by ultrasound, avoiding any surgical approach. The sonographic complete disappearance of the disease was documented at the end of the fourth month.

[An unusual case of macroscopic hematuria in pediatric age]. / Un caso insolito di ematuria macroscopica in età pediatrica.

An unusual case of macroscopic hematuria in a 14 year old boy is presented. At the time of the first hospital admission, no urinary tract infection could be demonstrated, in spite of the associated symptoms of stranguria and dysuria. At ultrasound examination, only a mild thickening of the upper bladder wall was detected, and cystoscopy showed a huge oedema and inflammation of the mucosal layer. The biopsy of the bladder was characterized by a definite eosinophilic infiltration; due to this particular hystologic pattern, the diagnosis of eosinophilic cystitis was made. In the following months, the boy did not improve. Recurrent hematuria occurred, and a pseudo-polypoid mass in the inner bladder wall was detected at ultrasonography. A limited resection of the vesical dome was performed, to remove completely the mass. The hystologic examination showed Schistosoma Haematobium eggs in the bladder wall, with a typical granulomatous reaction. The post-operative course was uneventful, and the child was completely cured after Praziquantel treatment. The Authors underline the need to take into account Schistosomiasis in cases of hematuria, particularly when this symptom affects boys coming from countries where Bilharziasis is endemic.