Megacystis-microcolon-intestinal hypoperistalsis syndrome: don't forget the bladder.

PURPOSE: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a well described clinical condition, but reports are focused on microcolon and intestinal hypoperistalsis, while data on bladder management are scant. Aim of the study is to present urological concerns in MMIHS. METHODS: Retrospective evaluation of clinical data on urological management of MMIHS patients treated in the last 10 years. RESULTS: Six patients were enrolled (3 male, 3 female). Three girls had prenatal diagnosis of megacystis (1 vesicoamniotic shunt was placed). All patients had genetic diagnosis: 5 had ACTG2 gene mutations and 1 MYH11 mutation. All patients were addressed to our attention for urinary symptoms, such as urinary retention, urinary tract infections, acute renal injury. Two patients presented frequent stoma prolapses. All children underwent a complete urological evaluation, and then started a bladder management protocol (clean intermittent catheterization, via urethra or cystostomy-tube placement), with improvement of urinary infections, upper urinary tract dilation and stoma prolapses, if present. All patients had good renal function at last follow-up. CONCLUSION: We believe that MMIHS patients must be addressed soon and before onset of symptoms for a multidisciplinary evaluation, including an early assessment by a pediatric urologist expert in functional disorder, to preserve renal function at its best.

Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association.

PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.

Fixed the gap, solved the problem? Eating skills in esophageal atresia patients at 3 years.

Although eating problems have been described as long-term morbidities of esophageal atresia (EA), there have been few studies exploring eating outcomes in children born with EA as primary aim. Parents of children operated on for EA in our Institution from January 2012 to January 2016, answered a telephone structured interview developed specifically to conduct the present study, assessing eating skills at 3 years of age. Clinical data were collected from children's medical records. Parents (45 mothers and 6 fathers) of 51 children (male = 34; female = 17) with a median age of 3.5 years form the object of the study. Considering eating problems, parents reported that 23 children (45%) still have episodes of choking during meals at 3 years of age, 9 (45%) of these have more than one episode a week, and 19 parents (39%) reported higher levels of anxiety during mealtimes. Forty-four children (86%) were described by their parents as able to eat alone, 32 (65%) accepted all food textures and 45 (90%) was described as curious about food (3 years). Forty-three (86%) parents let their children eat with other people. Correlations showed that weaning age was significantly associated with number of dilatations (rs = 0.35, P = 0.012), days of mechanical ventilation (rs = 0.40, P < 0.001), and presence of gastrostomy tube at discharge (rs = 0.45, P < 0.001). Chewing age resulted associated with number of dilatations (rs = 0.34, P < 0.01) and days of mechanical ventilation (rs = 0.38, P < 0.01). Presence of choking episodes was associated with curiosity about food (rs = 0.29, P < 0.05), while frequent choking episodes were associated with higher parental anxiety during mealtimes (rs = 0.45, P < 0.05). In order to prevent delay in the achievement of eating developmental milestones in children operated on of EA, we advocate a dedicated preventive intervention from birth to follow-up.

Couples Facing the Birth of a Newborn with a Congenital Anomaly: PTSD Symptoms in the First Year.

OBJECTIVE: The objective of this study was to assess the presence of posttraumatic stress disorder (PTSD) symptoms in parental couples of newborn requiring early surgery at 6 and 12 months after birth. STUDY DESIGN: A longitudinal study was set up from January 2014 to June 2015. As a measure of PTSD, we used the Italian version of the Impact of Event Scale-Revised (IES-R). RESULTS: Thirty-four couples form the object of the study. At 6 months, half of mothers (52.9%) and fathers (44.1%) reported traumatic stress symptoms above the clinical cutoff. Percentages remained stable at 12 months. When parental gender and length of follow-up were compared with two-factor analysis of variance, none had an impact on IES-R score, nor an interaction between these factors was found. A significant correlation of IES-R total score was present within the couple both at 6 and 12 months (6 months-r: 0.6842, p < 0.0001 and 12 months-r: 0.4045, p = 0.0177). CONCLUSION: Having a child with a repaired malformation represents a complex prolonged stressful situation with persistent burden for both parents who are at high risk of developing PTSD symptoms.

Does Ventilatory Time Retain Its Validity in Predicting Neurodevelopmental Outcome at Two Years of Age in High-Risk Congenital Diaphragmatic Hernia Survivors?

Objective To evaluate if in high-risk non-extracorporeal membrane oxygenation (ECMO)-treated congenital diaphragmatic hernia (CDH) survivors, ventilatory time (VT) is correlated to and can be used as clinical marker of neurodevelopmental delay at 2 years of age. Study Design Cohort study was conducted between 2008 and 2012. Mental, motor, and language development were assessed by the Bayley Scales of Infant and Toddler Development III. The correlation between VT and neurodevelopmental outcome (NDO) was analyzed using Pearson's test. Receiver operating characteristic (ROC) analysis was performed to determine the accuracy and best cutoff value of VT to predict the risk of neurodevelopmental delay. Statistical significance was set at p < 0.05. Results A total of 49 patients form the subject of this study. VT during first admission was inversely correlated with cognitive (r = -0.4116; p = 0.0033), motor (r = -0.4241; p = 0.0024), and language development (r = -0.3564; p = 0.0119). Using ROC curve analysis, VT was a significant predictor for neurodevelopmental delay in the cognitive (area under the curve [AUC]: 0.864, sensitivity: 100; specificity: 66.67; p < 0.0001) and motor (AUC: 0.902; sensitivity: 100; specificity: 73.17; p < 0.0001) scales, but not in the language scale. The best cutoff value for both scales was 9 days. Conclusion Within a population of high-risk non-ECMO-treated CDH survivors, VT appears to retain its validity as a clinical marker of adverse NDO in cognitive and motor domains.

Seeing Their Children in Pain: Symptoms of Posttraumatic Stress Disorder in Mothers of Children with an Anomaly Requiring Surgery at Birth.

Objective Assess the presence of posttraumatic stress disorder (PTSD) symptoms in mothers of newborns requiring early surgery. Study Design Mothers of newborns operated on for a congenital anomaly underwent a semi-structured interview on their experience 6 months postpartum. Interviews were audiotaped, transcribed verbatim, and analyzed for symptoms of the three major criteria of PTSD: re-experiencing, avoidance, and heightened arousal. Results A total of 120 mothers took part in the study; their children were affected by one of the following congenital anomaly: esophageal atresia (n = 29); congenital diaphragmatic hernia (n = 38); midgut malformations (n = 38); and abdominal wall defects (n = 15). Two mothers did not show any symptoms; 12 mothers (10%) had one posttraumatic symptom, 77 (64.2%) had two, and 29 (24.2%) had three. Overall, 106 mothers (88.4%) presented at least two symptoms. Conclusion PTSD can be considered a useful model to describe and comprehend mothers' reactions in this specific population. Preventive interventions and dedicated follow-up program should be offered to these families.

Prenatal Features of Bladder Agenesis in a Female Fetus with Genital Transposition.

Bladder agenesis is a rare condition, mostly affecting females, where diagnosis is usually made in infancy when investigating urinary incontinence. Neonatal cases are uncommon, and none have been reported antenatally. The few male patients with this condition rarely survive: among associated anomalies, different degrees of penoscrotal transposition are the most evident feature. The association of genital transposition in a female infant with prenatal description of bladder agenesis has not been previously reported. Early diagnosis is important, enabling planning of surgical reconstruction early in life and appropriate parental counseling.

Surgical gastrointestinal anomalies including diaphragmatic hernia: Does type of anomaly affect neurodevelopmental outcome?

OBJECTIVE: To describe short-term neurodevelopmental outcome of infants operated on for congenital anomalies (CA) and assess the impact of type of CA on the outcome. STUDY DESIGN: From 2008 to 2010 newborns operated on for CA were enrolled in a cross-sectional follow-up study including three distinct groups: infants of 6 months (group A), infants of 12 months (group B), and children of 24 months (group C). Each group was divided into five subgroups: (1) esophageal atresia; (2) congenital diaphragmatic hernia; (3) midgut malformations; (4) abdominal wall defects; (5) colorectal malformations. Each group of patients underwent a neurodevelopmental evaluation with Bayley III. RESULTS: In all, 150, 156, and 84 babies were enrolled in groups A, B, and C, respectively. Mean (standard deviation) Mental Scale score was 94.65 (8.75), 98.76 (11.03), and 100.60 (12.04) in groups A, B, and C. Mean (standard deviation) Motor Scale score was 96.89 (11.62), 99.23 (14.83), and 103.60 (12.90) in groups A, B, and C. No significant differences were found among the five subgroups considered. CONCLUSION: Regardless of type of malformation, short-term neurodevelopmental outcome of children with gastrointestinal anomalies including diaphragmatic hernia falls within normal range, suggesting that neither being born with a CA nor its type is per se a risk factor for neurodevelopmental delay.

Spinal ultrasound in patients with anorectal malformations: is this the end of an era?

PURPOSE: Even if lumbar magnetic resonance imaging (MRI) is considered the gold standard in the diagnosis of occult spinal dysraphism (SD) in patients with anorectal malformations (ARMs), spinal ultrasound (US) performed up to 5 months of life have been largely used as a screening test. The aim of the present study was to evaluate the accuracy in terms of sensibility and specificity of neonatal US to detect occult SD in patients with ARMs. METHODS: Retrospective analysis of all patients treated for ARMs between 1999 and 2013 at our institution who underwent both spinal US (up to 5 months of life) and MRI. Sensibility and specificity have been calculated for US based on MRI results. RESULTS: Of 244 patients treated for ARMs at our institution, 82 (34 females, 48 males) underwent both the imaging studies and have been included in this study. ARMs types were: anal stenosis (7), recto-vestibular fistula (19), recto-perineal fistula (3) and cloaca (5) in female and imperforate anus (7) recto-perineal fistula (14), recto-urethral fistula (22), recto-vesical fistula (5) in males. Forty-seven patients (57, 3 % of total, 18 females, 29 males) had some occult SD (tethered spinal cord, spinal lipoma, syringomyelia) at MRI. Only 7 (14, 8 %) patients of those with spinal anomalies at MRI had pathological US studies. In our population, sensibility and specificity of US for diagnosis of occult SD were, respectively, 14, 8 and 100 %. CONCLUSION: Since it is well known that a screening test must have a high sensibility, our data suggest that spinal ultrasound is not suitable as a screening test for occult spinal dysraphism in patients with ARMs. Furthermore, we strongly advise against the use of US as a screening test for spinal dysraphism to prevent a false sense of security in physician and patients' families.

Lower Urinary Tract Dysfunction in Pediatric Patients with Multiple Sclerosis: Diagnostic and Management Concerns.

BACKGROUND: Multiple sclerosis (MS) is increasing in the pediatric population and, as in adults, symptoms vary among patients. In children the first manifestations can sometimes overlap with acute neurological symptoms. Urological symptoms have not been much studied in childhood. We shared our experience with MS urological manifestation in children. METHODS: This article is a retrospective evaluation of all children with MS, according to the Krupp criteria, who also present with urological symptoms. We collected demographic and clinical history, the MR localization of demyelinating lesions, urological symptoms, and exams. RESULTS: We report on six MS pediatric cases with urological manifestation. Urinary symptoms, characterized by urinary incontinence in five patients and urinary retention in one patient, appeared in a different time frame from MS diagnosis. Urodynamic exams showed both overactive and underactive bladder patterns. Treatment was defined according to lower urinary tract dysfunction, using clean intermittent catheterization, oxybutynin, and intradetrusor Onabotulinum Toxin-A injection. A low acceptance rate of invasive evaluation and urological management was observed. CONCLUSIONS: The MS diagnosis was traumatic for all our patients. We believe it is important to address urological care in young people from the time of diagnosis for prompt management; it could be useful to include a pediatric urologist in multidisciplinary teams.

Lack of institutional pathways for referral: results of a survey among pediatric surgeons on prenatal consultation for congenital anomalies.

OBJECTIVE: This paper aimed to assess pathways through which pediatric surgeons receive couples for prenatal consultation after prenatal diagnosis. METHOD: A questionnaire was mailed to pediatric surgical centers to assess the following: (1) surgical caseload per year; (2) number of centers in which prenatal consultation is offered; (3) presence of a 'structured' prenatal consultation clinic; (4) number of consultations per year; (5) pathways for referral to the pediatric surgeon; and (6) the availability of psychological counseling. RESULTS: Response rate was 81%, (42/52 centers). Thirty-eight centers (93%) offered prenatal consultation. Seven centers (18%) reported to have a 'structured' clinic in terms of time and location. In 13 centers (34%), 1-9 consultations were carried out, from 10 to 19 in 18 centers (47%), from 20 to over 50 in 7 centers (18%). In 34 centers, internal referrals from the obstetric departments were counseled, and in 28 centers, there were also external referrals. Eleven centers reported that couples were self-referred. Information regarding prenatal counseling was available on the institutional website in 10/38 (26%) centers. Psychological counseling was available in 36 centers. CONCLUSION: Despite the fact that the majority of pediatric surgical centers provides prenatal consultation, caseloads are very variable as are referral modalities.

Long-term bladder function followup in boys with posterior urethral valves: comparison of noninvasive vs invasive urodynamic studies.

PURPOSE: We studied whether noninvasive urodynamic evaluation can be as effective and safe as invasive urodynamics in detecting lower urinary tract dysfunction and in preventing late onset renal failure during long-term management of boys with posterior urethral valves. MATERIALS AND METHODS: We evaluated 47 boys with posterior urethral valves using repeat urodynamics. A total of 28 patients with followup of at least 3 years and repeat evaluation of serum creatinine were included in the study. The first 14 boys in the series underwent cystometry and pressure-flow study at least every 3 years (group A), and the remaining 14 patients were monitored annually from age 5 with bladder diary, uroflowmetry, post-void residual urine on ultrasound and serum creatinine (group B). Lower urinary tract dysfunction and serum creatinine were compared (Fisher exact test and Mann-Whitney test) between groups A and B, and by stratifying patients into subgroups by age (5 to 6, 7 to 13 and older than 13 years). In all patients urodynamic diagnosis of lower urinary tract dysfunction was matched and confirmed with lower urinary tract symptoms. RESULTS: During followup the prevalence of lower urinary tract dysfunction did not differ significantly between group A (71% in boys 5 to 6, 43% in boys 7 to 13 and 85% in boys older than 13 years) and group B (36%, 43% and 60%, respectively). Late onset renal failure was observed in 2 boys in group A and 2 in group B. CONCLUSIONS: Noninvasive urodynamic evaluation seems to be as safe and effective as invasive urodynamic study in the long-term management of boys with posterior urethral valves. Based on these findings, invasive urodynamics may be reserved for cases of progressive deterioration of lower urinary tract dysfunction or renal function.

Posterior urethral valves: Role of prenatal diagnosis and long-term management of bladder function; a single center point of view and review of literature.

Posterior Urethral Valves (PUV) are the most common cause of lower urinary tract obstruction. More severe forms are detected early in pregnancy (mainly type I), while other forms are usually discovered later in childhood when investigating lower urinary tract symptoms. Bladder dysfunction is common and is associated with urinary incontinence in about 55% (0%-72%). Despite the removal of the obstruction by urethral valve ablation, pathological changes of the urinary tract can occur with progressive bladder dysfunction, which can cause deterioration of the upper urinary tract as well. For this reason, all children with PUV require long-term follow-up, always until puberty, and in many cases life-long. Therefore, management of PUV is not only limited to obstruction relief, but prevention and treatment of bladder dysfunction, based on urodynamic observations, is paramount. During time, urodynamic patterns may change from detrusor overactivity to decreased compliance/small capacity bladder, to myogenic failure (valve bladder). In the past, an aggressive surgical approach was performed in all patients, and valve resection was considered an emergency procedure. With the development of fetal surgery, vesico-amniotic shunting has been performed as well. Due to improvements of prenatal ultrasound, the presence of PUV is usually already suspected during pregnancy, and subsequent treatment should be performed in high-volume centers, with a multidisciplinary, more conservative approach. This is considered to be more effective and safer. Primary valve ablation is performed after clinical stability and is no longer considered an emergency procedure after birth. During childhood, a multidisciplinary approach (pediatric urologist, nephrologist, urotherapist) is recommended as well in all patients, to improve toilet training, using an advanced urotherapy program with medical treatments and urodynamic evaluations. The aim of this paper is to present our single center experience over 30 years.

Current state of nerve stimulation technique for lower urinary tract dysfunction in children.

PURPOSE: A variety of electrical nerve stimulation methods has been used through the years to treat lower urinary tract dysfunction. Relevant literature was reviewed to analyze techniques and available biomedical devices, technique applicability, indications and usefulness in pediatrics. MATERIALS AND METHODS: An extensive search was performed on PubMed® and MEDLINE® for scientific publications on intravesical, transcutaneous, sacral spine and root, and tibial nerve stimulation in children with lower urinary tract dysfunction of nonneurogenic and neurogenic origin. Relevant articles and controlled studies in adult patients were also considered. The search covered the period 1990 to 2009 and we found approximately 400 articles, of which 29 related to pediatrics. RESULTS: Due to feasibility problems with placebo studies the majority of the studies were noncontrolled, some of them clinical trials on acute urodynamic changes during electrical stimulation or experimental research in animals. Overall only a few randomized trials were found. Regarding types of electrostimulation and indications in children the recent literature emphasizes stimulation far from the anal-genital region, such as sacral transcutaneous electrical nerve stimulation, mainly for refractory overactive bladder. Intravesical stimulation is the procedure of choice to enhance sensation in patients with incomplete neurogenic lesions. Percutaneous tibial nerve stimulation is tolerated by children but has been poorly studied. Sacral neuromodulation using implanted devices remains questionable and needs further clarification of its indications. Magnetic stimulation has rarely been used in children to date. More experimental studies are needed to assess the method of action and refine the parameters of stimulation. CONCLUSIONS: Clinical controlled trials vs sham devices and predictable variables for successful response are urgently needed to address an apparently renewed focus on the use of nerve stimulation in the treatment of pediatric lower urinary tract symptoms.

A simplified technique for botulinum toxin injections in children with neurogenic bladder.

PURPOSE: Botulinum toxin type A has revolutionized the treatment of neurogenic bladder dysfunction. The original injection technique used a rigid cystoscope and a flexible collagen needle. To date botulinum toxin type A injection techniques have not been standardized. We present our experience in pediatric patients using a new flexible injection system. MATERIALS AND METHODS: We treated 24 patients 3.8 to 17.5 years old who had neurogenic bladder dysfunction with botulinum toxin type A bladder and/or sphincter injection using a rigid cystoscope and the new N-DO™ endo-injector needle system. Another 24 patients 3.6 to 17.8 years old were treated with a 3.7Fr standard flexible needle and served as controls. Operative time, hospital stay, complications and efficacy were considered. Selection criteria and treatment were the same in the 2 groups. The 10 IU/kg dose was determined according to European Association of Urology guidelines. RESULTS: All patients received botulinum toxin type A bladder injection while 11 patients in the endo-injector group and 5 controls also received urethral injection. In the endo-injector needle and control groups average operative time was 12.4 and 17.3 minutes for the bladder, and 5.1 and 10.1 minutes for the urethra, respectively (each p <0.05). All patients were discharged home the day after the procedure. No complications were observed. Urodynamics revealed an average maximum detrusor pressure decrease of 25 and 21 cm H(2)O, and an average bladder capacity increase of 75 and 80 ml in the endo-injector and control groups, respectively (p not significant). CONCLUSIONS: While retaining efficacy, the endo-injector needle technique appears to be more rapid than the standard procedure for botulinum toxin type A injection for neurogenic bladder dysfunction. Whether patients may be treated with sedation only remains to be clarified.

Delayed complete repair of exstrophy with testosterone treatment: an alternative to avoid glans complications?

PURPOSE: Since 1999, complete primary repair of exstrophy has represented a valid alternative in the treatment of exstrophy patients, offering one- stage reconstruction for all components of this malformation in newborns. The vast majority of cases are currently approached within 48/72 h of life, and risk of vascular injury to penile glans and/or corpora has been reported with increased frequency with this procedure. We report our initial experience with a delayed approach to complete repair, with bladder plate left intact and taken care at home by the parents, while awaiting for the patient to reach adequate weight. Delayed approach also enabled us to preoperatively stimulate phallic size with testosterone, a treatment which was so far confined only to redo or failed cases. METHODS: Six male exstrophy patients were treated over a three-year (2007-2009) period. After initial workup, newborns were discharged home with bladder plate taken care by the parents. A weight of 4,500 g was arbitrarily deemed satisfactory for surgery. While at home, patients underwent preoperative testosterone stimulation (testosterone enanthate, four biweekly administrations of 100 mg/per square meter body surface). In each case biopsies of bladder mucosa were taken at time of surgery. Complications, age at surgery, increases in phallic size were extracted from clinical and surgical case notes. RESULTS: Weight at surgery ranged from 4,510 to 5,600 g. Age range was 43-91 days. Mean increase in phallic size after testosterone stimulation was 8.3 mm. Three complications were observed: two were suprapubic fistulas, of these, one closed spontaneously and one required surgery subsequently. In one fascial dehiscence emergency closure was needed. Hypospadias occurred in all patients. All histologic specimens demonstrated a mildly inflamed bladder mucosa. CONCLUSIONS: Delayed repair of bladder exstrophy allows to approach patients who have reached adequate weight and stabilization; if adequately cared for bladder plate shows minimal inflammation at surgery and can be managed by the parents at home. Deferring surgery also offers the advantages of preoperative testosterone stimulation, promotion of mother-baby relationship as well as of transfer to Centers with adequate experience and proficiency in all aspects of bladder exstrophy reconstruction.

Robotic Excision of the Vagina in a 46 XX DSD Male Patient. First Pediatric Report.

The Disorders of Sex Differentiation (DSD) represent a wide range of congenital anomalies of the genitalia. Surgical treatment of these cases may become a challenge. We present a case of a 16-year-old boy with 46 XX DSD, SRY negative, presented with persistent dribbling incontinence, recurrent UTI and perineal pain. Past medical history included right orchiectomy, laparoscopic excision of uterus, fallopian tubes and left streak gonad at another institution at the age of 2 years. The native vagina was left in place. VCUG confirmed the presence of the residual vagina (8 cm in maximum length), connected with the bulbar urethra. Robotic-assisted laparoscopy of the vagina was performed with satisfying short and long-term results.

Pediatric medical traumatic stress (PMTS) in parents of newborns with a congenital anomaly requiring surgery at birth.

BACKGROUND: Pediatric medical traumatic stress (PMTS) is a psychological and physiological response of children and their families to pain, serious illness, and invasive medical procedures. We aimed to apply the PMTS model to parents of newborns operated at birth for a congenital malformation and to identify clinical and sociodemographic risk factors associated with PMTS symptoms at 6 months. METHODS: We designed a cross-sectional study to assess PMTS symptoms (avoidance, arousal, reexperiencing) in parents of six months children operated on for a congenital anomaly, with the Italian version of the Impact of Event Scale - Revised (IES-R). RESULTS: One-hundred-seventy parents form the object of the study. Eighty-two parents (48.2%) fell over the clinical cut-off. Ventilatory time (p = 0.0001), length of hospital stay (p = 0.0001), associated anomalies (p = 0.0002), medical devices at discharge (p = 0.0001) and Bayley motor scale (p = 0.0002) were significantly correlated with IES-R Total and Subscale Scores. Multivariate linear regression showed length of hospital stay and number of associated anomalies as significant predictors of IES-R Scores. CONCLUSIONS: Regardless the type of anomaly and sociodemographic factors, it is the clinical history of the child which seems to predict the severity of PMTS symptoms in this population of parents. PMTS represents a useful model to describe the psychological reactions of parents of newborns operated at birth for a congenital malformation. NICU and outpatient pediatric staff should be aware of risk factors to identify families who may request early multidisciplinary interventions since the first admission. LEVEL OF EVIDENCE: Prognosis study, level II.

A Simplified Management of Transverse Testicular Ectopia in Patients with Persistent Mullerian Duct Syndrome.

Persistent müllerian duct syndrome (PMDS) in the majority of cases is discovered during surgery for inguinal hernia or cryptorchidism. A transverse testicular ectopia (TTE) with cryptorchidism may be very rarely associated to PMDS. Assuming that müllerian remnants have a very low malignant degeneration potential if compared to the malignancy risk of an undescended and not relocated testis, we describe a simplified surgical technique of orchiopexy that avoids an extensive anatomical dissection, in this way minimizing the risk of losing the deferential blood supply to the testis.