Predictors of Gastrointestinal Involvement in Children with IgA Vasculitis: Results from a Single-Center Cohort Observational Study.

Background and objective: IgA vasculitis (IgAV), a predominantly pediatric leukocytoclastic disease, has an unpredictable, though largely benign, evolution. The aim of this study was to retrospectively investigate any potential clinical or laboratory predictors of gastrointestinal involvement in a single-center cohort of children with IgAV. Patients and methods: A total of 195 children with a history of IgAV, regularly followed-up for an average period of 1 ± 2.6 years via outpatients clinics of the pediatric rheumatology unit in our University, were assessed, analyzing their clinical and laboratory variables in relationship with their disease evolution and outcome. Results: Univariate analysis showed that a higher neutrophil granulocyte count and lower lymphocyte count (expressed as a percentage of the total white blood cells) were significantly associated with the presence of gastrointestinal involvement at the first examination (65.2 ± 13% versus 58.8 ± 12%, p = 0.02, and 26.4 ± 11% versus 32.1 ± 11%, p = 0.02, respectively). A positive pharyngeal swab for Streptococcus pyogenes, a deficiency of 25-hydroxyvitamin D, a persistence of purpuric rash for more than 1 month, and purpuric lesions in the genital area were also associated with gastrointestinal involvement (p = 0.0001, p = 0.0001, p = 0.007 and p = 0.001, respectively). However, multiple logistic regressions with correction for the patients' sex and age showed that lower 25-hydroxyvitamin D levels, persistent rash, and genital lesions were independently and significantly associated with signs of gastrointestinal involvement. We then performed a secondary analysis (both univariate and multivariate) to investigate whether vitamin D deficiency was associated with other IgAV manifestations: we found that only 25-hydroxyvitamin D deficiency remained significantly associated with gastrointestinal involvement in IgAV. Conclusions: Patients with IgAV and vitamin D deficiency might be more prone to developing gastrointestinal manifestations of variable severity.

Anorectal Malformations: The Pivotal Role of the Good Clinical Practice.

Anorectal malformations (ARM) without a fistula are a rare congenital condition. Although may seem more simple to repair compared with ARM with fistulas, surgery has proved to be challenging. We report the case of a newborn who presented a well-formed anus and normal genitalia; a blind-ending anal canal was detected after the insertion of a rectal probe, thus allowing the diagnosis of ARM. Anal probing straight after birth avoids the possible complications related to intestinal obstruction due to a missed diagnosis of ARM. Examination of the perineal region is an important step in the evaluation of the newborn and represents the tool for a prompt identification of ARM. Adding anal probing to accurate inspection perineum is a good clinical practice and should always be performed even in presence of a normal-looking perineum.

An Interesting Case of Neonatal AKI: What Is the Time to Consider Anuria Irreversible?

Acute kidney injury is a frequent complication for critical newborns. Its management is a significant challenge, especially in extremely low-birth-weight (ELBW) infants. Currently, peritoneal dialysis (PD) is the most manageable treatment. However, data are lacking regarding when diuresis can be declared irreversible relative to the start of PD. A female infant born at 28 + 0 weeks with a birth weight of 800 g by monochorionic diamniotic pregnancy, complicated by twin-to-twin transfusion syndrome, developed acute renal failure on the second day of life because of long-term intrauterine hypoperfusion. PD was started on day 7. The patient remained anuric until the 52nd day of dialysis, when she presented adequate urine output of 2.5 mL/kg/h and PD was suspended for 11 days. After an episode of sepsis, PD was re-started, and after 50 days of treatment, given a urine output of 1.5 mL/kg/h, it was discontinued. The patient died on day 132 after a disseminate infection, which led to multiorgan failure. In ELBW infants, PD is a valid therapeutic instrument to treat patients with renal failure. Despite the evidence of low renal functional reserve in these patients, the duration of recovery from diuresis after a period of anuria can be very long.

Persistent Diarrhoea after Percutaneous Endoscopic Gastrostomy (PEG) in Paediatric Patient: Lessons from a Complication.

Percutaneous endoscopic gastrostomy (PEG) is increasingly used in paediatric population. We report a case of a 4-year-old boy who, two weeks after PEG placement, presented persistent diarrhoea interpreted as intolerance to enteral feeding. His CT scan confirmed the correct placement of gastrostomy, but during gastroscopy, gastrostomy could not be found in the stomach, and the following colonoscopy revealed migration of gastrostomy to the transverse colon. The patient required removal of the misplaced PEG and conservative management of the fistula with surgical replacement of gastrostomy. We faced an unusual presentation of PEG placement complication due to colon interposition during blind gastric puncture. In children with anatomical deformities, previous surgery, or low weight or malnutrition (<10 kg), we suggest laparoscopic-assisted gastrostomy to avoid the risk of a major complication.

Prevalence of bladder cancer in Costello syndrome: New insights to drive clinical decision-making.

Costello syndrome (CS) is a rare disorder affecting development and growth characterized by cancer predisposition and caused by mutations in HRAS proto-oncogene. Somatic HRAS mutations drive bladder carcinogenesis. The aim of this study was to analyze prevalence and histological characterization of bladder cancer (BC) in a cohort of patients with CS to help clinicians plan effective management strategies. This study included 13 patients above 10 years of age with molecular diagnosis of CS. Screening cystoscopies (31 total procedures) were performed to exclude BC. Any lesion was analyzed through cold-cup biopsy or trans-urethral resection of the bladder. According to histology, patients were followed-up with urinalysis and abdominal ultrasound yearly, and cystoscopies every 12-24 months. During study enrollment, bladder lesions (often multifocal) were detected in 11/13 patients. Histological analysis documented premalignant lesions in 90% of cystoscopies performed, epithelial dysplasia in 71%, and papillary urothelial neoplasm of low-malignant potential in 19%. BC G1/low grade (Ta) were removed in 10%. Overall, 76% of patients showed a bladder lesion at first cystoscopy. The present findings document that individuals with CS aged 10 years and older have high prevalence of bladder lesions (premalignant/malignant), highlighting the importance of personalized screening protocols.

The Fakir Child: Clinical Observation or Invasive Treatment?

Accidental swallowing of foreign bodies is a common problem among the pediatric population (6 months to 3 years), especially if the foreign body (FB) presents a sharp end that could potentially lead to perforation of the gastrointestinal (GI) tract, resulting in infection and complications. We report the case of a 2-year-old, admitted to the Emergency Department of our hospital after ingesting two FBs classifiable as sharp objects, specifically two metal nails, both approximately 4-cm long, which had been swallowed in one go, as reported by the parents. The patient had been previously admitted to another hospital in the same region, where the Emergency Department (ED) doctors took an X-ray to confirm the ingestion. The foreign bodies ingestion was thus confirmed, and they were, according to their report, located in the GI tract over the stomach. The patient has been monitored through all of his stay in the hospital and the progression of the foreign bodies has been documented with serial X-rays. Since neither clinical nor radiological signs of perforation were present, putting the FBs in the small bowel, a non-operative expectant management was followed. After 4 days of admission, the patient had passed one of the two FBS and later on the second one, without any complication. Thereafter the patient was discharged. The management of sharp gastrointestinal foreign objects ingestion is still debated, and the data of the current literature are poor. A number of case reports and small case series describe successful conservative management for the majority of ingested sharp objects. According to the literature data, our report confirms that the ingestion of sharp objects and relatively big objects in a baby can be successfully non-operatively managed, even despite the age of the patient and though the FBs are multiple.

Applicability and Effectiveness of Laparoscopic Procedures in Pediatrics.

Background: Three-dimensional (3D) laparoscopic surgery in pediatrics is still uncommon and few studies assessed in clinical practice advantages and disadvantages. Applicability and effectiveness of 3D versus two-dimensional (2D) laparoscopic procedures in congenital and acquired conditions in children are still unknown. We assessed applicability and effectiveness of 3D compared with 2D laparoscopic procedures in a pediatric setting. Methods: Two groups of patients who underwent 3D or 2D laparoscopic surgical procedures between May 2016 and April 2018 were compared. Each 3D/2D laparoscopic procedure was assessed with a surgeon/assistant questionnaire. Results: The 3D group included 30 patients and the 2D group 32 patients. The analysis of the 3D/2D questionnaire showed statistically significant superiority of 3D technical aspects (P = .0000), allowing a better spatial orientation and depth perception, reducing manipulation and trauma to tissues. Moreover, no difference was reported in physical complaints (P = .7084), but decreased visual fatigue was highlighted by surgeon (P = .000). Conclusions: In pediatric patients, 3D laparoscopic procedures prove to be more effective facilitating the surgeon's performance, while maintaining the benefits of minimally invasive surgery.

Intraluminal Duplication of the Terminal Ileum with Ectopic Gastric Mucosa in an Infant: A Rare Cause of Intussusception.

Enteric duplication cysts are rare malformations mostly diagnosed before the age of two, with varied clinical presentations. Ectopic gastrointestinal epithelium can be present, and management involves surgical resection. A three-month-old girl presented with rectal bleeding due to an ileocolic intussusception. Abdominal ultrasound revealed a target sign in the right upper part of the abdomen. At hydrostatic contrast enema, an incomplete reduction of the intussusception was obtained: only a trickle of contrast material entered the terminal ileum. An exploratory laparotomy ensued with manual reduction of the intussusception. At the end of the maneuver, a soft intraluminal mass was palpated within the ileocecal valve. Thus, an ileocecal resection was performed. At histology, an intraluminal enteric duplication cyst was documented, containing ectopic gastric mucosa. Secondary intussusception should be suspected even in infants in case of abnormal findings at hydrostatic contrast enema. Intraluminal enteric duplication cysts may be a rare cause of intussusception.

Isolated Fetal Ascites, Neonatal Outcome in 51 Cases Observed in a Tertiary Referral Center.

Introduction Prenatal detection of isolated ascites is a rare finding on ultrasound, usually suggestive of an underlying pathology that may negatively impact on the pregnancy and neonatal outcome. The purpose of the present study was to evaluate the outcome of primary isolated ascites in relation to gestational age (GA) at diagnosis. Materials and Methods Data were prospectively collected for fetuses with ascites that have been followed in our center of prenatal diagnosis and therapy from 2004 to 2014. Patients have been divided in group I when ascites was detected before the 24th week of GA and group II if it was noticed later. Prenatal workup included detailed ultrasound, maternal blood group and presence of antibodies, maternal infection screening, fetal karyotyping, and if needed fetal paracentesis. Postnatal data included GA at birth, mode of delivery, weight at birth, neonatal and surgical outcome. Results During the study period, 51 fetuses were included. Among them, 28 in group I and 23 in group II. An associated anomaly was prenatally identified in 84% of the fetuses. Prenatal demise occurred only in patients belonging to group I for an overall incidence of 10%. An associated disease was confirmed after birth in 61% of cases in group I and in 74% in group II (p = ns). There was a higher incidence of gastrointestinal pathology in group II than in group I (47 vs. 10%, p = 0.004); with a significant prevalence of meconium peritonitis (32 vs. 4%, p = 0.016). Nine patients (17.6%) died after birth, all in group I, because of major systemic malformations. Overall, the postnatal outcome was good in 63% (n = 32) of the cases, and more than half of them belonged to group II (p = 0.003). Conclusion A wide range of etiologies have been found to be associated with isolated fetal ascites. A systematic diagnostic workup and multidisciplinary prenatal counseling can improve the accuracy of prenatal identification of associated pathologies. The early detection of fetal ascites, before the 24th week of GA, is associated with a significant risk of perinatal death; otherwise, a late diagnosis of fetal ascites is associated with an increased risk of gastrointestinal diseases, especially with meconium peritonitis.

Antegrade Sclerotherapy to Treat All Types of Varicoceles in the Pediatric Population: Experience of a Single Center.

OBJECTIVE: To analyze our experience with antegrade sclerotherapy for the treatment of Coolsaet types I, II, and III varicoceles in a pediatric and adolescent population. MATERIALS AND METHODS: Between 2005 and 2015, 73 patients who underwent antegrade sclerotherapy were retrospectively evaluated. Patient age, side, clinical and Doppler ultrasound grade, and anatomical variations were collected. Varicoceles were grouped following Coolsaet's classification: all types were sclerosed. Follow-up consisted in clinical examination 3 and 6 months following surgery and testicular Doppler ultrasound 6 and 12 months following surgery. Patients were then telephonically interviewed. Success was defined as varicocele resolution or reduction to a grade not requiring surgery. RESULTS: Mean patient age was 14.7 years and mean operating time was 50.8 minutes. Based on phlebographic imaging and following Coolsaet's classification, we identified 57 (78.1%) type I, 3 (4.1%) type II, and 13 (17.8%) type III varicoceles. No intraoperative complications were observed. Three patients (4.1%) presented a short-term complication in the form of pampiniform plexus thrombosis; 1 patient also developed wound dehiscence: all complications occurred in Coolsaet type I varicoceles and during surgeon learning curve. No hydrocele occurred. Out of 59 patients with a satisfactory follow-up (range: 14 months-10 years), 2 recurrences occurred, the success rate thus being 96.6%. CONCLUSION: Tauber's antegrade sclerotherapy is a simple and feasible technique, effective in treating all kinds of varicocele with low complication, recurrence, and persistence rates. Phlebography reveals frequent venous anatomical variations, allows grouping of varicoceles into Coolsaet types, and enables performing of sclerosis safely in all 3 kinds.

Incidental Diagnosis of Giant Cell Tumor After Urachal Remnant Removal in a Thalassemic Child.

Urachal anomalies are relatively uncommon; they usually present in childhood as urachal cysts, urachal sinuses, diverticula, and patent urachus. The malignant involvement of the urachus, although rare in children, is commonly found in adulthood when adenocarcinoma accounts for the majority of the cases, representing up to 20-30% of all adenocarcinomas of the bladder. Urachal tumors of variable histology have been reported in children and adolescents as single case report. We present the first case of a 4-year-old thalassemic boy, with an incidental finding of giant cell tumor arising in an urachal remnant.

Vacuum assisted closure for the treatment of complex wounds and enterocutaneous fistulas in full term and premature neonates: a case report.

BACKGROUND: The Vacuum Assisted Closure (VAC) system has become an effective treatment for acute and chronic wound defects. Although its use has been reported in wound care of children and premature infants, the management of the device in this population has not been well established. CASE PRESENTATION: We report the satisfactory results in two neonates (one full-term and one preterm) with complex wounds secondary to major abdominal surgery. In the premature baby an enterocutaneous fistula was also present. Complete epithelialization of the wounds was achieved in both patients within a few weeks thus avoiding any further surgical procedure. CASE PRESENTATION: The use of VAC system in neonates is safe and effective in the management of complex wounds and should be considered as a first line treatment in the event of a major dehiscence.

Antenatal ultrasonographic features associated with segmental small bowel dilatation: an unusual neonatal condition mimicking congenital small bowel obstruction.

Segmental dilatation of the intestine (SDI) is an unusual condition presenting during the neonatal period, with symptoms of obstruction. Late diagnosed cases are also reported. The clinical polymorphism, and the lack of specificity of radiological investigations, make diagnosis difficult. Prenatal detection of abdominal cystic lesions or bowel dilatation has occasionally been reported to be associated with SDI. We herein report two cases of SDI, with a prenatal ultrasonographic suspicion of intestinal abnormality. In both infants, a dilatation of the ileum was found at surgery, without any evident site of obstruction or abnormal histology. SDI must be taken into consideration when a prenatal alert of possible bowel obstruction is not followed by postnatal clinical signs.