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Twin obstructive megaureters associated with duplex renal system is a rare malformation, particularly when a giant ureter dilation is present. We report an original case, initially misinterpreted, of a right incomplete duplex renal system with huge disproportion between the upper pole giant megaureter and the lower pole moderate megaureter, with severe obstruction at the uretero-ureteral junction and minor obstruction at the uretero-vesical junction. Uromagnetic resonance imaging allowed conclusive diagnosis and the laparoscopic approach was effective for definitive, successful treatment.

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Altered state of consciousness in infants can be the sole sign of a compromised intestinal blood flow. The article reports the case of an infant with inconsolable crying and lethargy with an omphalomesenteric duct remnant causing small bowel volvulus.

Children with intellectual disability/neurodevelopmental delay (ID-ND) commonly ingest foreign bodies (FB) and often present complications due to peculiar aspects of their condition. The aim of this paper is to report the experience of two centers in the management of ID-ND patients after FB ingestion and to discuss a possible algorithm for clinical practice. We retrospectively evaluated data of patients managed for FB ingestion (period: 2017–2021), focusing on those with ID-ND, specifically demographics and baseline diagnosis, elements related to the event, symptoms, time to endoscopy, FB location, endoscopic details, and follow-up. A total of 457 patients were managed in the study period and 19 had ID-ND (mean age 9.8 ± 3.5 years, 15 males). A total of 16/19 (84.2%) were symptomatic and required an operative approach. Recurrent ingestions and multiple FB were found in 2 and 11 patients, respectively. Endoscopy (mean time 65.6 ± 41 min) was effective in 14 cases (73.6%) and 6 patients (31.6%) developed a complication. FB ingestion in ID-ND patients represents a challenging condition for the clinician and a potentially dangerous situation. It should be addressed specifically by a multidisciplinary team considering a tailored diagnostic and management protocol.

Background Ogilvie’s syndrome is described in the adult population, but rarely seen in children. Case presentation We present a case of a girl who suffered acute colonic pseudo-obstruction after laparoscopic appendectomy. Conclusions Ogilvie’s syndrome, although rare in the pediatric population, should be considered as possible diagnosis after a surgical procedure in presence of persisting subocclusive symptoms and radiological signs of massive colonic dilatation without mechanical obstruction.

Whilst most surgeons agree that conservative treatment of appendiceal abscess in children is an adequate treatment, the need for subsequent interval appendectomy is still controversial. We analyzed the histopathology in interval appendectomy in search of signs of inflammation. All patients admitted between 2010 and 2017 with appendiceal abscess and scheduled for interval appendectomy were reviewed. The specimens were evaluated for grade of inflammation, type and distribution of cellular infiltrate, presence of necrosis or hemorrhage and infiltrate in the serosa. Forty-two patients had appendiceal abscess and were treated conservatively. Seven underwent emergent appendectomy. Thirty-three out of 35 patients underwent elective interval appendectomy. Thirty-two specimens were revised. Carcinoid tumor or other malignant lesions were not found. All of them presented some amount of inflammation, grade 1 to 2 in 53%, grade 3 to 4 in 47%. Twenty-five percent of the specimens had signs of necrosis accompanied by hemorrhage and in more than the half (53%) the infiltrate extended to the serosa. Conclusions: Although the appendix was mostly found not macroscopically inflamed intraoperatively, histology confirmed a certain grade of inflammation even months after the conservative treatment. No correlation was found between histopathologic findings and lapse of time between abscess treatment and interval appendectomy.

Introduction A fast surgical treatment is the gold standard when a testicular torsion is diagnosed. However, an early diagnosis of torsion may not be feasible in case of torsion associated with undescended testis in the patients affected by cerebropalsy. Case presentation A Bolivian 16 year old male with acquired cerebropalsy and spastic neuromuscular disease was admitted to our Institute for a right inguinal swelling observed by the father in the morning. Indeed, the father had reported that the swelling had may be started two days before without pain or any other symptoms apparently. Two episodes of vomiting were only reported. At the general examination the patient, apparently, seemed to laugh repeatedly and a spastic movements increase were observed. The child had an infrainguinal bilateral cryptorchidism. An urgent left infrainguinal orchyectomy had been performed in the past and controlateral cryptorchidism was not corrected. At the right inguinal exploration, a complete twist of the spermatic chord was observed and a right orchyectomy was then performed. Discussion Testicular torsion in the inguinal canal is a rare reported condition that usually can involve patients with spastic neuromuscular disease. Processing, communication and verbalization of a chronic or acute pain seems to be different in a child with or without intellectual disability. It could be a lot more difficult to correct pain interpretation, with an important repercussion on pain accurate assessment and management. Conclusion In the patients with intellectual disability, a control of the testicles, it should always be done, mostly in case of atypical behaviour., Highlights • An early diagnosis of a testicular torsion may not be feasible in case of torsion associated with undescended testis in the patients affected by intellectual disability. • Processing, communication and verbalization of a chronic or acute pain seems to be different in a child with or without intellectual disability. • Atypical behaviour could be present in the children without capacity to verbalize the pain. • In the patients with intellectual disability, a control of the testicles should always be carried out, mostly in case of atypical behaviour.

Developmental dysplasia of the hip (DDH) is one of the most common congenital defects in the newborn. When its severe form is not corrected, it is associated with long-term morbidity. Closed reduction with casting is the standard primary treatment and reduction is confirmed by magnetic resonance imaging (MRI). We reported our experience on the reliability of MRI in postreduction assessment of DDH.All children who underwent closed reduction for Graf type IV DDH at our institution between September 2010 and June 2016 were retrospectively reviewed. Since 2010 we assessed postreduction position of the femoral head by performing a MRI.Twenty-five (5 male, 20 female) patients presented with 29 (15 left sided, 6 right sided, 4 bilateral) Graf type IV DDH and underwent closed reduction at a mean age of 3.4 months. In all patients MRI studies performed within 24 hours were diagnostic, showing a concentric reduction of the femoral head within the acetabulum in 24/25 patients. In the patient with persistent hip instability, a subsequent open reduction was performed. In all the cases, there was no need of any contention or sedation during MRI.On the basis of our experience, MRI is an excellent, safe and, reliable modality to confirm maintenance of adequate femoral head position and to evaluate soft tissue interposition. We agree that MRI is the gold standard to early depict dislocation after closed reduction of DDH.

Reason to report: Hydronephrosis caused by congenital ureteropelvic obstruction is quite common affecting up to 0.2% of infants and a pyeloplasty is the way to correct. Ultrasound is the technique of choice to follow up this kind of patient, but no other recommendations are present in literature. What was unique: So far no one has studied how the changing in the urinary way reflect the ultrasound image. We applied Laplace law to the geometric modelling of the urinary way of ten patients that underwent pyeloplasty before the age of three. Ramification of this report: After pyeloplasty, due to its reduced thickness, the pelvis presents a tendency to dilate, even by a low increase of urinary pressure. Ultrasound evaluation with an empty bladder can distinguish between an obstructive pattern and a reoccurrence of junctional occlusion.

Wandering spleen is a rare condition, typically not only due to embryological defects of the splenic ligaments, but also secondary to trauma and splenomegaly. The most common presentation is acute abdomen with a mobile abdominal mass or recurrent abdominal pain. However, the spleen may be temporary in its normal position, and patients could be asymptomatic. A familiarity, if present, strengthens the diagnostic suspect.Abdominal ultrasonography and computed tomography are the examination of choice, and the management is surgical.

A 10-year-old boy was seen in the outpatient clinic for a phimosis. When the prepuce was retracted, a wide fish-mouth meatus with a cleft deep glandular groove was observed (figure 1). Figure 1 Fish-mouth meatus visible after prepuce’s retraction. A megameatus concealed by a normally developed …

Background Short bowel syndrome (SBS) has an impact on children and their families not only physically, but also emotionally, mentally, and socially. This study aimed to evaluate quality of life and family impact in patients with SBS, using the Pediatric Quality of Life Inventory (PedsQL) measurement model. Materials and Methods PedsQL questionnaires were administered to patients with SBS followed at the Pediatric Surgery of Royal Manchester Children's Hospital. The scores were compared between two groups differing in age (children 5 y) and with known-groups from literature published by Varni et al. Results Forty-three patients were sent the questionnaires, and 30 (70%, 17 5) responded. Family Impact Module failed to distinguish between families of children younger and older than 5 years of age. In contrast, it distinguished between families with SBS children and the ones with children in a long-term care facility. Healthcare satisfaction was worse in families of children over 5 years regarding information received, inclusion of the family, and communication. When compared with normal population, SBS children scored worse on Generic Core Scales for all domains except for emotional functioning. Conclusion Although advances have been made on the treatment of children with SBS, and improvements have been reached on home PN, this condition is still significantly affecting the quality of life of children and their families. The future quality control of medical care must have greater focus on psychosocial and emotional functioning, aiming for the best possible quality of life.

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No agreed recommendations exist for timing of urethral stent removal, after distal hypospadias surgery. We compared our preliminary case series with outcomes from literature: 18/44 patients were treated with catheter and 26/44 without it. The surgical outcome was comparable in the two groups. After hypospadias surgery, the main advantage of the immediate postoperative catheter removal was the shorter hospital stay without negatively affecting the care and home management.

Background Short bowel syndrome (SBS) is the most common cause of intestinal failure in children. Many factors have been investigated in an attempt to define which parameters influence most survival and ability to wean off parenteral nutrition (PN). The aim of this study was to investigate if aetiology of SBS affects the outcomes in paediatric patients treated with autologous gastrointestinal reconstructive surgery. Methods All children with SBS who underwent autologous gastrointestinal reconstructive surgery between 2002 and 2012 were retrospectively reviewed and outcome measures were recorded. Results Forty-three patients were divided into 4 groups according to aetiology (gastroschisis, volvulus, necrotizing enterocolitis (NEC), intestinal atresia). No significant differences were found among groups regarding survival and median age at surgery. The volvulus group had a lower pre-operative bowel length in comparison with gastroschisis and intestinal atresia and the lowest percentage of patients off PN (30%). Gastroschisis had the lowest rate of preserved ileocaecal valve (10%), while intestinal atresia had the highest (66%). For children who weaned off PN, intestinal atresia had also the longest time to achieve enteral autonomy (14.5months), while NEC had the shortest (3.5months), followed by gastroschisis (8.5months). None of the patients needed transplant. Conclusions In our experience it does not appear that diagnosis is significantly related to outcome and this is consistent with the conclusions of other reviews. However, it should be noted that in our series patients with volvulus had the worse outcome in terms of weaning off PN when compared with intestinal atresia. Type of Study Retrospective Study. Level of Evidence II

Introduction The appropriate management of asymptomatic congenital pulmonary malformations (CPMs) remains controversial. Prophylactic surgery is recommended to avoid the risk for development of pulmonary infections and to prevent the highly debated development of malignancy. However, the true risk for development of malignancy remains unknown. A systematic review analyzed all cases in which lung tumors associated with CPMs in both the pediatric and adult populations were described. Methods A comprehensive literature search was carried out; it included all the cases in which an association between CPMs and malignant pulmonary lesions was reported. Results In all, 134 publications were eligible for inclusion. In 168 patients CPM was found associated with lung tumor. The diagnosis was made in 76 children at a mean age of 3.68 ± 3.4, whereas in the adult population (n = 92) it was made at a mean age of 44.62 ± 16.09. Cough was the most frequent presenting symptom both in children and in adults. Most of the patients underwent lobectomy. The tumor most often associated with CPM was pleuropulmonary bastoma in children (n = 31) and adenocarcinoma (n = 20) or bronchioloalveolar carcinoma (n = 20) in adults. The CPM most frequenty associated with tumors in children was congenital cystic adenomatoid malformation (n = 37), especially type 1 (n = 21), whereas in adults it was bronchogenic cyst (n = 25), followed by congenital cystic adenomatoid malformation (n = 21). Conclusions CPMs should be followed up and never underestimated because they may conceal a tumor. Apparently, there is no age limit for malignant progression of CPMs and no limit of the interval between first detection of the CPM and appearance of the associated tumor.

Lipoblastoma is a benign tumor that often has rearrangements of the 8q11-13 region targeting the PLAG1 gene, which is involved in the process of tumourigenesis. We described a signature of chromotripsis in a thigh lipoblastoma, as first report of chromotripsis in a benign tumor.

Subcutaneous granuloma annulare is an inflammatory lesion occurring in otherwise healthy children. We present 3 pediatric patients with different diagnostic-therapeutic paths depending on the ward they were referred to. The lesions regress spontaneously, and medical or surgical treatments are generally not necessary.

Background: Bronchial peristalsis modulates lung growth and is deficient in hypoplastic nitrofen-exposed rat lung explants. Retinoic acid (RA) rescues lung hypoplasia. This study examines whether decreased bronchial innervation contributes to this developmental deficiency and if RA is able to recover bronchial innervation and motility. Material and Methods: After IRB approval, pregnant rats received either 100 mg nitrofen or vehicle on gestational day 9.5 (E9.5). Embryonic lung primordia harvested on E13 were cultured for 72 h and RA was added daily to the medium when appropriate. Lung growth was assessed by counting the number of terminal buds and measuring explant surface, total DNA and protein in control, control + RA, nitrofen and nitrofen + RA groups. Peristaltic contractions were recorded for 10 min under an inverted microscope. Lung explants stained for anti-protein gene product 9.5 (PGP 9.5) and smooth muscle α-actin were examined under a confocal microscope for depicting the specific relationship between neural and smooth muscle cells. PGP 9.5 and smooth muscle α-actin levels were quantified by Western blot analysis for assessing the neural and muscle cell expressions. Comparisons between groups were made with non-parametric tests. Results: The number of terminal buds, the explants’ surface and the DNA and protein contents were significantly decreased in nitrofen-exposed lungs in comparison with controls. In contrast, these measurements were normal in explants exposed to both nitrofen and RA. Bronchial peristalsis (contractions/min) was significantly decreased in nitrofen-exposed lungs in comparison with controls; in contrast, in nitrofen + RA lungs it was similar to controls. In all study groups, the airways were surrounded by smooth muscle and ensheathed in a plexus of nerve fibers containing ganglia. PGP 9.5 protein levels were decreased in nitrofen-exposed lungs, but they normalized when RA was added. No differences were found in α-actin protein levels. Explants exposed only to RA were similar to control. Conclusions: Lung growth, bronchial innervation and peristalsis are decreased in nitrofen-exposed lung explants and are rescued by RA. If deficient airway innervation contributing to dysmotility and pulmonary hypoplasia can be pharmacologically rescued, new relatively simple prenatal interventions could be envisioned.

To test the hypothesis that two modalities of IPC should decrease acute rejection and BT after SBTx in rats. Orthotopic allogenic SBTx was performed from Wistar to BN. IPC was performed by 2 ' and 5 ' superior mesenteric artery clamping, following 2-min and 5-min reperfusion before graft cooling and retrieving. Donor-recipient sets were randomly allocated to five groups: IPC2m4d, IPC2m7d, IPC5min7d, and the control groups for the two end points; ctrl4d and ctrl7d. IRI, rejection, and BT were assessed after four or seven days depending on the groups. Measured variables included: histology, leukocyte activation by tissue MPO determination, and proinflammatory cytokines (IL-b and TNF-α) to assess inflammatory response. Leukocyte activation was significantly reduced in IPC2m7d in comparison with Ctrl and IPC5min7d. Rejection tended to be lower in IPC2min7d. Cytokine levels were contradictory and not consistent with histology. Finally, BT was less frequent in IPC2min4d group but this benefit was missed in animals with rejection (7d). Inflammatory response (MPO) was reduced and rejection tended to be lower after in IPC2m7d. Bacterial translocation was reduced in IPC2min4d but the benefit was missed at day 7.

The research on congenital diaphragmatic hernia (CDH) is often carried out on the nitrofen fetal rat model in which most investigations involve microdissections and fastidious assessment of serial sections of different anatomic areas. Current microscopic magnetic resonance (MMR) equipment allows detailed anatomic studies of alive, fresh or fixed fetuses. The purpose of the present study was to demonstrate that CDH itself and most of the associated malformations are adequately imaged and measured by MMR. Fetuses from pregnant rats treated with either i.g. vehicle (control, n = 10) or 100 mg nitrofen (only those with CDH, n = 18) on E9.5 were recovered on E21 (term = E22) and total body was scanned by MMR under sedation in a 7 T MRI system (Bruker Medical, Ettlingen, Germany). CDH was detected with a coronal multislice fast spin echo sequence with a long repetition time and short effective echo time. Oblique MPR and 3D reconstructions were used. All studies were processed with attention to the hernia and its contents and the structure of the tracheobronchial tree and the lung, the heart and great vessels, the thymus and cervico-thoracic vertebrae. The findings in both groups were compared. Congenital diaphragmatic hernia, lung hypoplasia and parenchymal features were clearly depicted. Tracheal ring anomalies were also demonstrated. The thymus was significantly smaller in CDH pups (2.9 × 1 × 2.4 mm) than in controls (4 × 1.3 × 2.8 mm) (p

We previously demonstrated that tracheobronchial innervation, originated from the vagus nerve and hence of neural crest origin, is deficient in rats with experimental congenital diaphragmatic hernia (CDH). The present study examines the development of this innervation during fetal life in an attempt to understand the nature of these deficiencies.Pregnant rats were given either 100 mg nitrofen or vehicle on E 9.5. Embryos were recovered on E15 and E18. Control and nitrofen/CDH pups (n = 10 each) were studied on each of these days and compared with our previous results on E21. Whole mount preparations of tracheas stained for anti-protein gene product 9.5 (PGP 9.5) and smooth muscle contractile alpha-actin were examined under confocal microscopy for the morphology of intrinsic neural network. Sections of tracheas were immunostained with anti-low-affinity neurotrophin receptor (p75(NTR)), neural cell marker PGP 9.5, and anti-glial cell marker S100 antibodies. The proportions of sectional areas occupied by neural and glial structures were measured in the proximal and distal trachea. PGP 9.5 protein, and mRNA expressions were determined. Mann-Whitney tests with a threshold of significance of P0.05 were used for comparison.Positive staining for p75(NTR) confirmed the neural crest origin of tracheal neural cells. The neural network appeared less organized on E15, and it was less dense on E18 in nitrofen-exposed embryos than in controls. The proportions of section surface occupied by neural elements were similar in both groups on E15, but that of glial tissue was significantly increased in nitrofen-exposed embryos. On E18, the relative neural surface was significantly reduced in CDH embryos in contrast with increased glial tissue surface. On E21 the proportion of neural tissue was reduced only in the distal trachea. The expression of PGP 9.5 protein was decreased in CDH fetuses on E18 and E21. In contrast, PGP 9.5 mRNA levels were increased in CDH fetuses on E18 and E21.The development of intrinsic innervation of the trachea in rats with CDH is abnormal with reduction of neural tissue accompanied by increase of glial tissue that could represent a response to neural damage. The significance of increased PGP 9.5 mRNA levels is unclear.

Background/Aims Better antacid medications and the introduction of laparoscopy destabilize the indications for fundoplication. This study aims at raising a discussion among pediatric surgeons on these indications, modalities, and the results of this operation. Materials and Methods A total of 252 refluxing children operated between 1992 and 2006 were divided into groups according to predominant symptoms (93 digestive, 47 respiratory, and 68 neurologic) or to comorbidities (27 esophageal atresia, 10 diaphragmatic hernia, 5 abdominal wall defects, and 2 caustic stricture), and the indications, complications, mortality, and long-term results were reviewed. Features of open (n = 135) and laparoscopic (n = 117) approaches were compared, and long-term integrity of the wrap was analyzed using the Kaplan-Meier method. Results Digestive, respiratory, and neurologic patients had more often laparoscopic plications, whereas all others rather had an open approach. The rate of complications was 22%, and they were more frequent in children operated by laparotomy ( P Conclusions Fundoplication is a powerful method of reflux control. It is indicated after failure of medical treatment in gastroesophageal reflux disease and in symptomatic refluxers with some particular comorbidities. Surgery should be offered only after diagnosis has been firmly established, and the indications must remain identical for open and laparoscopic procedures. High technical standards and rigorous report of the results are required for keeping a relevant place of pediatric surgery in the treatment of this disease.

Increased infertility and smaller volume accompany undescended testis. Timing of orchiopexy is still a matter of debate. We evaluated the growth of non-palpable testes after laparoscopic orchiopexy according to age at surgery, intraoperative findings and type of procedure.Forty-one boys undergoing laparoscopy for nonpalpable testes were retrospectively reviewed and divided into two groups, ≤18 months and18 months, according to their age at surgery.At follow-up, 14 testes in the younger group had normal size, while 3 atrophied either after single (2) or two stage procedure (1). Similarly, in older boys 11 testes grew normally, while 5 atrophied after both procedures.Most of the non-palpable testes grew normally after laparoscopic orchiopexy and the postoperative volume seemed independent from the surgical strategy. Both techniques led to a few cases of testicular hypotrophy. In our experience, the age at surgery did not affect the outcome in terms of testicular growth.

Respiratory failure secondary to pulmonary hypoplasia is the main cause of death in congenital diaphragmatic hernia (CDH). Lung growth is regulated by growth factors (GFs), whose imbalances are reported in pathological conditions. We measured amniotic fluid levels of GFs, regulating lung development, in pregnancies with CDH and compared them with normal gestations.Amniotic fluid was collected at amniocentesis and delivery from 4 women carrying fetuses with CDH and 12 with normal pregnancy. GFs were isolated and quantified. Same GFs were measured in lung biopsies collected during autopsy of three newborns dead of CDH.Impairment expression of lung GFs in the amniotic fluid of CDH pregnancies in comparison with normal was found. Fibroblast growth factor 10 (FGF10), fibroblast growth factor 7, vascular endothelial growth factor and transforming growth factor β (TGFβ) were decreased at amniocentesis, while platelet-derived growth factor (PDGF) increased. While FGF10 and PDGF tended to normalize at delivery, epidermal growth factor increased and TGFβ was still decreased. Same GFs were similarly expressed in both lungs of babies dead of CDH.Anomalies in lung GFs expression of embryos and fetuses with CDH can be detected by measuring their levels in the amniotic fluid during pregnancy. Further investigation would help to correlate prenatal expression of GFs and clinical outcome of babies with CDH after birth.

Complications such as mechanical accidents, infections, and thrombosis are commonly described in the presence of a central venous catheter. We present a case of a boy who had fainting episodes due to dislocation of a central venous catheter.

We present 3 children affected by B. quintana infection treated at the IRCCS Burlo Garofolo of Trieste between March and April 2013. B. quintana infection is rare but it should be suspected in patients with fever and lymphadenopathy who do not respond to conventional antibiotic treatment. All patients had a complete recovery without sequelae or relapses.

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Asymmetric vulva swelling during prepubertal period is related to a variety of inflammatory and neoplastic lesions. An 11-year-old girl with childhood asymmetric labium majus enlargement is presented.

The authors present a magnetic resonance image showing a soft tissue mass misdiagnosed as sarcoma.

Gastroesophageal reflux and intestinal distension have been described in survivors of congenital diaphragmatic hernia (CDH). Deficient enteric innervation demonstrated in experimental models is a likely explanation for these symptoms. This study aimed at further characterizing these anomalies and examining esophageal and intestinal motility in this condition. Pregnant rats received either nitrofen or vehicle on E9.5. Sections of E15, E18, and E21 esophagus and small bowel were stained for protein gene product 9.5, nicotinamide adenine dinucleotide phosphate (NADPH) diaphorase (NADPHd), and acetylcholinesterase (AChE). The proportion of neural tissue/muscle surface was measured and the NADPHd- and AChE-positive motor endplates (MEPs) were counted. E18 and E21 stomachs were stained for AChE, the ganglia were counted and measured. The peristalsis of the esophagus and small bowel was video recorded. The relative neural/muscle surface and the number of NADPHd- and AChE-positive MEPs were decreased on E15 and E18 in the esophagus and small bowel of embryos with CDH, but they tended to improve on E21. The number and the mean surface of stomach ganglia were smaller in E18 and E21 fetuses with CDH. Peristaltic movements were decreased in the esophagus and small bowel of animals with CDH. Deficient enteric innervation impaired gastrointestinal motility in experimental CDH. This could explain some long-term morbidity in the human condition.