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We present the case of a 28-year-old man with a history of unexplained syncope, frequent ventricular arrhythmias, familial LMNA-related dilated cardiomyopathy (DCM), and mitral annular disjunction (MAD). We provide the first association of a novel truncating LMNA variant serving as a potential vulnerable substrate for arrhythmogenic MAD syndrome. This could suggest a possible synergistic role between concealed genetic variants (resulting in fibrosis as a "substrate" for arrhythmogenesis) and the presence of mitral annular disjunction (the "trigger" with mechanical stretch initiating ventricular arrhythmias), which may provide a link between mitral valve prolapse and sudden cardiac death.

Patent foramen ovale (PFO) has a high prevalence in general population and can be implicated in cryptogenic stroke among young people. Recent trials have shown that transcatheter PFO closure is superior to medical treatment in the secondary prevention of ischaemic stroke. The benefit in the reduction of stroke recurrence is particularly evident in patients who have documentation of a PFO with high-risk characteristics. Therefore, after the assessment of a clear causal relationship with the event, a thoughtful documentation of anatomic (height, length, presence of an aneurysmatic or a floppy atrial septum, a prominent Eustachian valve or Chiari’s network, an acute angle with the inferior vena cava) and functional high-risk characteristics is mandatory.

Background Patients with low levels of antithrombin III (AT III) are at an increased risk of developing arteriovenous thromboembolic disease.Case summary We report a case of a 28-year-old woman who presented with a 1-week history of spontaneous right calf pain and swelling. A heterozygous AT III deficiency, phenotypically expressed as deep vein thrombosis, was reported in the patient’s mother and sister. Blood workup revealed residual AT III activity at 58% with normal protein C and protein S levels. Computed tomographic angiography (CTA) revealed subsegmental bilateral pulmonary embolism (PE) and deep vein thrombosis in the right leg extending into the inferior vena cava up to the confluence of the left renal vein. Placement of an inferior vena cava filter was not considered. Given the patient’s haemodynamic stability, anticoagulant therapy with 15 mg of rivaroxaban twice a day was initiated instead. Echocardiography after 10 days of treatment revealed complete resolution of the thrombus located in the inferior vena cava, while CTA revealed complete resolution of the PE.Discussion Patients with AT III deficiency are likely to be heparin-resistant and will require higher heparin doses or the administration of AT III replacement therapy for the treatment of thrombosis, both of which are associated with an increased risk for haemorrhagic complications. Direct factor Xa inhibition by rivaroxaban provided an alternative mechanism for anticoagulation, which was found to be particularly useful in this patient with familial AT III deficiency, deep vein thrombosis, and PE.

Secondary mitral regurgitation (MR) occurs despite structurally normal valve apparatus due to an underlying disease of the myocardium leading to disruption of the balance between tethering and closing forces with ensuing failure of leaflet coaptation. In patients with heart failure (HF) and left ventricular dysfunction, secondary MR is independently associated with poor outcome, yet prognostic benefits related to the correction of MR have remained elusive. Surgery is not recommended for the correction of secondary MR outside coronary artery bypass grafting. Percutaneous mitral valve repair (PMVR) with MitraClip implantation has recently evolved as a new transcatheter treatment option of inoperable or high-risk patients with severe MR, with promising results supporting the extension of guideline recommendations. MitraClip is highly effective in reducing secondary MR in HF patients. However, the derived clinical benefit is still controversial as two randomized trials directly comparing PMVR vs. optimal medical therapy in severe secondary MR yielded virtually opposite conclusions. We reviewed current evidence to identify predictors of PMVR-related outcomes in secondary MR useful to improve the timing and the selection of patients who would derive maximal benefit from MitraClip intervention. Beyond mitral valve anatomy, optimal candidate selection should rely on a comprehensive diagnostic workup and a fine-tuned risk stratification process aimed at (i) recognizing the substantial heterogeneity of secondary MR and its complex interaction with the myocardium, (ii) foreseeing hemodynamic consequences of PMVR, (iii) anticipating futility and (iv) improving symptoms, quality of life and overall survival.

Background: The combination of esophageal atresia, congenital duodenal obstruction, and anorectal malformation has seldom been reported. We describe the largest series of patients with such association, which we summed up with the mnemonic acronym DATE [D-duodenal obstruction, A-anorectal malformation (ARM), and TE-tracheoesophageal fistula with esophageal atresia].Methods: This was a multicenter retrospective review of 13 patients recruited from 8 institutions over a nearly 5-decade period (1968-2017). Information gathered included type of DATE malformations, other associated anomalies, type and timing of surgery, and clinical outcomes.Results: The DATE association consisted of type C esophageal atresia (13), complete (9) or incomplete (4) congenital duodenal obstruction (CDO), and high or intermediate (8) or low (5) ARM. Eight patients had at least one additional component feature of VACTERL association. A total of 6 patients died. Overall, 9 patients achieved complete restoration of gastrointestinal continuity, 7 of whom are alive at a median follow-up of 4 y (range, 1 to 9). Survivors received a median of 6 major operations (range, 4 to 14) to overcome their anomalies and surgical complications. Two incomplete duodenal obstructions were initially overlooked. All survivors with high or intermediate ARM defects required some form of bowel management to keep them clean.Conclusions: The DATE association is a low-frequency entity, often occurring among the wider spectrum of VACTERL association. Functional outcomes largely depend on the severity of ARM or other major associated malformations. Awareness of the DATE association may avoid untoward diagnostic delays of subtler component features of the spectrum, such as an incomplete CDO. (C) 2019 Elsevier Inc. All rights reserved.

Purpose To quantify our experience with and assess the literature on diagnosis and management of isolated fallopian tube torsion (IFTT) with hydrosalpinx (HSX) in children. Methods A PubMed search was performed on pediatric cases of IFTT with HSX to provide a comprehensive review analyzing details and management of this association, focusing on the problem of fertility preservation. Results In addition to our 3 cases, 17 patients of pediatric IFTT associated with HSX were identified, for a total of 21 cases (median age 12.2 years). Menarchal status was present in 10/13 (76.9%); blood tests were reported in 9/20 (42%) showing leucocytosis in 7/9 (75%). Ultrasonography was performed in all cases except one. Laparoscopy was the surgical approach in 84.6% of the reported cases. The torsion was to the right in 36.8%, and to the left in 63.2% of the cases. In one case the torsion was bilateral and asynchronous. Performed procedures were salpingectomy (52.4%) and partial salpingectomy (14.3%); conservative management was reported in 33.3% of the cases. Conclusions The literature describes different management techniques. Salpingectomy is the most frequently performed procedure but recently conservative management seems to be increasingly applied. A long-term study is necessary to define the most effective treatment for the preservation of future fertility in pediatric patients. Type of study Treatment study (Retrospective Study): LEVEL IV.

Objective: The aim of this study was to review the English literature about a rare condition such as symptomatic mesodiverticular bands (MDBs) in children. Background: The MDB is an embryologic remnant of the vitelline circulation, which carries the arterial supply to Meckel’s diverticulum. In the event of an error of involution, an arterial band persists and extends from the mesentery to the apex of the antimesenteric diverticulum. This can create a snare-like opening through which bowel loops may herniate and become obstructed. This type of internal hernia is a very rare and often overlooked cause of small bowel obstruction. Materials and methods: A computer-assisted (PubMed) search of the literature to identify all cases of symptomatic MDB reported in English with patients’ age ranging from 0 to 14 years was performed. Results: Eight cases of symptomatic MBD in pediatric age (0–14 years) were found in the literature in the last 50 years. Male : female ratio was 3 : 1. The age of onset ranged from 10 days to 12 years. All cases reported an intestinal occlusion as clinical picture. Internal hernia was the cause of the obstruction in six cases, whereas in two patients the occlusion was due to a direct compression. All patients were approached with emergent laparotomy except one case of laparoscopic approach. Conclusion: MDB causing internal hernia is a very rare event but it should be kept in mind concerning patients with the presentation of small bowel obstruction when early surgery is important to prevent strangulation, gangrene of the bowel, and to avoid dramatic events. Keywords: internal hernia, laparoscopy, Meckel’s diverticulum, mesodiverticular band, small bowel obstruction

Infantile hypertrophic pyloric stenosis (IHPS) is a common surgical cause of gastric outlet obstruction among infants, characterized by elongation and narrowing of pyloric canal due to hypertrophy of pyloric muscle. IHPS is characterized by a progressive gastric outlet obstruction causing non-bilious projectile vomiting. Although the etiology is unclear, the pyloric muscle thickens abnormally in time and causes the obstruction of gastric outlet. Depending on the duration of symptoms, dehydration and hypokalemic hypochloremic metabolic alkalosis may occur. Physical examination and whether necessary ultrasonography may be enough for diagnosis of IHPS, and its standardized operative intervention is pyloromyotomy. In this chapter, other pyloric affections were also described.

Background Recurrent ipsilateral ovarian torsion at pediatric age is a rare event. Different surgical techniques for its prevention are available. We present a case of recurrent ipsilateral ovarian torsion in a prepubertal girl and we reviewed the literature about the management of this condition. Case A 6-year-old girl presented with right ovarian torsion and underwent a laparoscopic untwisting. Nine months later an ipsilateral recurrence occurred. Laparoscopic untwisting and right-sided oophoropexy with plication to the round ligament was performed. Summary and Conclusion In addition to our presented case, four cases of recurrent ipsilateral ovarian torsion in pediatric patients were identified in the literature. The few available reports in the pediatric literature show different management techniques. A long-term study is necessary to define the most effective treatment.

Objective The aim of this paper is to present the results of a 6-year experience with a laparoscopic-assisted approach to infantile hypertrophic pyloric stenosis (IHPS): the singleport, laparoscopic-assisted pyloromyotomy (SPLAP). Summary background data Ramstedt pyloromyotomy is the procedure of choice for IHPS; however, the best way to approach the pylorus is still debated. The recent literature reports many comparisons between various open and laparoscopic approaches. Here, we report our long-term result with a laparoscopic-assisted technique for IHPS. Materials and methods Thirty-eight infants underwent SPLAP. The approach to the abdominal cavity is performed through a right circumbilical incision and then a 10mm trocar is inserted. After the pneumoperitoneum is established, an operative telescope is introduced. Once the telescope is inserted, the pylorus is easily located and then grasped and exteriorized by the umbilical incision. At this point, conventional Ramstedt pyloromyotomy is performed. Once the pylorus is reintroduced into the abdomen, a new pneumoperitoneum is created to control mucosal integrity and hemostasis. Results In all 38 cases, adequate pyloromyotomy was performed in a good operative time, without any major intraoperative or postoperative complications, achieving excellent cosmetic results. Conclusion The feasibility of SPLAP found over these 6 years suggests that this procedure is an excellent alternative to open or laparoscopic pyloromyotomy. Keywords : infantile hypertrophic pyloric stenosis, laparoscopic-assisted, pyloromyotomy

Study Objective To report results of a retrospective multicentric Italian survey concerning the management of pediatric ovarian torsion (OT) and its recurrence. Design Multicenter retrospective cohort study. Setting Italian Units of Pediatric Surgery. Participants Participants were female aged 1-14 years of age with surgically diagnosed OT between 2004 and 2014. Interventions Adnexal detorsion, adnexectomy, mass excision using laparoscopy or laparotomy. Different kinds of oophoropexy (OPY) for OT or recurrence, respectively. Main Outcome Measures A total of 124 questionnaires were returned and analyzed to understand the current management of pediatric OT and its recurrence. The questionnaires concerned patient age, presence of menarche, OT site, presence and type of mass, performed procedure, OPY technique adopted, intra- and postoperative complications, recurrence and site, procedure performed for recurrence, OPY technique for recurrence, and 1 year follow-up of detorsed ovaries. Results Mean age at surgery was 9.79 ± 3.54 years. Performed procedures were open adnexectomy (52 of 125; 41.6%), laparoscopic adnexectomy (25 of 125; 20%), open detorsion (10 of 125; 8%), and laparoscopic detorsion (38 of 125; 30.4%). Recurrence occurred in 15 of 125 cases (12%) and resulted as significant ( P = .012) if associated with a normal ovary at the first episode of torsion. Recurrence occurred only in 1 of 19 cases after OPY (5.2%). Ultrasonographic results of detorsed ovaries were not significant whether an OPY was performed or not ( P = 1.00). Conclusion Unfortunately, oophorectomy and open technique are still widely adopted even if not advised. Recurrence is not rare and the risk is greater in patients without ovarian masses. OPY does not adversely affect ultrasonographic results at 1 year. When possible OPY should be performed at the first episode of OT.

Background Necrotizing enterocolitis (NEC) is the most common surgical emergency in newborns and it is still a leading cause of death despite the improvements reached in the management of the critically ill neonate. The purpose of this study was to evaluate risk factors, surgical treatments and outcome of surgical NEC. Methods We retrospectively evaluated a multicentric group of 184 patients with surgical NEC over a period of 5 years (2008-2012). Indications to operation were modified NEC Bell stages IIIA or IIIB. The main outcome was measured in terms of survival and postsurgical complications. Results Data on 184 patients who had a surgical NEC were collected. The majority of patients (153) had a primary laparotomy (83%); 10 patients had peritoneal drainage insertion alone (5%) and 21 patients had peritoneal drainage followed by laparotomy (12%). Overall mortality was 28%. Patients with lower gestational age (P=0.001), lower birth weight (P=0.001), more extensive intestinal involvement (P=0.002) and cardiac diseases (P=0.012) had a significantly higher incidence of mortality. There was no statistically significant association between free abdominal air on the X-ray and mortality (P=0.407). Mortality in the drainage group was 60%, in the laparotomy group and drainage followed by laparotomy group was of 23-24% (P=0.043). There was a high incidence of stenosis (28%) in the drainage group (P=0.002). On multivariable regression, lower birth weight, feeding, bradycardia-desaturation and extent of bowel involvement were independent predictors of mortality. Conclusions Laparotomy was the most frequent method of treatment (83%). Primary laparotomy and drainage with laparotomy groups had similar mortalities (23-24%), while the drainage alone treatment cohort was associated with the highest mortality (60%) with statistical value (P=0.043). Consequently laparotomy is highly protective in terms of survival rate. Stenosis seemed to be statistically associated with drainage. These findings could discourage the use of peritoneal drainage versus a primary laparotomy whenever the clinical conditions of patients allow this procedure.

Rational: Patent urachus (PU) is due to an incomplete obliteration of the urachus, whereas patent omphalomesenteric duct (POMD) is due to an incomplete obliteration of the vitelline duct. These anomalies are very rarely associated with one another. We describe a case of a newborn with a PU associated with a POMD, who was diagnosed by an abdominal ultrasound (US) and laparoscopy, and managed with a minimally invasive excision. Patient concern: A 28-day-old male neonate was referred to our hospital to investigate a delay in umbilical healing, with blood-mucinous material spillage for 3 weeks prior to the referral. The baby had no symptoms and was in good general health. Diagnosis: After a thorough cleaning of the umbilical stump, a clear granuloma with a suspected fistula was evident under the seat of the ligature of the stump. An abdominal US examination revealed the formation of a full communication, starting below the umbilical stump and developing along the anterior abdominal wall that connected with the bladder dome. The US also revealed a tubular formation containing air, which was compatible with POMD, in the deepest portion of the same umbilical stump. Considering these findings, the rare diagnosis of a PU associated with a POMD duct was suspected. Interventions: The child was then hospitalized for an elective laparoscopy that confirmed the US picture, and a minimally invasive excision was performed. Outcome: The postoperative course was favorable and uneventful. Lessons: Our case underlines the importance of evaluating all persisting umbilical lesions without delay when conventional pharmacological therapies fail. Using a US as the first approach is valuable and should be supported by laparoscopy to confirm the diagnosis; a minimally invasive excision of the remnants appears to be an effective therapeutic approach.

Traumatic abdominal wall hernias (TAWHs) can be defined as a herniation through disrupted musculature and fascia associated with blunt trauma. They are seen in approximately 1% of patients with blunt abdominal trauma. Data on TAWH in the pediatric population are very limited and principally based on case reports and a few case series. Past reports have indicated that the presence of the "handlebar sign" confers an increased risk of internal injury. Concomitant internal injuries are reported with an incidence between 25% and 70%, and occult hernias may also occur and are usually detected only by abdominal computed tomography scan and ultrasonography. The treatment of TAWH consists in surgical exploration through closure of the defect. We describe 2 cases of TAWH due to blunt impact by bicycle handlebars that occurred in our department with a brief literature review. Our objectives are to describe the variable clinical presentations and management of these events. We hope to provide a useful tool for the clinician to increase early clinical suspicion and detection of this insidious injury.

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.

Objective The aim of this study was to analyze retrospectively the 9-year experience of a single institution in the use of the double cross flap technique to interpose a double dartos flap to protect the neourethra with the aim of preventing fistula formation. Patients and methods Between October 2005 and September 2014 a total of 127 children with distal and midshaft primary hypospadias underwent tubularized incised plate urethroplasty by means of a double dartos flap obtained with a double cross flap to protect the neourethra. A Foley catheter was left in situ for 7 days. Success was defined as no incidence of complications requiring reintervention, along with good cosmetic result. A questionnaire was administered to estimate parental satisfaction. Parents were asked to evaluate the cosmetic appearance of the penis as good, acceptable, bad, or indifferent. Results The patient age at the time of surgery ranged from 12 months to 10 years (median 39 months). Three patients exhibited fistula at follow-up (2.3%). In two patients a glandular dehiscence of the urethroplasty occurred (1.5%). Mild stenosis of the neomeatus occurred in three patients (2.3%). No penile iatrogenic rotation occurred. A ‘good’ cosmetic result was reported by 68.5% of parents, acceptable by 22.8%, bad by 3.9% and indifferent by 4.7% of parents as per the parental questionnaire. Conclusion The double cross flap technique is not the panacea to prevent fistula formation in hypospadias surgery. Nevertheless, it offers an unquestionable advantage in terms of avoidance of fistula formation. The experience and skills of the surgeon performing urethroplasty remain the mainstay for best results.

Background: Reoperation for failed hypospadias has been considered to be seriously problematic. The dense fibrotic tissue causes difficulties in wound healing and increases the rate of complications. The tubularised incised-plate urethroplasty (TIPU) method has become a preferred method for all varieties of hypospadias in the past decade. However, fistulas are still one of the most common complications of this technique. The aim of this paper was to present the preliminary results of TIPU procedure with double de-epithelised dartos flaps in failed hypospadias repair. Materials and Methods: All patients were treated between January 2009 and August 2010 by the same procedure, utilising TIPU with double de-epithelised dartos flaps. Vascularised ventral dartos flaps harvested from each side of the penis with their vascular supply were transposed to cover the suture line by wrapping them from either side of the penis. Results: There were 21 boys with failed hypospadias: 20 had previously undergone TIPU, and one Koyanagi repair. Patients presented with very large fi stulas in four and dehiscence in 17. Repair of the failed hypospadias using TIPU with double de-epithelised dartos flaps was quite successful, with no fistula recurrence or dehiscence observed. Conclusion: The preliminary results showed that TIPU with double de-epithelised dartos flaps is a useful method of successfully repairing failed hypospadias.

OBJECTIVES • To evaluate urethrocutaneous fistula repair after urethroplasty using n-butyl cyanoacrylate (NBCA) in an outpatient setting. • To compare results of application of NBCA in 'early' and long-standing fistulae. PATIENTS AND METHODS • From January 2003 to December 2006, 13 children (mean age 36 months) were treated in our Outpatient Department for urethrocutaneous fistula, which occurred after hypospadias repair. • We analysed the data dividing the patients in two groups: group A comprised six children (mean age 22.6 months) that developed a fistula within 2-3 days of catheter removal after urethroplasty ('early' fistula) and group B comprised seven children (mean age 42.6 months) with long-standing fistulae. • In all patients, a multilayer of NBCA was applied, attaching the edges of the fistula taking care to perform a scarification of the edges of the fistula in patients in group B before the application of the glue. RESULTS • Four patients in group A and three patients in group B had definitive fistula repair (seven of 13), and six of the 13 had fistula recurrence. • Fistulae of ≤ 2 mm were more easily repaired than fistulae of > 2 mm (five of seven vs two of six). • Fistulae that tended to recover were those that responded to the first applications. CONCLUSIONS • In this experience the use of NBCA as a minimally invasive treatment for fistula repair gave good results for repairing fistulae. • These preliminary results encourage the use of NBCA as a first non-surgical attempt to repair urethrocutaneous fistulae especially if the fistula is ≤2 mm. • The failure of this procedure does not compromise a possible subsequent surgical repair.

The authors report their experience in laparoscopic repair of inguinal hernias in children. From May 2010 to November 2013, 122 patients with inguinal hernia underwent laparoscopic herniorrhaphy (92 males and 30 females). Telescope used was 5 mm, while trocars for the operative instruments were 3 or 2 mm. After introducing the camera at the umbilical level and trocars in triangulation, a 4-0 nonabsorbable monofilament suture was inserted directly through the abdominal wall. The internal inguinal ring was then closed by N or double N suture. All operations were performed in one-day surgery setting. In the case of association of inguinal and umbilical hernia an original technique was performed for positioning and fixing the umbilical trocar and for the primary closure of the abdominal wall defect. The postoperative follow-up consisted of outpatient visits at 1 week and 1, 3, and 6 months. The mean age of patients was 38.5 months. Of all patients, 26 were also suffering from umbilical hernia (19 males and 7 females). A total of 160 herniorrhaphies were performed; 84 were unilateral (66 inguinal hernia, 18 inguinal hernia associated with umbilical hernia), 38 bilateral (30 inguinal hernia, 8 inguinal hernia associated with umbilical hernia). Nine of 122 patients (6 males and 3 females) were operated in emergency for incarcerated hernia. A pre-operative diagnosis of unilateral inguinal hernia was performed in 106 cases. Of these patients, laparoscopy revealed a controlateral open internal inguinal ring in 22 cases (20.7%). The mean operative time was 29.9±15.9 min for the monolateral herniorrhaphies, while in case of bilateral repair the mean operative time was 41.5±10.4 min. The mean operative time for the repair of unilateral inguinal hernia associated with umbilical hernia was 30.1±7.4 while for the correction of bilateral inguinal hernia associated with umbilical hernia 39.5±10.6 min. There were 3 recurrences (1.8%): 2 cases in unilateral repair and 1 case a unilateral recurrence in a bilateral repair. No other complications were seen. Laparoscopic repair of inguinal hernia in children performed in this experience resulted a safe and effective procedure.

We report a case of a 5-year-old boy with acute abdomen following an upper airways infection who developed Meckel’s diverticulum perforation secondary to group A β-hemolytic streptococcus and its successful treatment by a laparoscopic-assisted intervention. To the best of our knowledge, such an event has never been reported previously. Keywords: group A β-hemolytic streptococcus, intestinal perforation, Meckel’s diverticulum

Urachal remnants (URs) are manifestations of an incomplete regression of the urachus; therefore, there may be different types of remnants such as cyst, sinus tract, diverticulum or patent urachus. The clinical presentation of a urachal anomaly includes umbilical discharge, lower abdominal pain and urinary tract infection, although a UR may also be asymptomatic. We present the case of a 2.5-year-old girl who presented with abdominal pain, stranguria and massive pyuria in which a giant infected UR was found. The diagnosis was made using abdominal MRI. The child was subjected to laparoscopic-assisted drainage and had an uneventful postoperative course. Keywords : differential diagnosis, infected urachal remnant, laparoscopy, pyuria

The authors report their preliminary experience in laparoscopic repair of associated inguinal and umbilical hernias in children.Twenty-six patients affected by the association of inguinal and umbilical hernia with an umbilical defect larger than 5 mm underwent a laparoscopic procedure. A 5-mm trocar was placed through the umbilical defect for the optic. To fix the trocar to avoid loss of carboperitoneum, we fashioned and tightened a purse-string non-absorbable suture with a sliding knot around the defect. In this manner, we ensured the trocar, fixing it and avoiding any loss of CO2, proceeding safely to the laparoscopic IH repair, by means of two additional 3 mm operative trocars. At the end of the inguinal herniorrhaphy, the previously fashioned purse-string suture was tightened to repair the umbilical defect.The mean operative time for the repair of associated inguinal and umbilical hernias was 30.1 ± 7.4 min in cases of unilateral inguinal hernia and 39.5 ± 10.6 for bilateral inguinal hernia. Follow-up ranged from 8 to 32 months. Neither intra- nor post-operative complications nor recurrences were seen.This small sample suggests that this simple method is safe, effective and might be useful for pediatric surgeons performing laparoscopic repair for inguinal hernia in presence of an associated UH with a statistically significant decrease of operative time.

To evaluate laparoscopic treatment of symptomatic urachal remnants in children, the authors review their experience analyzing different approaches and results obtained in a 8-year period.From July 2005 to September 2013, 12 children underwent 13 interventions for treatment of symptomatic urachal remnants. In four patients, the technique was a laparoscopic-assisted removal of the remnant, in two patients, a laparoscopic-assisted drainage of an urachal abscess, and in seven patients, a laparoscopic excision of the remnant. One patient underwent a double intervention-laparoscopic drainage of an infected urachal remnant and its delayed laparoscopic excision.The laparoscopic-assisted removal of the urachal remnant was performed in two cases of infected urachal sinus, in one case of symptomatic sinus, and in one cases of infected urachal cyst. The laparoscopic-assisted drainage of urachal abscesses was performed in two patients: In one patient, the abscess was because of an infected sinus while in the other patient, the abscess was caused by an infected cyst. Of the seven patients treated with pure laparoscopic technique, one had a symptomatic sinus, another had an association between a symptomatic urachal sinus and an urachal cyst, and five patients had a symptomatic urachal cyst. In all cases, intraoperative or postoperative complications and recurrences did not occur, and the cosmetic results were good. Follow-up ranged from 6 months to 8 years and 8 months.Laparoscopic surgery for symptomatic urachal remnants is safe and reliable in cases of drainage of urachal abscess and in cases of excision of the remnant. Laparoscopy allows a radical excision of the remnants with all the advantages of this procedure. In case of conversion, laparoscopic-assisted technique with minimal incision could be a good alternative to open surgery.

Human infections caused by Dirofilaria repens have been reported in many areas of the world. We describe a case of a 3-year-old child with an intrascrotal mass caused by D repens mimicking an acute scrotum. This represents the first case of scrotal dirofilariasis described in pediatric age with such an unusual presentation.

Objectives This study aimed to review the literature about symptomatic Meckel's diverticulum (MD) in the neonatal period with 2 additional uncommon cases. Methods The authors describe 2 interesting neonatal cases of symptomatic MD and analyze the literature on this topic, with particular reference to the prevalence of sex, age at presentation, most common signs and symptoms, treatment, histology, associated anomalies, and outcome. Results The first patient was a term newborn with bowel obstruction by a pseudocystic MD. The second patient was a preterm infant with double perforation of the MD and ileum. Literature search for published case reports and case series on this topic reveals only 18 cases of neonatal symptomatic MD. Males are more frequently involved than females, and even preterm infants may be affected. Bowel obstruction (58.3%) and pneumoperitoneum (33.3%) are the most frequent clinical manifestation. Acute inflammation of the MD is the prominent histopathological finding (75%), although it does not seem to be related with the presence of heterotopic tissue within the MD. Surgical treatment is essential. The association of neonatal symptomatic MD with other anomalies is exceptional but is otherwise life threatening despite surgery. Conclusions Bowel obstruction and pneumoperitoneum are the most frequent clinical manifestations of symptomatic MD in the newborn. Surgery is required for a definitive diagnosis and successful outcome.

Dog bite injuries are common in children and represent an important health-care problem. Most dog bite injuries involve the face or an extremity. Victims tend to seek medical care quickly. Dog bites to the external genitalia are rarely reported, but they potentially result in morbidity if improperly managed. Morbidity is also directly related to the severity of initial wound. Guidelines for the management of dog bites include irrigation, débridment, antibiotic therapy, consideration of tetanus and rabies immunisation and suture of wounds or surgical reconstruction. Literature review was conducted and focused to analyze the management of dog bite lesions involving external genitalia.Key words: Children, dog bite, genitalia, management

The purpose of this study is to describe the neuroimaging (CT and MR imaging) findings in liver transplant patients who develop severe neurologic side effects during immunosuppressive therapy with tacrolimus and to correlate these findings with clinical signs and tacrolimus levels in blood.Brain CT and/or MR imaging was performed on six patients who developed neurologic symptoms while receiving tacrolimus in the post-transplant period. All patients were evaluated by the neurology staff, and imaging studies were independently interpreted by three neuroradiologists. Trough tacrolimus levels in blood were measured with the IMX immunoassay and were correlated with neurologic symptoms and imaging findings.Imaging abnormalities were observed in five of six patients during the course of their neurologic illnesses. For each patient, neurologic symptoms began when the tacrolimus level in blood was at a peak, exceeding the therapeutic limit in all but one case. In five patients, neurologic symptoms eventually resolved after the tacrolimus dose was reduced or after the drug was stopped. Multifocal low attenuation of white matter was the predominant finding seen on CT images, and matching hyperintense white matter foci were observed on long-TR MR images. In three patients, clinical recovery was accompanied by reversal of the white matter abnormalities seen on CT and MR images.Immunosuppressive therapy with tacrolimus may produce neurologic side effects that are associated with brain CT and MR imaging abnormalities. Resolution of symptoms and reversal of imaging findings occur when the tacrolimus dose is reduced.

In some patients who are hospitalized for acute illness, we have noted a reversible syndrome of headache, altered mental functioning, seizures, and loss of vision associated with findings indicating predominantly posterior leukoencephalopathy on imaging studies. To elucidate this syndrome, we searched the log books listing computed tomographic (CT) and magnetic resonance imaging (MRI) studies performed at the New England Medical Center in Boston and Hôpital Sainte Anne in Paris; we found 15 such patients who were evaluated from 1988 through 1994.Of the 15 patients, 7 were receiving immunosuppressive therapy after transplantation or as treatment for aplastic anemia, 1 was receiving interferon for melanoma, 3 had eclampsia, and 4 had acute hypertensive encephalopathy associated with renal disease (2 with lupus nephritis, 1 with acute glomerulonephritis, and 1 with acetaminophen-induced hepatorenal failure). Altogether, 12 patients had abrupt increases in blood pressure, and 8 had some impairment of renal function. The clinical findings included headaches, vomiting, confusion, seizures, cortical blindness and other visual abnormalities, and motor signs. CT and MRI studies showed extensive bilateral white-matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres, but the changes often involved other cerebral areas, the brain stem, or the cerebellum. The patients were treated with antihypertensive medications, and immunosuppressive therapy was withdrawn or the dose was reduced. In all 15 patients, the neurologic deficits resolved within two weeks.Reversible, predominantly posterior leukoencephalopathy may develop in patients who have renal insufficiency or hypertension or who are immunosuppressed. The findings on neuroimaging are characteristic of subcortical edema without infarction.

To determine the features of the cardiac cycle-related cerebrospinal fluid (CSF) flow pulsations in with Chiari I malformation before and after decompressive surgery.Fifteen patients and 23 healthy subjects underwent cine phase-contrast magnetic resonance imaging. Ten patients were also examined after decompressive surgery. Four regions of interest were selected in the upper cervical subarachnoid space and premedullary cistern. Velocity and fraction of the cardiac cycle waveforms were plotted, and amplitude, temporal, and CSF displacement parameters were assessed.In the Chiari I patients, impaired systolic and unaltered diastolic CSF flow pulsations immediately below the foramen magnum were identified. After surgery, the systolic flow pulsations immediately below the foramen magnum improved. Good correlation with post-surgical improvement was observed.CSF flow waveform analysis helps demonstrate abnormalities in CSF flow at the foramen magnum and the benefits of decompressive surgery in patients with the Chiari I malformation.

Ramstedt pyloromyotomy is still the procedure of choice for infantile hypertrophic pyloric stenosis; however, the best way to approach the pylorus is debated. Recent literature reports many comparisons between various open approaches and laparoscopic one. The purpose of this preliminary experience is to show a new approach to infantile hypertrophic pyloric stenosis: single-port, laparoscopic-assisted pyloromyotomy. Nineteen infants underwent single-port laparoscopic-assisted pyloromyotomy. The approach to the abdominal cavity is performed through a right circumbilical incision, and then a 12-mm trocar is inserted. After the pneumoperitoneum is established, an operative telescope is introduced. Once the telescope is inserted, the pylorus is easily located, and then grasped and exteriorized via the umbilical incision. At this point, conventional Ramstedt pyloromyotomy is performed. Once the pylorus is reintroduced in the abdomen, a new pneumoperitoneum is created to control mucosal integrity and hemostasis. A retrospective statistical analysis was performed to compare patients who underwent this technique to others approached by the same team with right upper quadrant incision or right semicircular umbilical skin-fold incision. In all 19 cases, adequate pyloromyotomy was performed in a good ranging time without any intra- or post-operative complications, achieving excellent early cosmetic results. The feasibility of single-port, laparoscopic-assisted pyloromyotomy obtained in this small sample suggests that this procedure could be an excellent alternative to open or laparoscopic pyloromyotomy as long as it acts as intermediary between the two techniques.

Many surgical approaches to posterior urethral diseases are reported in published data. The authors report a case of a patient with prostatic urethral post-traumatic fistulae, probably developed from an abscess that developed after a surgical intervention to correct a pubic symphysis fracture. The fistulae were repaired with an unusual mininvasive endourologic procedure, using the hyaluronic acid dextranomer, which is commonly used in vesicoureteral reflux treatment.

Animal bites to human external genitalia are rare. Only a few cases of scrotal dog bite in children have been reported. We present an additional specific case of a scrotal dog bite in a child because the lesion and its repair have not been previously reported in published pediatric studies. A traumatic resection of the right testicular vas deferens was repaired by microsurgical vasoepididymal anastomosis. A review of the published data was also performed to analyze the management of scrotal dog bite lesions.

We analyzed a new urethra preserving approach, double-cross flap protection, during tubularized incised plate urethroplasty. We compared the results of 57 patients treated with this new procedure and 80 patients treated with 2 different techniques.We studied 137 patients with hypospadias between October 2002 and March 2008. Patients were divided into 3 groups. Group 1 consisted of 40 patients (mean age 50 months) undergoing tubularized incised plate urethroplasty. Group 2 included 40 patients (mean age 48 months) undergoing tubularized incised plate urethroplasty with dorsal subcutaneous flap. Group 3 consisted of 57 patients (mean age 39 months) undergoing tubularized incised plate urethroplasty with 2 de-epithelialized preputial flaps twisted ventrally and sutured individually over the neourethra. All patients were operated on by the same surgeons. Statistical analysis of postoperative complications was performed by ANOVA and chi-square test.In Group 1 fistula developed in 6 patients (15%) and was associated with stenosis of the neourethra in 4 (10%). In Group 2 fistula developed in 4 patients (10%). In Group 3 no fistula was observed, but mild stenosis of the neomeatus developed in 3 patients (5.3%), which was treated with dilation. Thus, the incidence of fistula in group 3 was significantly less (p = 0.015).Double-cross flap protection is safe and significantly reduces the incidence of postoperative fistula.

Objective: To define the behavioral profile of periventricular nodular heterotopia (PNH), a malformation of cortical development that is associated with seizures but reportedly normal intelligence, and to correlate the results with anatomic and clinical features of this disorder. Methods: Ten consecutive subjects with PNH, all with epilepsy and at least two periventricular nodules, were studied with structural MRI and neuropsychological testing. Behavioral results were statistically analyzed for correlation with other features of PNH. Results: Eight of 10 subjects had deficits in reading skills despite normal intelligence. Processing speed and executive function were also impaired in some subjects. More marked reading difficulties were seen in subjects with more widely distributed heterotopia. There was no correlation between reading skills and epilepsy severity or antiepileptic medication use. Conclusion: The neuronal migration disorder of periventricular nodular heterotopia is associated with an impairment in reading skills despite the presence of normal intelligence.

This study includes 117 patients operated upon in the period from 1970 to 1999. Indications, surgical techniques, complications, and results are reviewed. Indications included: long-gap oesophageal atresia with or without fistula in 81 patients; peptic stenosis in 19; caustic stenosis in 12; oesophageal varices in 2; and 1 case each of oesophageal epidermolysis bullosa, total oesophageal leiomyomatosis, and a non-functioning antiperistaltic retrosternal colic graft operated upon in another hospital. A retrosternal bypass was performed 106 times: 98 first operations and 8 redos; the intrathoracic technique was used 19 times. The left transverse colon was used in 107 cases (85.6%), the right transverse colon in 8 (6.4%), and the ileocecum in 10 (8%). All the intestinal bypasses were placed in the isoperistaltic direction. There were 5 deaths in the first 11 years of our experience; no patient died from 1982 on. Ten complications were treated conservatively (8%): 2 wound infections healed with medical treatment, and 8 leaks of the cervical anastomosis closed spontaneously. The major surgical complications were 8 gangrenous bypassess (6.4%), removed and reopeated about 1 year later utilizing an ileocolic retrosternal graft. Three cases of peptic disease of the colic bypass (2.4%) were successfully treated with the author's technique. Nine patients had minor surgical complications (7.2%): 3 strictures of the oesophagocolic anastomosis in a retrosternal bypass (resected and reoperated) and 6 cases of adhesive occlusion. In our opinion, the best substitute of the oesophagus is the colon, particularly the left transverse segment, which may be placed behind the sternum or in the oesophageal bed, always in the isoperistaltic direction. The low mortality (4%), restricted to the early period of our experience, and few major surgical complications (6.4%) are acceptable considering the importance of the operation, and the long-term results may be considered very satisfactory.

The authors report the case of a female patient, affected by long-gap oesophageal atresia, who, at 5 months old, was operated on for retrosternal substitution with a right-transverse antiperistaltic colic segment, in her local district hospital. Due to the anomalous position the neooesophagus never worked, and the baby was seriously dysphagic and failed to thrive. For this reason, when she was 11 months old, she was reoperated in our department. Through a medium sternolaparotomy the antiperistaltic colon was removed and replaced between the left and right colon; reconstruction was carried out with a retrosternal and isoperistaltic segment of ileo-caecum. The reoperation resolved her problems. This case is reported to confirm the author's opinion that all intestinal conduits must be positioned in the isoperistaltic direction.

The purpose of this study was to determine by MR imaging the prevalence and types of dysraphic abnormalities of the spinal cord (i.e., myelodysplasias) associated with urogenital and anorectal malformations of childhood.Since 1987, 92 patients with imperforate anus complex, cloacal malformation, and cloacal exstrophy have had MR imaging as a screening examination for occult dysraphic myelodysplasia. The prevalence and types of myelodysplasia were determined for each group.The prevalence of dysraphic myelodysplasia in each group of children was 17% (1/6) for low imperforate anus (ectopic anus), 34% (11/32) for high imperforate anus (with fistulization), 46% (19/41) for cloacal malformation, and 100% (13/13) for cloacal exstrophy. The most common abnormalities in each group were tethered cord with intradural or filar lipoma in imperforate anus; low-placed or dysplastic conus medullaris and tethered cord with lipoma or myelolipoma in cloacal malformation; and lipomyelocele, lipomyelomeningocele, or lipomyelocystocele in cloacal exstrophy.Our results show that the prevalence of myelodysplasia as seen on MR imaging is high in patients with urogenital and anorectal anomalies.

The authors report a case of a two-year-old child with esophageal stricture -- caused first by caustic ingestion and by the end-to-end anastomosis, performed after the excision of the stenotic esophageal segment-- that required repeated dilations. These manouvres unfortunately led to bacteremia and a serious complication of a brain abscess, in the right fronto-parietal area, that was promptly removed. A few months later the child was subjected to an esophageal substitution with a colonic transposition. At present he is in good condition and the follow-up showed normal function of the neo-esophagus.

The authors report a case of duplication of the entire small intestine manifested by frequent haemorrhages originating from a peptic ulcer located distally to the duplication. The peptic ulcer was provoked by the ectopic gastric mucosa of the duplication. Surgery consisted "stripping" the mucosa of the duplicated tract according to Wrenn's technique. Other surgical methods for treating this malformation are discussed in detail. The literature describing similar cases is reviewed.