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Purpose: The purpose of the study was to evaluate our experience with enteric duplication cysts in 40 children during the past 26 years, while assessing the variability of their presentations and to propose an algorithm for surgical management., Methods: We retrospectively analysed sex, age, clinical presentations, duplication site, surgical treatment, presence of ectopic tissue, complications, associated anomalies, and prognosis of 40 patients with gastrointestinal tract duplications who were surgically treated in our clinic., Results: Overall, there was a predominance of boys (28 males, 70 %; 12 females, 30 %). The presenting symptom was vomiting in 23 patients, rectal bleeding in 11 patients, abdominal mass in 10 patients, abdominal pain in 9 patients, constipation in 6 patients, cough in 2 patients, and respiratory distress in 2 patients. In 30 patients, a complete excision of the cyst with additional segmental intestinal resection and anastomosis was performed. Cystectomy was performed in seven patients, while complete excision of the cyst with additional wedge resection was performed in two. A Wrenn procedure (mucosectomy) was performed in one patient., Conclusion: Due to the variability in the site of enteric duplications, a wide range of presenting symptoms can exist, which is challenging for diagnosis. In children with a diagnosis of acute abdomen, enteric duplication cysts should be considered, and these children should be further investigated for additional skeletal, urogenital, and gastrointestinal system pathologies. Surgical treatment depends on the site and type of the cyst.

Rotation abnormalities may be asymptomatic or may be associated with obstruction caused by bands, midgut volvulus, or associated atresia or web. The most important goal of clinicians is to determine whether the patient has midgut volvulus with intestinal ischemia, in which case an emergency laparotomy should be done. If the patient is not acutely ill, the next goal is to determine whether the patient has a narrow-based small bowel mesentery. In general, the outcomes for children with a rotation abnormality are excellent, unless there has been midgut volvulus with significant intestinal ischemia., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Intestinal obstruction due to congenital intestinal malrotation is usually diagnosed in neonates but may, in rare cases, occur during pregnancy. The absence of specific symptoms in combination with its low incidence makes timely detection of intestinal malrotation-related obstruction difficult in expectant mothers. We present a rare case of a 23-year-old woman with a twin pregnancy following in vitro fertilization-embryo transfer (IVF-ET) who presented with symptoms of intestinal obstruction at 22+4 weeks of gestation. This diagnosis was not confirmed by imaging and the patient was managed conservatively. Following caesarean section, she gave birth to two healthy full-term infants. During the operation, malposition of the bowel and the typical Ladd's band confirmed intestinal malrotation. This is the first report of a congential malrotation complicating a multiple pregnancy, and highlights that malrotation without volvulus can be managed conservatively.

Gall bladder (GB) duplication is a rare anomaly, not often seen in regular clinical practice. Though a vestigial organ, the presence of calculi within the GB can cause the patient to be acutely symptomatic with right hypochondriac pain, which can even be life threatening if not treated. The presence of two GBs means this pathology can be seen in both the GBs, highlighting the importance of diagnosing this condition, lest the patient returns years after a cholecystectomy with cholecystitis yet again!, Competing Interests: Conflicts of Interest: None declared., (2017 BMJ Publishing Group Ltd.)

Paul SP, Hicks SS, Sanjeevaiah MK, Heaton PA. Where did the salt go? Turk J Pediatr 2017; 59: 345-348. Bronchiolitis is a self-limiting viral respiratory-tract-infection seen commonly in infants. Some infants require hospitalization for feeding or respiratory support. A wide range of extra-pulmonary complications such as arrhythmias, myocarditis, central apneas, seizures, and hyponatremia are uncommonly known to occur with respiratory syncytial virus (RSV) infections. We present a 4-week-old-female infant admitted with RSV bronchiolitis for feeding support by nasogastric-tube. The infant suffered unexpected desaturations and seizure-like event 30-hours post-admission. Severe hyponatremia (sodium: 114 mmol/L) was detected although cause for this remained unexplained initially. Serum sodium improved following a bolus of 2.7% hypertonic-saline. The infant subsequently needed advanced respiratory support. Around time of transfer to PICU, the infant developed abdominal distension and continued to have bilious aspirate even after 6-days. An upper gastrointestinal contrast-study confirmed malrotation; improved following surgery. Co-existence of two serious pathologies may have accounted for the hyponatremia: malrotation (possible source of sodium loss into third-space) and severe bronchiolitis (inappropriate ADH-secretion). This case highlights the importance of determining origin of hyponatremia associated with acute bronchiolitis.

Background: Determining the cause of bowel obstruction without a history of laparotomy (BO without HL) is difficult and can result in delay of treatment and development of a potentially life-threatening situation. We herein investigated the clinical characteristics of pediatric patients who underwent laparotomy due to BO without HL., Methods: All surgical pediatric patients aged <16 age who were diagnosed with BO without HL between January 2004 and September 2014 were included. Etiology of BO, intraoperative findings and postoperative outcome were investigated retrospectively., Results: Seventy patients were diagnosed with BO without HL in this period. In these 70 patients, malrotation (n = 34), intussusception (n = 19), internal hernia (n = 6) and Meckel's diverticulum (n = 5) were predominantly identified. Regarding preoperative definitive diagnosis, prevalence of internal hernia, Meckel's diverticulum or idiopathic volvulus was significantly lower than that of malrotation or intussusception (P < 0.05). Intraoperatively, the rates of strangulation and bowel resection were 55.7% and 30.0%, respectively. The optimal time for emergency operation in order to avoid strangulated bowel resection was <19 h from onset of symptoms., Conclusions: Malrotation and intussusception are major causes of BO without HL in children, but internal hernia, Meckel's diverticulum and idiopathic volvulus should always be taken into account, particularly because of the preoperative diagnostic difficulty and resulting high rate of intestinal resection. In order to avoid resection of the bowel, surgery should be done within 19 h before bowel ischemic change occurs., (© 2016 Japan Pediatric Society.)

Background: The aim of this study was to evaluate clinical presentation, diagnostic studies, and volvulus rate and to describe the unusual clinical clues of intestinal malrotation., Methods: A retrospective descriptive review was carried out of all patients diagnosed with intestinal malrotation between 2002 and 2014. Patients were divided into two groups: infants (≤1 year, n = 16; group 1); and children (>1 year, n = 12; group 2). Patient demographics, clinical history, symptoms, physical examination, diagnostic work-up, operative findings and early outcome were evaluated., Results: Bilious vomiting was the cardinal complaint in both groups. Unusual symptoms such as respiratory insufficiency, dehydration, afebrile convulsion, and lethargy were prominent symptoms in six patients in group 1, whereas history of frequent hospitalization due to recurrent abdominal pain and feeding intolerance were prominent in six patients in group 2. Midgut volvulus was identified in 15 patients, four of whom were in group 2. Standard Ladd's procedure was done in addition to correction of volvulus., Conclusion: Malrotation with or without midgut volvulus is not a rare condition and should be kept in mind for any age group. Specific signs of diagnosis are not easily identified. In the case of unusual clinical presentation, diagnosis may be delayed and can result in catastrophic consequences if intestinal perfusion occurs. Although midgut volvulus is seen most frequently in infants, risk and complication rate are high beyond 1 year of age as well, and can manifest as failure to thrive, food intolerance, and abdominal pain needing recurrent hospitalization. Diagnostic suspicion and interdisciplinary coordination are essential for timely diagnosis and surgical treatment., (© 2016 Japan Pediatric Society.)

A 66-year-old man attended a health check-up after a positive fecal occult blood test. Colonoscopy revealed a 2 cm type 2 lesion near the ascending colon and the upper lip ofBauhin 's valve. Contrast-enhanced computed tomography ofthe abdominal region revealed aplasia ofthe ligament ofTreitz, with the duodenum running through the center. Three-dimensional vascular construction revealed intestinal malrotation, in which the superior mesenteric artery was present on the right side of the superior mesenteric vein. A diagnosis ofascending colon cancer(T2N0M0, cStage I )was made, and a laparoscopic ileocecal resection was performed. The ascending colon was not fixed to the abdominal wall, and the colon was widely depressed within the pelvis. Because no adhesion was noted, vascular processing was performed after converting to extracorporeal manipulation via a small incision in the umbilical region. The controlling vessel was the ileocolic artery, and D2 dissection was performed. The patient suffered no postoperative complications and was discharged on hospital day 13. Most reports ofintestinal malrotation concern pediatric patients, and reports ofadult cases are relatively rare. The intestinal malrotation was confirmed intraoperatively, and the procedure was performed safely. This suggests that if intestinal malrotation can be confirmed, laparoscopic surgery can be safely performed.

Objective: Recurrent acute pancreatitis is an uncommon diagnosis in teenagers. Excluded alcohol and biliary stones, more prevalent aetiologies in these group of patients are genetic pancreatitis, pancreatic duct system abnormalities, neoplasia, traumas and congenital abnormalities of the duodenum such as duodenal duplication or diverticulum. Two reported cases of recurrent pancreatitis associated to midgut malrotation were described in English literature. Bowel malrotation is a difficult diagnosis in a teenager or a young adult and a common delay is documented. Ladd's procedure is largely recognized to be the standard treatment for a symptomatic malrotation., Case Report: Our Report describes in details a case of recurrent pancreatitis, where a late diagnosis of midgut malrotation was obtained and an endoscopic management was attempted. A literature review and an analysis of two previously reported cases were performed to explore a possible aetiopathogenesis of the recurrent acute pancreatitis in patients with midgut malrotation., Results: 24 months of follow-up showed an asymptomatic patient on a free diet, with a mild deranged Liver Functional Tests and a normal Amylase and Lipase. The recurrence of acute pancreatitis has not been observed until the present day., Conclusions: Recurrent episodes of acute pancreatitis in young adults, without a history of alcohol abuse or evidence of gallstones, might be an atypical presentation of midgut malrotation and it should be in the differential diagnosis. In this case, a Ladd's operation is beneficial and an endoscopic procedure does not obtain advantages.

The pancreatic biliary maljunction is a rare anomaly that affects mainly females, defined as an anatomical maljunction of the pancreatic duct and the biliary duct confluence, and may be a rare cause of recurrent acute pancreatitis. In order to early diagnosis and prompt treatment, ERCP has an important role in it.

Background: This report deals with two deaths of children involving intestinal volvulus, i. e. a pathological knotting and twisting of the mesenterial root on the basis of congenital malrotation followed by obstruction and extensive ischaenia., Case Reports: (1) A 10-year-old girl (premature, 25th week) with severe disability, amaurosis and epilepsy was admitted to hospital due to general agitation and a bloated abdomen without peristaltic sounds, but died some hours later. Autopsy revealed a volvulus of 2/3 of the small intestine based on congenital malrotation with additional clamping of the intestine underneath adhesions (previous appendectomy). The abdominal cavity showed beginning peritonitis as the cause of death. (2) A 2-month-old premature male baby (surviving twin, 29th week) with a persisting ductus arteriosus was hospitalised for four weeks after failed vascular surgery due to acute general deterioration. Radiological diagnostics using a contrast medium revealed a vascular anomaly (right-sided aortic arch). Around 10 hours later, the infant developed an acute abdomen with ileus symptoms. Emergency surgery showed infarction of the entire small intestine due to an anti-clockwise 180⁰-volvulus, with death occurring 24h later. Further examination showed a malrotation as anomaly. Apparently, the volvulus had been caused by extensive use of contrast medium resulting in increased intestinal mobility.

Introduction Malrotation of the midgut and appendiceal mucocoele are both extremely rare pathological conditions in adults. To our knowledge, there are only two reported cases in the English literature with a combination of both conditions. Case History A 65-year-old man presented with a 10-day history of upper abdominal pain associated with abdominal bloating and weight loss. He was otherwise fit and healthy with no significant past medical history. On examination, his abdomen was soft with tenderness and palpable fullness over the left upper quadrant. The initial blood test, chest x-ray and abdominal x-ray demonstrated no significant abnormality. Computed tomography showed a 17cm x 8cm x 6cm elongated cystic mass with possible malrotation of the intestines. Histopathology showed a low grade mucinous tumour of the appendix. At 12 months following surgery, there was no evidence of recurrence or postoperative complications and the patient was discharged from the care of the colorectal team. Conclusions We report a patient with a combination of two rare conditions. This case illustrates how a combination of pathologies can present a challenge to the unwary general surgeon.

Occurrence of accessory lobes of liver and anomalies of gall bladder is quite common. A thorough knowledge of their variation can minimise diagnostic and surgical errors. We found concurrent variations of liver and gall bladder. A small accessory liver lobe was attached to the quadrate lobe through a stalk formed by peritoneum. The gall bladder was "bean shaped" due to the presence of a constriction in the middle of its body. Since the accessory lobe was quite close to gall bladder, it could compress the gall bladder and hinder normal functioning of it. The knowledge of these variations might be of importance to radiologists and surgeons dealing with the hepatobiliary system.

Foreign body ingestion is not an uncommon problem in clinical practice. While most ingested foreign bodies pass uneventfully through the gastrointestinal tract, sharp foreign bodies such as toothpicks should cause intestinal perforation. We reported the case of a perforation of the appendix caused by a toothpick, which also pierced the liver without hepatic damages, in a male with an intestinal malrotation and subhepatic appendix. The patient was admitted to our hospital for abdominal pain in the right upper quadrant. An abdominal computed tomography scan revealed the anomalous position of the first portion of the large intestine with inflamed appendix. A laparoscopic appendicectomy and the exploration of the abdominal cavity was performed using minimally invasive technique.

Purpose: To determine the number of term infants with bilious vomiting (BV) referred to a neonatal surgical centre for exclusion of malrotation by upper gastrointestinal contrast (UGI) examination., Methods: Retrospective review of term (>37/40) neonates <28 days of age undergoing UGI for exclusion of malrotation between Jan 2010 and Dec 2014 in a neonatal network with 30,000 term deliveries annually. Only infants with BV in the absence of alternative clinical/radiological diagnosis were included., Results: One hundred and sixty-six infants met the inclusion criteria. Fourteen (9 %) infants had malrotation diagnosed by UGI and confirmed at laparotomy. Only 1 of 110 infants referred at 0-2 days of age had positive UGI compared to 13 of 56 infants referred after this age (p < 0.01). An increase in referrals followed the death of an infant from midgut volvulus and as a result one in 500 term infants are currently being referred., Conclusion: Increasing awareness of the potential consequences of bilious vomiting appears to have resulted in increased referrals with no increase in detection of malrotation. Prospective studies are required to determine whether investigation of all infants with unexplained bilious vomiting is required and if it is possible to select cases for surgical referral.

Two children developed hepatoblastoma concurrent with congenital portosystemic shunts (PSSs) (Abernethy malformations). Both underwent operative ligation of their PSSs. One received concurrent tumor resection, whereas the other was deemed initially unresectable and underwent biopsy followed by neoadjuvant chemotherapy. Although benign hepatic masses, such as focal nodular hyperplasia and nodular regenerative hyperplasia, are common in patients with Abernethy malformations, malignant tumors have also been documented and should always be considered in the differential diagnosis of a patient with a congenital PSS and a hepatic mass.

Background: There are a lot of different causes of abdominal pain; in this case, a young woman suffers from three diseases with similar symptoms. Adult intestinal mal-rotation is a rare condition of deviation from the normal 270° counter clockwise rotation of the midgut resulting in, not only mal-position of the small intestine, but also mal-fixation of the mesentery. Fitz-Hugh-Curtis syndrome is a rare complication of pelvic inflammatory disease; it involves liver capsule inflammation associated with genital tract infection, which is usually caused by Neisseria gonorrhoea and Chlamydia trachomatis. Neuroendocrine tumors are enterochromaffin cell neoplasms that arise from cells of the endocrine (hormonal) and nervous systems; the appendicular one is the most common primary malignant lesion of these tumors, it's incidence is about 0.3 - 0.9% of appendectomies done. Just for knowledge, this is the first described case of concomitant presence of all these diseases with clinical symptoms attributable to each one., Case Presentation: 40-years-old woman suffers from acute abdominal pain, predominantly on the right quadrants, without abdominal distension, no guarding nor rigidity and normal intestinal peristalsis. She has a long history of abdominal intermittent pain, with cramps every 30-40 min, resolving spontaneously. She was diagnosed as intestinal mal-rotation through computed tomography scan which has evidenced a mobilized intra--peritoneal duodenum with cecum/ascending colon predominately lying on the left side and the small intestine almost entirely lying on the right side of abdomen, without evidence of effusion, edema or signs of intestinal ischemia or infarction. Exploratory laparoscopy demonstrated an inflammatory process in the hepatic-renal space, with bloody adhesions above the liver capsule; this is additional to the typical pelvic inflammatory disease signs (Fitz-Hugh-Curtis syndrome). Appendectomy was performed with histological analysis resulting in appendicular neuroendocrine tumor., Conclusions: Although the patient has an intestinal mal-rotation which could explain the abdominal painful symptoms, it is not possible to exclude other concomitant causes, such as perihepatitis on pelvic inflammatory disease or neuroendocrine tumors. Even if all these diseases are rarely seen in daily clinical practice, they should be considered in the differential diagnosis of chronic intermittent abdominal pain in a young woman.

Introduction: Malrotation of midgut is considered to be a condition of childhood. This study evaluated malrotation in adults with recurrent abdominal pain (RAP)., Methods: Sixty-four consensus-confirmed cases of intestinal malrotation were reviewed. The diagnosis was based on radiological criteria, and the consensus was arrived at by at least three of the five authors in any individual case., Results: Abnormal duodenojejunal junction (DJJ) was a consensus finding in 64 cases referred for RAP. Most were in their fourth decade of life, and 12 were beyond 60 years. Besides RAP, intolerance to food was the next common symptom. Acute intestinal obstruction was seen in 16. Forty-two of 64 patients consented for surgery. Ladd's procedure was the commonest. All patients who underwent surgery were symptom free except for two, of which, one had liver cyst and the other had hernia. Of those who refused surgery (22), all had continued symptoms and 10 patients took alternative therapies. On follow up of initially unwilling patients (for surgery) with abnormal DJJ, only eight consented for surgery; three underwent open Ladd's procedure, and one had laparoscopic Ladd's done., Conclusion: Malrotation is not uncommon as a cause of RAP in adults.

Objective: This study is the first in the literature to characterize and detail the clinical and surgical experience of one of the few multidisciplinary salivary gland disorder clinics in North America., Methods: This is a retrospective chart review of a descriptive nature, including patient information from February 2013 to August 2014. The Salivary Gland Disorder Clinic (SGDC) is located at Nationwide Children's Hospital, a public academic hospital. All patients seen at the SGDC were included in this study, 54 in total. Patients were seen for any salivary gland disorder presentation, ranging from sialorrhea to glandular masses., Results: A total of 70.4% of patients presented to the SGDC with a chief complaint of sialorrhea. Nearly 28% had previously experienced documented aspiration, and drooling severity and frequency was 4.06 and 3.3 on the Thomas-Stonell and Greenberg Drooling Rating Scales. 50% of patients presented with neurologic comorbidities and 25.9% with aerodigestive tract anatomic defects. Over 35% of patients had previously attempted medical treatment and 31.5% had no prior treatment attempts. 87% of all patients were offered further treatment options, including interventional, surgical, medical, and conservative measures., Conclusions: Pediatric salivary gland disorders, especially sialorrhea, can profoundly impact overall health and quality of life. Appropriate management of this symptom requires expert training and knowledge of the upper aerodigestive tract anatomy and appropriate treatment modalities. Our study demonstrates that there is community need for a centralized salivary gland disorder clinic given the complex comorbidities and social challenges that face this population. Many patients present with great risk for aspiration-related complications and the vast majority are offered new treatment options., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objective: Neonatal gastric perforation is a rare and serious issue. This study aimed to highlight the vital clinical features and identify prognostic factors in such cases., Design, Setting, Patients, Interventions, and Measurements: Medical charts from January 1997 through December 2008 were reviewed retrospectively. Neonates with a diagnosis of gastric perforation were included., Results: Thirteen patients were identified with a male:female ratio of 9:4. Five (38%) were preterm infants. The mortality rate was 30% (4/13), and the median age of onset was 3 days (range: 1-14 days). The most common presenting sign was abdominal distension, followed by respiratory distress and vomiting. Except for one patient in whom gastric perforation was diagnosed during surgical repair for gastroschisis, all patients had pneumoperitoneum on admission; 70% and 46% of patients had peritonitis and sepsis, respectively. Concomitant gastrointestinal (GI) tract anomalies or disorders included ischemic bowel/necrotizing enterocolitis (5 patients), intestinal malrotation (2), duodenal web (1), hiatal hernia (1), and gastroschisis (1), which necessitated secondary operations during hospitalization in 5 patients. Seven patients had leukopenia on admission, and 9 developed thrombocytopenia in the following 48 h. All patients who died presented with leukopenia on admission and thrombocytopenia in the following 48 h, yielding sensitivity and specificity rates of 100% and 67%, respectively., Conclusions: Neonatal gastric perforation is often concomitant with GI anomalies or inflammatory/infectious disease. Patients who were outborn and those with leucopenia, peritonitis, and thrombocytopenia development within 48 h were at risk for poor outcome., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Prompt diagnosis of malrotation and midgut volvulus in infants with bilious emesis is critical. However because of the limited frequency of pediatric upper gastrointestinal (UGI) fluoroscopic procedures in neonates, many diagnostic radiology residents complete their training never having seen or performed a UGI on a baby for evaluation of malrotation and midgut volvulus. A UGI simulation model for infants with bilious emesis was created to supplement the hands-on fluoroscopic experience of residents in training. We are now studying the addition of simulated UGI studies to our pediatric radiology curriculum.

Although the outcome of newborns with surgical congenital diseases (e.g., diaphragmatic hernia; esophageal atresia; omphalocele; gastroschisis) has improved rapidly with recent advances in perinatal intensive care and surgery, infant survivors often require intensive treatment after birth, have prolonged hospitalizations, and, after discharge, may have long-term sequelae including gastro-intestinal comorbidities, above all, gastroesophageal reflux (GER). This condition involves the involuntary retrograde passage of gastric contents into the esophagus, with or without regurgitation or vomiting. It is a well-recognized condition, typical of infants, with an incidence of 85%, which usually resolves after physiological maturation of the lower esophageal sphincter and lengthening of the intra-abdominal esophagus, in the first few months after birth. Although the exact cause of abnormal esophageal function in congenital defects is not clearly understood, it has been hypothesized that common (increased intra-abdominal pressure after closure of the abdominal defect) and/or specific (e.g., motility disturbance of the upper gastrointestinal tract, damage of esophageal peristaltic pump) pathological mechanisms may play a role in the etiology of GER in patients with birth defects. Improvement of knowledge could positively impact the long-term prognosis of patients with surgical congenital diseases. The present manuscript provides a literature review focused on pathological and clinical characteristics of GER in patients who have undergone surgical treatment for congenital abdominal malformations.

Objective: Management of short bowel syndrome (SBS) has significantly evolved recently. We present our single-center, 25-year experience focusing on the implementation of a refined multidisciplinary SBS care protocol., Methods: This is a retrospective review of the patients with SBS treated at our tertiary center from 1988 to 2014, with either <25% short bowel remaining or duration of parenteral nutrition (PN) >3 months. Patients with primary intestinal motility disorders were excluded. Clinical characteristics, including intestinal anatomy, markers of cholestasis, and catheter-related infections (CRIs), were analyzed. The implementation of a refined modern uniform management protocol in 2009 divided the cohort into 2 subgroups, whose outcomes are compared., Results: Forty-eight patients with SBS were identified (median gestational age 33 weeks). Of them, 22 were born between 2009 and 2014. The main causes of SBS were necrotizing enterocolitis (46%) and midgut volvulus (23%). Median remaining small bowel length was 36 cm. The overall survival was 23 of 26 (88%) before 2009 and 21 of 22 (95%) thereafter, whereas none were transplanted. Duration of PN shortened from a median of 15 to 6 months (P = 0.0015) in the latter cohort, whereas frequency of autologous intestinal reconstruction surgery (31%) remained unchanged. Frequency of neonatal cholestasis was similar in both groups (75%), but cleared in all after 2009. Before 2009, 2 patients died of progressive cholestatic liver failure. The CRI rates decreased from 1.7 to 0.7 per 1000 catheter-days between 2000-2008 and 2009-2014, respectively (P = 0.0178)., Conclusions: Uniform refined multidisciplinary approach decreased the duration of PN and CRI rates with high transplant-free survival and avoidance of liver failure, although the frequency of transient neonatal cholestasis remained unchanged.

Visceral myopathy is one of the causes of chronic intestinal pseudo-obstruction. Most cases pathologically reveal degenerative changes of myocytes or muscularis propia atrophy and fibrosis. Abnormal layering of muscularis propria is extremely rare. We report a case of a 9-mo-old Thai male baby who presented with chronic intestinal pseudo-obstruction. Histologic findings showed abnormal layering of small intestinal muscularis propria with an additional oblique layer and aberrant muscularization in serosa. The patient also had a short small bowel without malrotation, brachydactyly, and absence of the 2(nd) to 4(th) middle phalanges of both hands. The patient was treated with cisapride and combined parenteral and enteral nutritional support. He had gradual clinical improvement and gained body weight. Subsequently, the parenteral nutrition was discontinued. The previously reported cases are reviewed and discussed.

Serous microcystic adenoma of the pancreas is an uncommon tumor in adults, and reported in pediatric patients only a handful of times. The authors present a case of a 6 month patient with incidental finding of a large cystic mass on the tail of the pancreas during an operation for Hirschsprung's disease. Final pathology was consistent with serous microcystic adenoma. This is the second case report describing this lesion in an infant, and the first time it has been associated with Hirschsprung's disease.

Abnormalities and variations of the biliary ducts are not rare. Most aberrant bile ducts eventually drain into the descending part of duodenum through the papilla of vater. However, drainage of the left hepatic bile duct into the stomach is extremely rare. A 29-year old man was admitted to the hospital with the diagnosis of biliary reflux gastritis. Comprehensive imaging modalities were performed including electronic endoscopy, endoscopic ultrasonography, endoscopic retrograde cholangiopancreatography and magnetic resonance cholangio-pancreatography. Finally, congenital ectopic left intrahepatic bile duct draining into the stomach was found, which caused biliary reflux gastritis. The patient did not receive any surgery. Good recovery was achieved by medical treatment.

Heterozygous mutations in GATA6 have been linked to pancreatic agenesis and cardiac malformations. The aim of this study was to describe a new mutation in GATA6 in an infant with pancreatic agenesis, associated with truncus arteriosus and absent gallbladder. Clinical data were obtained from chart review. Gene sequencing was performed on genomic DNA. The patient was a female infant diagnosed shortly after birth with a severe cardiac malformation, absent gallbladder, anomalous hepatic blood flow, unilateral hydronephrosis and hydroureter, neonatal diabetes, and pancreatic exocrine insufficiency. Despite prolonged intensive management care, she died at 3 months of age because of cardiac complications. Analysis of her genomic DNA revealed a novel missense mutation of GATA6. The novel mutation described in this case extends the list of GATA6 mutations causing pancreatic agenesis and cardiac malformations., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Congenital anomalies of the digestive system represent a complicated topic concerning many medical specializations. The goal of this article is to describe two cases of children with mesenterium commune. First was an infant (5.5 months old female) who died shortly after being admitted to hospital with acute bronchitis, vomiting and diarrhoea. The autopsy revealed the cause of death--volvulus of the entire small intestine and the first portion of the large intestine with haemorrhagic infarsation of the intestinal wall in an infant with congenital anomaly of intestinal fixation--mesenterium commune. Second case was a 2.5 years old female after two heart surgeries, with pulmonary hypertension, who died suddenly at home. The cause of death was volvulus of a portion of the small intestine with haemorrhagic infarsation of the intestinal wall and also mesenterium commune. Congenital malpositions of the intestine originate due to malrotation and malfixation of the intestine during prenatal and early postnatal period. This wide range of the individuals developmental disorders always result in a condition where the topographical findings in the abdominal cavity are not as commonly found. Abnormally positioned intestines cannot develop a normal mesentery and are prone to volvulus, which represents the most serious complication with acute abdominal symptoms and when diagnosed late, it can lead to sudden death. Whilst performing autopsies such cases are rarely seen. However in forensic medicine and also in clinical practice it is important to consider intestinal malposition as a cause of acute abdomen.

The rare combination of intestinal lymphangiectasia with malrotation of the duodenum in a child of three months of life is described. Basing on the literature review only 3 similar cases were described in the world practice. The boy with protein-losing enteropathy was examined at Moscow Scientific Centre of Children's Health. The child had vomiting, diarrhea, loss in body weight, hypoproteinemia, lymphopenia. The infectious nature of the disease was excluded. It had been suggested the Waldman desease (primary intestinal lymphangiectasia). The prognosis for such disease is unfavorable. An examination of the child was continued against the backdrop of ongoing symptomatic therapy. Complete physical examination included monitoring laboratory blood tests, X-ray examination with contrast, CT-scan, gastroduodenoscopy with biopsy of the mucosa of the small intestine. Malrotation duodenum with the recurrent mid-gut volvulus with the development of secondary intestinal lymphangiectasia was diagnosed. Modern methods of examination and multidisciplinary approach made it possible to diagnose the case. Operation to eliminate fixation duodenum resulted in the recovery of the patient. At the present time the child grows and develops according to age and does not require treatment. The prognosis for this disease is regarded as favorable.

The sterno-costal hernia of Morgagni is a congenital vice in which is represented unfinished fusion of the transverse septum and the muscular diaphragmal wall. The malrotation syndrome is an embryonic vice as well, with various degree of incomplete and even reverse rotation of the gut, because of disturbed or unfinished development of the midgut. The described case presents a 37 years old female without anamnesis of any previous complains, who was pointed to the clinic after a X-ray photograph because of breathless and asteno-adynamic syndrome. After a precise CT investigation performance was found the presence of the above described anomalities, as well as ectopy of the right kidney and the right liver part. An operative treatment was performed with correction of the hernia and the bowel malrotation. The patient was followed up to the 12th month post-operatively. After a review of the literature in the discussion we inspect the diagnostics and the definition of the right surgical tactics and operative approach in this case with combined malformative pathology.

Background: Chronic pancreatitis (CP) is a rare disease in childhood. Although ERCP is commonly performed in children, the effect of pancreatic duct stenting therapy in children with CP is unknown., Objective: To investigate the efficacy of pancreatic duct stenting in children with CP., Design: Retrospective analysis., Setting: National referral center., Patients: A total of 208 children with CP hospitalized between 1988 and 2012., Interventions: ERCP with pancreatic duct stenting., Main Outcome Measurements: Results of endoscopic therapy and number of pancreatitis episodes per year before and after treatment., Results: A total of 223 pancreatic duct stenting procedures were performed in 72 children. The median number of stent replacements was 3 (range 1-21). A statistically significant decrease in the number of pancreatitis episodes per year was observed: from 1.75 to 0.23 after endoscopic treatment (P < .05). Pancreatic duct stenting was performed more frequently in patients with hereditary pancreatitis (61.5%) and in children with CP and anatomic anomalies of the pancreatic duct (65%; P < .05)., Limitations: Retrospective analysis with the assessment of adverse events based on medical history., Conclusion: Pancreatic duct stenting therapy is a safe and effective procedure in children with CP. This therapy should be recommended especially for children with hereditary pancreatitis and patients with anatomic anomalies of the pancreatic duct., (Copyright © 2014 American Society for Gastrointestinal Endoscopy. Published by Elsevier Inc. All rights reserved.)

Background and Objectives: Waugh syndrome (WS) is the association of intussusception and intestinal malrotation. The association is rarely reported in the literature though intussusception is a commonly encountered problem in pediatric patients as a cause of intestinal obstruction. We present our experience in 7 patients with a review of published reports., Design and Setting: Retrospective analysis of 7 patients with the diagnosis of Waugh syndrome who were treated at our department between February 1982 to December 2012., Patients and Methods: Seven patients with Waugh syndrome presented to our unit during the period February 1982 to December 2012. The clinical findings and management are presented and discussed., Results: Seven patients (three males and four females) presented with intussusception in association with mal.rotation. The age range was from 4 to 11 months; the patients had bilious vomiting and blood in the stool; the diagnosis was confirmed by ultrasound (2), Ba enema (2) and intraoperatively (3). All required operative intervention; either manual reduction or bowel resection and Ladd procedure; one patient died of sepsis; recurrence of obstruction was seen in another patient while the rest did well postoperatively., Conclusion: The relationship between intestinal malrotation and intussusceptions may be more frequent than is reported; failure of non-operative management of intussusception may be due to this association and hence brings the attention to its existence. A prospective study is needed to look for intestinal malrotation in patient with intussusceptions who undergo abdominal sonographic examination to determine the true incidence of this association. The anomaly is suspected by presence of a reversed anatomic relationship of the superior mesenteric artery and vein and in such cases to perform an upper gastrointestinal contrast study to define the exact location of the duodenojejuonal (DJ).

Introduction: Currarino syndrome is a type of caudal regression syndrome characterized by the association of hemisacrum, anorectal malformation and presacral mass. Only few studies on small series report the incidence of urinary dysfunction in Currarino syndrome. Our aim was to evaluate the urological outcome in patients with Currarino syndrome., Patients and Methods: We retrospectively reviewed all Currarino syndrome patients treated in our institution. Of 20 patients, we could evaluate the urological outcome in 16. This group of patients underwent clinical, radiological and urodynamic evaluation., Results: All 16 patients had a sacral defect, fourteen of them presenting a presacral mass (87.5%), eight a tethered cord (50%), and 7 anorectal malformations (43.7%). Eight patients underwent neurosurgical treatment for neural tube defects. In 14 patients, the presacral mass was resected. One case presented detrusor overactivity, 2 recurrent urinary tract infections and 2 vesicoureteral refluxes. Both patients with lipomyeloschisis had a neuropathic bladder. All the other patients could void the bladder spontaneously. Renal function was normal in all., Conclusion: Currarino syndrome is a rare congenital disorder presenting a variable phenotype. Urological outcome is good in the majority of patients., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Intestinal malrotation is an abnormality that usually presents in infanthood. Following correction, complications are rare in adults. We describe a case of a 64-year-old woman with a history of malrotation who presented with anaemia and weight loss. Colonic cancer was apparently excluded by colonoscopy. In fact, endoscopy had only been performed up to the hepatic flexure, which was unexpectedly positioned in the right iliac fossa. The patient then underwent a CT pneumocolon study, which demonstrated a caecal tumour, unusually located subhepatically. Repeat colonoscopy was performed to the true caecum and the lesion was biopsied. The patient underwent uncomplicated open right hemicolectomy and made a full recovery. To avoid diagnostic delay and, at worst, missing pathology entirely, clinicians must always thoroughly review a patient's history. Adults with malrotation may have atypical presentations of abdominal disease and so it is pertinent that surgeons, endoscopists and general practitioners remain alert and cognisant of their patients' history., (2014 BMJ Publishing Group Ltd.)

Alimentary tract duplications are rare congenital lesions normally diagnosed in newborns and children that can occur anywhere from the mouth to the anus and have a reported incidence of approximately 1 in 4500 life births. Symptoms and clinical presentation vary greatly. The presentation varies according to age and location. The treatment finally is surgical; total resection when possible should be the aim of the intervention. In pediatric surgery minimally invasive surgical procedures became more and more important over the last decades. In consequence the operative procedure on alimentary tract duplications changed in this manner. We review on case reports and clinical reports on minimally invasive surgery in the treatment of alimentary tract duplications, determine the importance of minimally invasive techniques in the treatment of this rare entity and rule out that further studies in the field should be performed.

We report a case of non-identical twins who presented with identical neonatal intestinal obstruction with features of anorectal stenosis, presacral mass and sacral anomaly consistent with Currarino's syndrome or triad. Plain sacral radiograph, contrast enema and MRI were diagnostic. Initial management involved a defunctioning colostomy followed by a posterior sagittal anorectoplasty with excision of the teratoma ± anterior sacral meningocele and finally closure of colostomy in a staged multidisciplinary approach. The twins' father is also affected with features of Currarino's syndrome but was diagnosed during family screening. Currarino's syndrome presenting with identical neonatal low intestinal obstruction in a non-identical set of twins is rare and interesting. Antenatal diagnosis of Currarino's syndrome is difficult and may prove to be a challenge even in the postnatal period. Sacral spine radiograph, contrast enema and MRI are diagnostic. Management requires high index of suspicion, low threshold for MRI and multidisciplinary staged approach., (2014 BMJ Publishing Group Ltd.)