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Pelviureteric junction obstruction, also known as ureteropelvic junction obstruction, is a congenital narrowing of the urinary excretory tract at the junction between the renal pelvis and the ureter and is a common cause of congenital pelvicalyceal dilatation. The outcome is variable, from spontaneous resolution to renal parenchymal function loss in cases of untreated high-grade obstruction. Abnormalities in renal ascent, rotation and vascularity can be associated with pelviureteric junction obstruction and easily overlooked radiologically. In this pictorial review, we explore the anatomical, radiological and surgical correlations of pelviureteric junction obstruction in the context of a normal kidney and a spectrum of renal abnormalities, including hyper-rotation (also known as renal malrotation), failed renal ascent, fusion anomalies and accessory crossing renal vessels. For each scenario, we provide technical tips on how to identify the altered anatomy at the first ultrasound assessment and correlation with scintigraphic, cross-sectional and postoperative imaging where appropriate. A detailed ultrasound protocol specifically to assess and characterise pelviureteric junction obstruction in paediatric patients is also offered. [ABSTRACT FROM AUTHOR]

Upper gastrointestinal series is considered the gold standard imaging test to evaluate for malrotation and midgut volvulus. US has been explored for this purpose in recent years and has been shown to be a good alternative because of its relatively good accuracy, fast learning curve for sonographers and radiologists, as well as convenience for children, clinical staff and radiologists in the neonatal intensive care unit. In this article we describe our experience and review the evidence supporting the use of emergency US primarily to diagnose midgut volvulus as well as its use as a problem-solving tool for the diagnosis of malrotation. The examination technique, normal and abnormal findings are described, with emphasis on the whirlpool and superior mesenteric artery cutoff signs to diagnose midgut volvulus. [ABSTRACT FROM AUTHOR]

Background: Malrotation of the bowel refers to any variation in the rotation and fixation of the gastrointestinal tract during the first trimester and is most commonly detected postnatally. Nonrotation of the bowel and incomplete rotation of the bowel are subtypes of malrotation. Objective: To determine if the nonrotation subtype of malrotation of the bowel can be detected on prenatal magnetic resonance imaging (MRI). Materials and methods: Cases from 2012 to 2018 with nonrotation of the bowel without obstruction confirmed by imaging, surgery and/or autopsy were compared to prenatal imaging. Prenatal imaging was retrospectively reviewed to determine if prenatal diagnosis of malrotation could be made. Exclusion criteria included diaphragmatic hernia, omphalocele and gastroschisis. Results: Ten cases of nonrotation diagnosed postnatally by upper gastrointestinal series (upper GI)/small bowel follow-through (SBFT) or autopsy had prenatal MRI. Prenatal MR studies were performed for assessment of heterotaxy syndrome with congenital heart disease (6/10), congenital heart disease with additional anomalies (suspected VACTERL [vertebral, anorectal, cardiac, tracheoesophageal, renal, limb] and suspected lung agenesis, ventriculomegaly) (3/10) and skeletal dysplasia (1/10). Eight upper GI/SBFT cases demonstrated nonrotation of the bowel without obstruction with the small bowel completely on one side of the abdomen contralateral to the stomach and the colon ipsilateral to the stomach; four cases were confirmed by surgery. The small bowel in one upper GI/SBFT case was unilateral contralateral to the stomach with a meandering colon. One case had nonrotation diagnosed at autopsy. There were no cases of postnatal midgut volvulus. Retrospective review of the 10 cases had prenatal MRI performed between 23 and 37 weeks of gestation. The coronal plane was the most optimal plane to assess the position of the stomach, small bowel and colon in relationship to each other. The small bowel was best assessed on T2-weighted images while the colon was best assessed on T1-weighted images. A nonrotated position of the small bowel was present in all 10 fetal MRI cases mirroring postnatal findings, with the small bowel contralateral to the stomach in 9/10 cases and ipsilateral to the stomach (in the right abdomen) in 1/10 cases. The colon was visualized by prenatal MRI in 9/10 cases, with 1 case limited due to a lack of T1-weighted imaging. A nonrotated position of the colon contralateral to the small bowel was present in 7/9 cases. In 2/9 cases, the colon was wandering, positioned on both sides of the midline. Colonic position in all nine cases matched postnatal findings. No cases presented with prenatal bowel obstruction. Conclusion: Detection of nonrotation of the bowel is possible on prenatal MRI. [ABSTRACT FROM AUTHOR]

Abdominal US is emerging as an alternative to the upper gastrointestinal (GI) series as the preferred diagnostic imaging test for midgut malrotation complicated by volvulus. Unlike the upper GI series, US is free from ionizing radiation, does not require oral contrast agent, and can be performed portably and at times remotely from the interpreting radiologist, expediting diagnosis. Although some institutions do not have a standardized US protocol for midgut volvulus, many routinely use US in the setting of an infant or child with acute abdominal signs or symptoms to evaluate for common conditions such as hypertrophic pyloric stenosis, intussusception, necrotizing enterocolitis and appendicitis. Because these common conditions can overlap in age and clinical presentation with midgut volvulus, the aim of this pictorial essay is to provide instruction on the technique and diagnostic findings of midgut volvulus on US to ensure confident, accurate interpretation, and prompt treatment. [ABSTRACT FROM AUTHOR]

Focused abdominal sonography (US) has been suggested as a first-line modality for the diagnosis of malrotation and volvulus in newborns. In this paper, the author reviews the strengths and weaknesses of US in the diagnosis and exclusion of malrotation and volvulus. The author reviews published implementation programs for using US in both conditions and describes the anatomical variations that limit the utility of US depiction of a retromesenteric duodenum in the exclusion of malrotation. [ABSTRACT FROM AUTHOR]

Background: The clinical and plain radiographic differentiation of congenital intrinsic duodenal anomalies (atresia, web, stenosis) from intestinal malrotation is not always clear. Although sonography has been documented as an important diagnostic tool in the differentiation of these two entities, its role is still not widely appreciated and it is still not universally utilized in this clinical setting.Objective: To assess the usefulness of sonographic features of the duodenal and gastric wall in the differentiation of congenital intrinsic duodenal anomalies from midgut malrotation in a large series of neonates and to compare them with other features on abdominal radiographs, ultrasound and upper gastrointestinal series.Materials and Methods: Using the surgical database at our tertiary pediatric hospital, we identified neonates who had surgically proven congenital intrinsic duodenal anomalies or malrotation over a period of 15 years (2000-2015). We reviewed imaging findings in both groups of neonates (blinded to the final diagnosis) with attention to the echogenicity and thickness of the wall of the duodenum and stomach, the relationship between the superior mesenteric artery and vein, the position of the third portion of the duodenum and the presence of the whirlpool sign. Findings were compared between the groups using the unpaired t-test and Fisher exact test.Results: We included 107 neonates in the study, 40 with a congenital intrinsic duodenal anomaly, 49 with malrotation (36 with volvulus) and 18 with a combination of both. Duodenal and gastric wall thickening and hyperechogenicity were significantly more common in the group with a congenital intrinsic duodenal anomaly compared to those with malrotation (P<0.0001). Conversely, an abnormal relationship between the superior mesenteric artery and vein, abnormal position of the third part of the duodenum, and the whirlpool sign were significantly more common in neonates with malrotation than in those with congenital intrinsic duodenal anomalies (P<0.0001).Conclusion: Duodenal or gastric wall thickening, and increased wall echogenicity are helpful sonographic features in the differentiation of congenital intrinsic duodenal anomalies from malrotation. Evaluation of the duodenal and gastric wall should thus be added to the features routinely assessed on ultrasound examinations in the clinical setting of suspected duodenal obstruction in the neonate. [ABSTRACT FROM AUTHOR]

Background: Anatomical imaging findings indicating normal bowel rotation can be identified on cross-sectional imaging, including magnetic resonance imaging (MRI) performed for non-related indications.Objective: The goal of our study was to assess whether non-targeted MRI can accurately assess intestinal malrotation.Materials and Methods: Four anatomical landmarks were assessed on MRIs of the chest, spine or abdomen performed from January 2006 to June 2014, on patients who also had upper gastrointestinal series (UGI) performed within 10 years of the MRI date: 1) retroperitoneal duodenum, 2) left upper quadrant duodenojejunal junction, 3) superior mesenteric artery to the left of the superior mesenteric vein, and 4) right lower quadrant cecum. Two attending radiologists, one pediatric and one abdominal radiologist, independently reviewed the MR images. The pediatric radiologist reviewed images from UGI (considered the gold standard) to determine the intestinal rotation for each case. Validation of the criteria was performed on new patients imaged through January 2016.Results: The original cohort included 109 MRIs (15 chest, 41 spine and 53 abdomen) done on 109 patients (42% males, mean age: 10.2 years). If each of the 4 anatomical questions were answered "yes" (4-YES), specificity was 100% for each radiologist and malrotation was appropriately excluded. Using the 4-YES criteria, the pediatric radiologist excluded malrotation in 71 patients (65%) and the abdominal radiologist excluded it in 65 (60%), with concurrence for 57 patients. Validation of the 4-YES criteria in 23 new patients appropriately proved the 4-YES rule, with neither labeling the one new malrotation case 4-YES.Conclusion: If a radiologist can confidently answer "yes" to the four questions evaluated in this study, then intestinal rotation can be safely considered normal. Normal bowel rotation should be commented upon in MRI reports when these four anatomical locations are imaged, thus helping patients avoid unnecessary UGI and radiation exposure. [ABSTRACT FROM AUTHOR]

Background: Life-threatening midgut volvulus usually occurs in infants with malrotation and requires rapid diagnosis and surgical treatment to prevent bowel necrosis and death. However, because of the low frequency of upper gastrointestinal studies performed in infants younger than 1 month, many diagnostic radiology residents finish their residency training having limited or no opportunity to perform or observe an upper gastrointestinal (GI) series for evaluation of bilious emesis in a neonate.Objective: To determine whether adding simulated upper GI series on neonates with bilious emesis to the curriculum improves residents' skill and accuracy in diagnosing midgut volvulus.Materials and Methods: We assessed the performance of 12 residents in training whose curriculum included simulated upper GI series (study group) and 10 traditionally trained residents (control group) using a multiple-choice test, checklist procedure evaluation and diagnostic accuracy scores for 3 randomly selected simulated upper GI series. We then compared the results from the study group that had simulation curriculum to the scores for the control group using the Mann-Whitney test. We also analyzed the scores for the study group obtained prior to and after simulation curriculum using Wilcoxon signed rank test.Results: There was a significant difference in test scores (study group median = 84.5%, control group median = 67.2%, P=0.001), overall diagnostic accuracy (study group median = 100%, control group median = 50%, P=0.011) and checklist evaluation (study group median = 83.3%, control group median = 70.8%, P=0.025) for the residents in the study group who completed simulation curriculum compared with the control group. There was also a significant difference in multiple-choice test scores for the study group before and after completion of simulation curriculum (before simulation curriculum median = 56.9%, after simulation curriculum median = 84.5%, P=0.002), checklist evaluation (before simulation curriculum median = 58.3%, after simulation curriculum median = 83.3%, P=0.002) and overall diagnostic accuracy scores (before simulation curriculum median = 50%, after simulation curriculum median = 100%, P=0.024).Conclusion: Radiology residents had significantly higher scores on a multiple-choice test, checklist procedure evaluation and overall diagnostic accuracy after completing a structured pediatric fluoroscopy curriculum that included simulated neonatal upper GI series and when compared to a control group of traditionally trained residents. [ABSTRACT FROM AUTHOR]

Background: Midgut volvulus is a complication of malrotation of bowel and mesenteric malfixation. In contrast, primary volvulus of the small bowel is a distinctly different and rare entity characterized by torsion of the entire small bowel with normal mesenteric fixation.Objective: To present the clinical and imaging findings in four infants with primary small bowel volvulus and normal bowel fixation in order to improve awareness of this entity among clinicians and radiologists and to discuss the potential etiologies of this entity to distinguish it from other causes of small bowel volvulus.Materials and Methods: A retrospective review of imaging studies (two ultrasounds and four upper gastrointestinal series) in four infants (three full-term and one premature) from three institutions with surgically proven volvulus of the entire small bowel and normal bowel fixation were reviewed by three board-certified pediatric radiologists and correlated with clinical and surgical reports when available.Results: The infants presented during the first week to 6 months of life and were acutely ill. The upper gastrointestinal series showed complete duodenal obstruction with beaking in one and partial duodenal obstruction in three. All studies were interpreted as highly suspicious for malrotation and midgut volvulus. Emergent laparotomy demonstrated primary small bowel volvulus with normal mesenteric fixation in all infants. The base of the small bowel mesentery was described by the operating surgeon as smaller than normal in one infant (case 3). There was no mesenteric defect or other abnormality predisposing to volvulus in the other three. In both infants who had abdominal US, a retroperitoneal position of the third portion of the duodenum was demonstrated. All infants survived. One infant required resection of the necrotic small bowel and currently has short gut syndrome, one has malabsorption and two were lost to follow-up.Conclusion: Primary small bowel volvulus with normal fixation is indistinguishable from malrotation with midgut volvulus in the acutely ill infant or child. Radiographic diagnosis can be difficult in patients with intermittent or incomplete small bowel volvulus without malrotation. In these patients, neither an upper gastrointestinal series demonstrating a normal position of the duodenojejunal junction nor the sonographic demonstration of a retromesenteric third portion of the duodenum excludes the diagnosis. In young infants, the clinical and imaging findings may mimic necrotizing enterocolitis. Sonography may be useful to evaluate the bowel for signs of bowel wall compromise or a whirlpool sign. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

In the introductory article of a new series geared toward pediatric radiology trainees, the radiological management of newborn bilious emesis is discussed through the details of a specific case. [ABSTRACT FROM AUTHOR]

Background: The upper gastrointestinal (UGI) series is the preferred method for the diagnosis of malrotation. A bedside UGI technique was developed at our institution for use in low birth weight, critically ill neonates to minimize the risks of transportation from the neonatal intensive care unit (NICU) such as hypothermia and dislodgement of support lines and tubes. Objective: To determine the ability of a bedside UGI technique to identify the position of the duodenojejunal junction (DJJ) in low birth weight, critically ill infants in the NICU. Materials and methods: We retrospectively reviewed bedside UGI examinations performed in premature infants weighing less than 1,500 g from 2008 to 2013 and correlated the findings with clinical data, imaging studies and surgical findings. Results: Of 27 patients identified (weight range: 633-1,495 g), 21 (78%) bedside UGI series were diagnostic. Twenty of 27 cases (74%) demonstrated normal intestinal rotation. One case demonstrated malrotation with midgut volvulus, which was confirmed at surgery. In six cases (22%), the position of the DJJ could not be accurately determined. No cases of malrotation with midgut volvulus were missed. None of the patients with normal bedside UGI studies was found to have malrotation based on clinical follow-up (mean: 20 months), surgical findings or further imaging. Conclusion: The bedside UGI is a useful technique to exclude malrotation in critically ill neonates and minimizes potential risks of transportation to the radiology suite. Pitfalls that may preclude a diagnostic examination include incorrect timing of radiographs, patient rotation, suboptimal enteric tube position and bowel distention. In cases of diagnostic uncertainty, a follow-up study should be performed. [ABSTRACT FROM AUTHOR]

Background: Bowel rotation abnormalities in heterotaxy are common. As more children survive cardiac surgery, the management of gastrointestinal abnormalities has become controversial. Objective: To evaluate imaging of malrotation in heterotaxy with surgical correlation and provide an algorithm for management. Materials and methods: Imaging reports of heterotaxic children with upper gastrointestinal (UGI) and/or small bowel follow-through (SBFT) were reviewed. Subsequently, fluoroscopic images were re-reviewed in conjunction with CT/MR studies. The original reports and re-reviewed images were compared and correlated with surgical findings. Results: Nineteen of 34 children with heterotaxy underwent UGI, 13/19 also had SBFT. In 15/19 reports, bowel rotation was called abnormal: 11 malrotation, 4 non-rotation, no cases of volvulus. Re-review, including CT (10/19) and MR (2/19), designated 17/19 (90%) as abnormal, 10 malrotation (abnormal bowel arrangement, narrow or uncertain length of mesentery) and 7 non-rotation (small bowel and colon on opposite sides plus low cecum with probable broad mesentery). The most useful CT/MR findings were absence of retroperitoneal duodenum in most abnormal cases and location of bowel, especially cecum. Abnormal orientation of mesenteric vessels suggested malrotation but was not universal. Nine children had elective bowel surgery; non-rotation was found in 4/9 and malrotation was found in 5/9, with discrepancies (non-rotation at surgery, malrotation on imaging) with 4 original interpretations and 1 re-review. Conclusion: We recommend routine, early UGI and SBFT studies once other, urgent clinical concerns have been stabilized, with elective laparoscopic surgery in abnormal or equivocal cases. Cross-sectional imaging, usually obtained for other reasons, can contribute diagnostically. Attempting to assess mesenteric width is important in differentiating non-rotation from malrotation and more accurately identifies appropriate surgical candidates. [ABSTRACT FROM AUTHOR]

Background: Intestinal malrotation and particularly volvulus are potentially devastating conditions. Upper gastrointestinal (UGI) contrast studies have been considered the gold standard for diagnosis. However the use of ultrasonography (US) has been increasingly described. We describe a method for delineating the duodenal anatomy with US as a means to exclude malrotation. Objective: To report our experience using US to assess intestinal rotation. Materials and methods: We conducted a retrospective audit of US scans performed at a tertiary referral centre to exclude malrotation for paediatric surgery between 2008 and 2011. Results: One hundred thirty-nine infants were included, of whom 114 had a normal US scan. Of the 114, nine had subsequent upper gastrointestinal contrast studies that confirmed the initial results; there were no false-negatives. There were abnormal US scans in four infants associated with midgut volvulus and malrotation; there were no false-positives. The other 21 US scans were equivocal, and 11 of these had a confirmatory UGI contrast study; only one required surgery to correct malrotation. Conclusion: US has been a safe and effective tool in the assessment of intestinal rotation at our institution. The main advantages of US imaging are its lack of ionising radiation and its rapid and accurate diagnosis of volvulus. [ABSTRACT FROM AUTHOR]

Background: In some series of malrotation small numbers of children are described in whom the position of the duodenojejunal flexure was considered to be normal on straight anteroposterior (AP) view of an upper gastrointestinal (UGI) series. Objective: The purpose of this study was to illustrate children with disorders of midgut rotation in whom the diagnosis was difficult because on the straight AP view of the UGI series the duodenojejunal flexure was either not clearly depicted or was projected to the left of the midline close to its expected normal position at or close to the level of the duodenal cap. Materials and methods: We reviewed 111 children with malrotation to determine the frequency that duodenojejunal flexure was not clearly depicted or was close to normal position. Results: Seven patients had close to normal position of duodenojejunal flexure on AP view. The correct diagnosis was made on initial UGI series in four patients based on other features on AP and lateral views. In two of the other three patients, a repeat UGI series facilitated the correct diagnosis. In the final patient, an abnormal position of a nasojejunal tube suggested the correct diagnosis. Conclusion: Accurate diagnosis of anomalies of midgut rotation requires careful assessment of the entire duodenal sweep on both AP and lateral views to avoid false-negative interpretations. [ABSTRACT FROM AUTHOR]

Upper gastrointestinal contrast studies in children may cause false-positive or -negative diagnosis of intestinal malrotation from rotation of the patient. To alleviate this problem, skin markers can be used to reduce rotation of children undergoing this procedure, e.g., two metal markers (sheathed and sealed hypodermic needles) can be fixed onto the skin for gastro-intestinal contrast studies. We reviewed two Katz criteria influenced by patient rotation: duodenojejunal junction on or to the right of the left pedicle and pylorus to the left of the midline. A test group was positioned using markers; a control group without markers was positioned conventionally. Markers during a pilot study were applied, but positioning was done by helpers who had no on-screen visualization. In the test group, only 1 child (3%; n = 39) had a feature of malrotation. In the control group, there were features of malrotation in 12 children (25%; n = 48). No other features of malrotation were seen. The pilot study showed radiographic rotation with markers projecting off the midline in 78% of 58 children. This resulted in 48% of 58 patients having false features of malrotation. The use of metal skin markers results in reduction of rotational errors that could have caused false diagnosis of intestinal malrotation in children. [ABSTRACT FROM AUTHOR]

Background: The upper gastrointestinal (UGI) contrast study is used in the assessment of children with gastro-oesophageal reflux (GOR) and for detection of structural anomalies. The pH study is more sensitive than the UGI study for the diagnosis of GOR. The pH study has been replaced by the pH/impedance test, which detects both acid and nonacid reflux. Objective: To compare the UGI contrast study with the pH/impedance test for the diagnosis of GOR in children. Materials and methods: We retrospectively reviewed consecutive records of children investigated for GOR from October 2008 to February 2010, and compared the findings of UGI studies with those of pH/impedance tests. Results: The UGI studies revealed GOR in 116 of 579 children (20%). Of the children undergoing a UGI study, 66 also underwent a pH/impedance test. Using the pH/impedance tests as the reference for GOR, UGI had a sensitivity of 42.8% and a negative predictive value of 24%. There was no significant correlation ( P > 0.05) between the reflux index and the number of reflux episodes in the pH/impedance tests and height of reflux in the UGI study. There were low incidences of malrotation (0.9%), hiatus hernia (1%) and delayed gastric emptying (0.4%). Conclusion: The UGI study had low sensitivity for the diagnosis of GOR and low yield for the diagnosis of structural anomalies. [ABSTRACT FROM AUTHOR]

Background: Internal hernias are uncommon in children and their clinical and imaging findings have not been widely addressed . Objective: To determine the spectrum of clinical and imaging findings of internal hernia (IH) in children and to highlight diagnostic features. Materials and methods: Review of clinical, imaging and surgical findings in 12 children with surgically proven IH. Results: IH found in seven girls and five boys. Five of the children were neonates and seven were between ages 8-17 years. All neonates presented acutely and had transmesenteric internal hernias (TMIH) (four congenital, one acquired). In the older children, five presented with chronic symptoms and two presented with acute symptoms; the former had paraduodenal hernias (all congenital) and the latter had a congenital pericecal and an acquired TMIH. Only 2/5 neonatal TMIH could be appreciated on GI contrast examination. All five paraduodenal hernias were easily diagnosed on UGI series. CT, in two older children, depicted a paraduodenal hernia and the acquired TMIH. In 7/10 (70%) congenital IH, there was associated malrotation (in all four right paraduodenal hernias). Conclusion: There is a wide spectrum of clinical and imaging findings of IH in children. TMIH were difficult to appreciate on GI contrast examinations, but paraduodenal hernias were easy to appreciate. One must have a high index of suspicion for right paraduodenal hernia if UGI series shows duodenum and proximal small bowel to the right of the spine. [ABSTRACT FROM AUTHOR]

Background: Demonstration of the third duodenal segment (D3) in retroperitoneal location has been recently proposed as a method for excluding malrotation. Objective: This study was performed to determine whether a retroperitoneal third duodenal segment can reliably exclude malrotation. Materials and methods: CTs of 38 patients with proven malrotation and 100 patients without malrotation were evaluated for the location of the duodenum/proximal small bowel, and the relationship of the superior mesenteric vein (SMV) to superior mesenteric artery (SMA). Results: The D3 segment was in normal retroperitoneal location in 100% of control patients, compared to 2.5% or (1 of 38) of patients with malrotation. Nine of 11 patients (91%) with malrotation imaged prior to surgery had the proximal bowel in an abnormal location, while all 100 control patients had it in a normal location. The SMV was in normal relationship to the SMA in 11/38 patients (29%) with malrotation, compared to 79% of normal controls. In 10 controls, a branch of the SMV was partially wrapped around the SMA, potentially mimicking partial mesenteric volvulus. Conclusion: A retroperitoneal location of the D3 segment makes the diagnosis of malrotation unlikely but not impossible. Additional imaging of the duodenojejunal junction or cecum may be necessary to reliably exclude intestinal malrotation. [ABSTRACT FROM AUTHOR]

US can be used to assess bowel and does not require ionizing radiation or the administration of contrast material. Prior studies of the duodenum with US are limited. This study assesses the success rate of US demonstration of the third portion of the duodenum (D3) between the superior mesenteric artery (SMA) and the aorta in newborns to exclude malrotation based on embryologic and anatomic principles. Thirty-three newborns underwent US studies. The structures between the SMA and the aorta, including D3, were evaluated in axial and longitudinal planes. The length of time to acquire diagnostic images was recorded. In both the axial and longitudinal planes, D3 was seen between the SMA and the aorta in all 33 infants, including some with abundant bowel gas. The mean length of time to acquire diagnostic images was 34 s. Bedside US successfully illustrated the retromesenteric position of D3 in all 33 infants. Overlying gas-filled bowel was effectively effaced by graded compression. The short study duration indicates the practicality of the method. Further studies in broader patient populations and in correlation with other imaging and/or surgical findings is required to validate our technique. [ABSTRACT FROM AUTHOR]

The article offers information on the implication of malrotation with midgut volvulus in children. It is considered one of the surgical emergencies in children that usually occurs in the 4th and 10th week of gestation and characterized by the rapid growth and lengthening of midgut than the abdominal cavity forcing the bowel to herniate through the yolk sac. To correct the problem, laparoscopy in malrotation and midgut volvulus is done by surgeons.

The purpose of this communication is to highlight the shortcomings of all currently used imaging criteria in diagnosing or excluding malrotation and offer ultrasound demonstration of the 3rd portion of the duodenum (D3) between the AO and the SMA in transverse and sagittal plains as the most reliable diagnostic method. Although UGI is currently considered to be the imaging modality of choice in diagnosis of malrotations, numerous publications indicate that in certain patients, false positives and negatives can be encountered. The material consists of more than 10 years experience in university settings, during which the author has used US as the definitive imaging modality for the work-up of malrotation. High resolution linear transducers (5-17 MHz) are the transducers of choice. In overwhelming majority of cases, by illustrating a retromesenteric D3 malrotation and, therefore, midgut volvulus were excluded. None of the current imaging criteria addresses the following most fundamental anatomic and embryologic facts regarding the gut rotation and fixation. The position of the DJJ, the configuration of the duodenal sweep, the orientation of the mesenteric vessels are all wrong horses to bet on because none of them addresses the fundamental anatomic and embryologic facts. Only the cross-sectional imaging, US, CT and MRI can prove that the D3 is retromesenteric, therefore, excluding malrotation and volvulus. Therefore, demonstrating a retromesenteric duodenum is the reference standard of imaging in the work-up of malrotation, not any other previously published criteria. The US imaging is the most acceptable imaging method for malrotation work-up, in the spirit of ALARA principle and “Image Gently” campaign. [ABSTRACT FROM AUTHOR]

The complex and sometimes controversial subject of malrotation and midgut volvulus is reviewed commencing with the 19th-century Bohemian anatomist, Václav Treitz, who described the suspensory muscle anchoring of the duodenal-jejunal junction in the left upper quadrant, and continuing with William Ladd, the 20th-century American “father of pediatric surgery” who pioneered the surgical treatment of midgut volvulus. In this review, we present the interesting history of malrotation and discuss the current radiologic and surgical controversies surrounding its diagnosis and treatment. In the symptomatic patient with malrotation and possible midgut volvulus, prompt diagnosis is critical. The clinical examination and plain film are often confusing, and delayed diagnosis can lead to significant morbidity and death. Despite recent intense interest in the position of the mesenteric vessels on US and CT scans, the upper gastrointestinal series remains the fastest and most accurate method of demonstrating duodenal obstruction, the position of the ligament of Treitz, and, if the contrast agent is followed distally, cecal malposition. Controversy exists over the management of asymptomatic patients with malrotation in whom the diagnosis is made incidentally during evaluation for nonspecific complaints, prior to reflux surgery, and in those with heterotaxy syndromes. [ABSTRACT FROM AUTHOR]

Malrotation is a congenital disorder of abnormal intestinal rotation and fixation that predisposes infants to potentially life-threatening midgut volvulus. Upper gastrointestinal tract (UGI) examination is sometimes equivocal and can lead to inaccurate diagnosis. To determine the diagnostic performance of UGI examinations in children who subsequently underwent a Ladd procedure for suspected malrotation or volvulus. We reviewed all children up to 21 years old who had undergone both a UGI examination and a Ladd procedure for possible malrotation across 9 years. Children were excluded if they had not undergone either a UGI examination or a Ladd procedure and if congenital abdominal wall defects were present. Of 229 patients identified, 166 (59% male, median age 67 days) were included. Excluded were 47 without a UGI series, 12 with omphalocele or gastroschisis, 1 without verifiable operative data, 1 who had not undergone a Ladd procedure, and 2 older than 21 years. Of the 166 patients, 40% were neonates and 73% were <12 months old, and 31% presented with bilious vomiting and 15% with abdominal distention. Of 163 patients with surgically verified malrotation, 156 had a positive UGI examination, a sensitivity of 96%. There were two patients with a false-positive UGI examination and seven with false-negative examination. Jejunal position was normal in six of the seven with a false-negative examination and abnormal in the two with a false-positive examination. Of 38 patients with surgically verified volvulus, 30 showed volvulus on the UGI series. Five required bowel resection and three died. Jejunal position can lead to inaccurate UGI series interpretation. Meticulous technique and periodic assessment of performance will help more accurately diagnose difficult or equivocal cases. [ABSTRACT FROM AUTHOR]

Duodenal obstruction caused by ileocolic intussusception in the absence of intestinal malrotation is extremely rare. We present and discuss the imaging findings in an infant with an intussusception secondary to a duplication cyst in whom sonography also showed inversion of the orientation of the mesenteric vessels and a distended stomach. A contrast medium study revealed a proximal duodenal obstruction with a beak appearance suggestive of midgut volvulus. At surgery, an ileocolic intussusception causing duodenal obstruction without concomitant malrotation or volvulus was found. The combination of duodenal obstruction and abnormal relationship of the mesenteric vessels as a result of ileocolic intussusception has not previously been reported in the literature. [ABSTRACT FROM AUTHOR]

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare, often fatal condition. Infants present with a functional obstruction of the gastrointestinal tract (GI), malrotation, microcolon, and a large nonobstructed bladder. Several features common to both MMIHS and Eagle-Barrett or prune belly syndrome (PBS) include hydronephrosis, bladder distension and laxity of the abdominal wall musculature. Additionally, MMIHS and PBS have been reported in the same family, suggesting the possibility of a common pathogenesis. MMIHS usually presents in female infants. We present a male infant diagnosed with both MMIHS and PBS. This is a unique case in which both MMIHS and true PBS are present in the same infant. [ABSTRACT FROM AUTHOR]

Malrotation with volvulus is one of the true surgical emergencies of childhood. Prompt radiological diagnosis is often paramount to achieving a good outcome. An understanding of the normal and anomalous development of the midgut provides a basis for understanding the pathophysiology and the clinical presentation of malrotation and malrotation complicated by volvulus. In this essay, the radiologic findings of malrotation and volvulus are reviewed and illustrated with particular attention to the child with equivocal imaging findings. [ABSTRACT FROM AUTHOR]

A case of congenital short gut is reported in a 4-month-old boy presenting with failure to thrive. Upper gastrointestinal examination (UGI) with small bowel follow-through (SBFT) demonstrated dilation of the duodenum and jejunum, with rapid transit to rectum. On barium enema (BE), rapid transit of barium was noted to the dilated proximal small bowel seen on UGI/SBFT. Neither study delineated the ileocecal region, and the overall length of bowel appeared short. The diagnosis of congenital short gut was confirmed at surgery. Congenital short-gut syndrome is a rare entity. The diagnosis can be suggested by imaging, but is usually confirmed operatively. [ABSTRACT FROM AUTHOR]

malrotation and Hirschsprung's disease are common gastrointestinal abnormalities in neonates and infants. Four patients with the association of malrotation and Hirschsprung's disease are reported. A delay in diagnosis may be complicated by midgut volvulus or enterocolitis. The diagnostic and therapeutic implications of this important association are discussed. [ABSTRACT FROM AUTHOR]

Two cases of midgut malrotation are presented. Each had a clinical picture suggesting a possible intussusception. The radiographs also demonstrated findings suggestive of intussusception though these were subtle findings which could have suggested the correct diagnosis of malrotation. [ABSTRACT FROM AUTHOR]

This paper describes the radiological findings obtained in 20 patients with the Cornelia de Lange syndrome. The pattern profiles of the hand skeleton were studied in these patients, using the method described by Poznanski et al. This study revealed a short first metacarpal bone, combined with relatively long third and fourth metacarpals, to be the most characteristic feature of the syndrome. No radiological characteristic was found to be pathognomonic of the syndrome, but the combination of a deformed proximal metaphysis of the radius with the above mentioned abnormality in the pattern profile of the hand is described as highly suggestive of the Cornelia de Lange syndrome. [ABSTRACT FROM AUTHOR]

The life of Martin H. Wittenborg was short. During his tenure at Boston Children's Hospital, he wrote brilliant papers based on his observations regarding such entities as intestinal malrotation and vascular rings and slings. He wrote about his extensive experience using radiotherapy in the treatment of neuroblastoma and its effects on the growing spine. This article reflects the memories of some of his students, who describe him as a brilliant teacher. His effect on so many and his contributions are all the more remarkable considering the fact that Wittenborg was deaf. [ABSTRACT FROM AUTHOR]

We report a case of intestinal malrotation without any associated GI tract complications diagnosed antenatally by fetal MRI. Antenatal US revealed a midline stomach. Subsequent fetal MRI confirmed the midline stomach and, in addition, revealed all loops of small bowel to the right of the midline and all large bowel to the left. All these features were consistent with intestinal malrotation. There was no abnormal bowel wall thickening, bowel dilatation, ascites or polyhydramnios. To our knowledge, this is a unique case of intestinal malrotation without associated GI tract complications diagnosed antenatally on fetal MRI. [ABSTRACT FROM AUTHOR]

The article offers information on the diagnosis of malrotation and volvulus in infants and children. It is a congenital abnormality characterized by the abnormal rotation of the bowel of both small and large bowel within the peritoneal cavity, leading to acute or chronic volvulus and bowel necrosis. To manage the ailment early, initial bowel imaging is vital to prevent volvulus and bowel ischemia. The diagnostic imaging techniques recommended for malrotation are also discussed.

The article offers information on the attributes of malrotation and significance of radiology in its treatment. The illness is characterized as the abnormal development of the midgut associated with abnormal rotation and/or fixation, resulting to the obstruction of the duodenum due to peritoneal bands that anchor the cecum to the retroperitoneum. Before surgery is done on patients, surgeons rely on radiologist to confirm or exclude the diagnosis.

We present a case of malrotation with midgut volvulus in an infant in which we discovered a unique CT sign called the perfusion cut-off sign. We hope this case will help establish this crucial diagnosis in other cases. [ABSTRACT FROM AUTHOR]

Intestinal malrotation is a developmental anomaly affecting the position and peritoneal attachments of the small and large intestines during fetal life. Most often the diagnosis is established in the first year of life on the basis of abdominal pain and bile-stained vomiting secondary to bowel obstruction. The antenatal diagnosis can be suggested by identification of the complications such as bowel dilatation, ascites or meconium peritonitis. We describe two cases of isolated antenatal gastric malposition without any other associated anomaly that were confirmed after birth to be due to intestinal malrotation. We suggest that such an antenatal finding should alert the paediatrician to close clinical follow-up and prompt the diagnosis and surgical treatment in case of abdominal pain and/or bilious vomiting. [ABSTRACT FROM AUTHOR]

We report on a 10-year-old girl who presented with worsening pain and anorexia after blunt trauma to the abdomen. Contrast-enhanced CT of the abdomen was performed, and a counterclockwise rotation of the superior mesenteric vein around the superior mesenteric artery was seen. An upper gastrointestinal (UGI) series with small-bowel follow-through demonstrated a normally located duodenal-jejunal junction. This is the first case report of a counterclockwise barber-pole sign seen by CT with UGI that was negative for malrotation or volvulus. [ABSTRACT FROM AUTHOR]

Midgut volvulus, the most common serious complication of malrotation, can be diagnosed using conventional contrast fluoroscopy, US or CT. CT is a quick and comprehensive examination in the evaluation of complex acute abdominal pathology in children. Contrast-enhanced CT can readily help the radiologist recognize perfusion abnormalities of the bowel, which is vital for reducing morbidity and mortality in affected children. Our case emphasizes and demonstrates additional CT features of bowel infarction in a child with a proven malrotation with midgut volvulus. [ABSTRACT FROM AUTHOR]

A case of non-rotation of the bowel is presented wherein volvulus of the colon occurred causing acute obstruction. Spontaneous reduction following a barium enema as well as the patient's history leads us to suspect that volvulus had occurred in the past and was responsible for the intermittent nature of the patient's symptoms. This type of volvulus is distinctly different from midgut volvulus and is a rare complication of the anomaly of intestinal rotation and fixation known as non-rotation of the bowel. Efforts should be made to study patients with malrotation anomalies at a time when they are acutely symptomatic so that intermittent volvulus such as shown here is not overlooked. [ABSTRACT FROM AUTHOR]

The radiographic pattern of high intestinal obstruction - gastric and duodenal distension with absence of small bowel gas (ASBG) or a completely gasless abdomen - may be present in infants with midgut volvulus. In most instances, ASBG is associated with viable small bowel. However, when associated with abdominal distension and tenderness ASBG usually reflects strangulated midgut volvulus and bowel necrosis. In some patients with midgut volvulus there is a radiographic pattern of low small bowel obstruction this is generally associated with necrotic bowel. These seemingly opposite observations can be explained by reviewing the dynamics of gas absorption in normal bowel and in closed loop intestinal obstruction. Impaired blood supply can result in either gaslessness or gaseous distension under differing conditions. [ABSTRACT FROM AUTHOR]