Congenital intestinal malrotation presenting in octogenarians: a report of two cases.

Malrotation is part of a spectrum of small and large bowel positional and fixational abnormalities caused by the failure of the fetal intestine to complete a 270-degree rotation around the superior mesenteric artery axis. Rarely, it presents in the adult as a cause of acute small bowel obstruction. Chronic symptoms of malrotation in adults are subtle, and include intermittent abdominal pain, nausea and vomiting. We present two cases of malrotation in octogenarian men presenting acutely with small bowel obstruction. Both patients were treated with emergency surgery. In one case the chronic symptoms resolved postoperatively. Malrotation and midgut volvulus should be considered as a rare differential diagnosis for small bowel obstruction in adults. Suspicions should be increased when there is a history of recurrent presentations with similar symptoms.

Malrotation in Congenital Diaphragmatic Hernia: Is It Really a Problem?

INTRODUCTION: Congenital diaphragmatic hernia (CDH) has been associated with other congenital malformations, such as intestinal rotation abnormalities. However, there is no standard evaluation and treatment of intestinal rotation during initial CDH repair. This study evaluates intestinal malrotation in CDH patients. MATERIALS AND METHODS: All patients with a CDH treated in a high-volume center between 2000 and 2015 were retrospectively evaluated. Demographics, gastrointestinal characteristics, surgical treatment, and abdominal outcomes (acute surgery, small bowel obstruction, and volvulus) were described. RESULTS: A total of 197 CDH patients were surgically repaired. In 76 (39%) patients, a malrotation was described at initial CDH repair, in 39 (20%) patients, a normal rotation, but 82 patients (42%) had no report on intestinal rotation. During follow-up (range: 2-16 years), 12 additional malrotations were diagnosed, leading to a prevalence of at least 45% (n = 88). These were mostly diagnosed due to acute small bowel obstruction, of which three had a volvulus. Of the 12 later diagnosed malrotations, 58% required acute surgery, compared to 3% of the 76 initially diagnosed patients (p < 0.001). CONCLUSION: Malrotation is associated with CDH, with a prevalence of at least 45% and in 21% a normal intestinal rotation. "Missed" malrotations have a higher risk on need for acute surgery later in life.

In utero ileal volvulus and intestinal perforation associated with enteric duplication cyst presenting with preterm labour and acute abdomen in newborn.

Enteric duplication cysts are a rare cause of intestinal obstruction in the neonatal period. We present the unusual case of an in utero ileal volvulus secondary to an enteric duplication cyst causing an acute abdomen in a 35-week estimated gestational age newborn female delivered to a mother in preterm labour.

Adult with intestinal malrotation and colocolic intussusception: an unusual combo.

Intestinal malrotation is a congenital anomaly that results from an abnormality in the rotation and fixation of the gut as it returns to the abdominal cavity during development. Intussusception is a condition characterised by telescoping of one segment of the bowel into another segment. The combination of malrotation and intussusception is frequently a cause of intestinal obstruction in the paediatric age group. The coexistence of these two conditions is termed as Waugh syndrome and is uncommon in adults. We share our experience with an elderly woman who was admitted with diarrhoea and anaemia. Investigations revealed a large colonic polyp, colocolic intussusception and malrotation. The adenomatous polyp and mobile right colon would have lead to intussusception. The diagnosis was based on the findings of colonoscopy and contrast-enhanced CT scan of the abdomen. She was managed with a transverse colectomy with an uneventful recovery.

Transverse colon volvulus in a patient with sickle cell disease.

Although colonic volvulus is a relatively rare cause of large bowel obstruction, accounting for up to 5% of all cases of intestinal obstruction, transverse colon volvulus is extremely uncommon compared with volvulus of the sigmoid colon or caecum and is responsible for only 3% of all reported cases. We report an unusual case of spontaneous volvulus of the transverse colon in a young man with sickle cell disease who underwent resection with primary anastamosis. Having a high index of suspicion and early operative intervention allowed for this patient to have an uneventful postoperative course.

Mimics of malrotation on pediatric upper gastrointestinal series: a pictorial review.

Intestinal malrotation is a continuum of congenital anomalies due to lack of rotation or incomplete rotation of the fetal intestine around the superior mesenteric artery axis. The abnormal bowel fixation (by mesenteric bands) or absence of fixation of portions of the bowel increases the risk of bowel obstruction, acute or chronic volvulus, and bowel necrosis. The clinical presentation of patients with malrotation without, with intermittent, or with chronic volvulus can be problematic, with an important minority presenting late or having atypical or chronic symptoms, such as intermittent vomiting, abdominal pain, duodenal obstruction, or failure to thrive. The diagnosis is heavily reliant on imaging. Upper GI series remain the gold standard with the normal position of the duodenojejunal junction lateral to the left-sided pedicles of the vertebral body, at the level of the duodenal bulb on frontal views and posterior (retroperitoneal) on lateral views. However, a variety of conditions might influence the position of the duodenojejunal junction, potentially leading to a misdiagnosis of malrotation. Such conditions include improper technique, gastric over distension, splenomegaly, renal or retroperitoneal tumors, liver transplant, small bowel obstruction, the presence of properly or malpositioned enteric tubes, and scoliosis. All of these may cause the duodenojejunal junction to be displaced. We present a series of cases highlighting conditions that mimic malrotation without volvulus to increase the practicing radiologist awareness and help minimize interpretation errors.

Small bowel volvulus in the adult populace of the United States: results from a population-based study.

BACKGROUND: Small bowel volvulus is a rare entity in Western adults. Greater insight into epidemiology and outcomes may be gained from a national database inquiry. METHODS: The Nationwide Inpatient Sample (1998 to 2010), a 20% stratified sample of United States hospitals, was retrospectively reviewed for small bowel volvulus cases (International Classification of Diseases, 9th Edition [ICD-9] code 560.2 excluding gastric/colonic procedures) in patients greater than or equal to 18 years old. RESULTS: There were 2,065,599 hospitalizations for bowel obstruction (ICD-9 560.x). Of those, there were 20,680 (1.00%) small bowel volvulus cases; 169 were attributable to intestinal malrotation. Most cases presented emergently (89.24%) and operative management was employed more frequently than nonoperative (65.21% vs 34.79%, P < .0001). Predictors of mortality included age greater than 50 years, Charlson comorbidity index greater than or equal to 1, emergent admission, peritonitis, acute vascular insufficiency, coagulopathy, and nonoperative management (P < .0001). CONCLUSION: As the first population-based epidemiological study of small bowel volvulus, our findings provide a robust representation of this rare cause of small bowel obstruction in American adults.

Heterotaxy syndrome infants are at risk for early shunt failure after Ladd procedure.

BACKGROUND: Cardiac-specific risks and complications after a Ladd procedure in patients with heterotaxy syndrome (HS) and intestinal rotational anomalies (IRA) are unknown. We sought to (1) describe rates of hospital mortality and early systemic-to-pulmonary (S-P) artery shunt failure after the Ladd procedure in patients with HS and (2) explore risk factors associated with early shunt failure in patients with HS with single ventricle (SV). METHODS: This retrospective study included all Ladd procedures performed from January 1999 to December 2012 in patients with HS at a single center. Risk factors investigated for early S-P artery shunt failure included birth weight, gestational age, sex, age at and timing of Ladd procedure relative to cardiac operations, and shunt type. RESULTS: Ladd procedure was performed on 54 infants with HS and congenital heart disease. Hospital mortality for the entire cohort was 5.6% (3 of 54 patients). Early shunt failure occurred in 19% (4 of 21) of HS infants with SV. Mean preoperative blood urea nitrogen (BUN) levels were higher in HS infants with early shunt failure (20 versus 12.5 mg/dL; p = 0.054). CONCLUSIONS: Patients with SV and HS with S-P artery shunts are at risk for early shunt failure after a Ladd procedure. A higher mean preoperative BUN level is noted in patients with HS and early shunt failure. Careful risk-benefit analysis is indicated before recommending routine elective Ladd procedures in patients with HS.

Cerebral candidiasis in a 4-year-old boy after intestinal surgery.

Cerebral candidiasis is a devastating disease which contributes to a high mortality. Most of the cerebral candidiasis are never microbiologically or radiologically confirmed. In this case, a 4-year-old boy who developed cerebral candidiasis was successfully rescued and presented. The diagnosis of cerebral candidiasis was established based on both microbiologic and radiologic examinations. The pathogen was revealed to be Candida albicans by cerebrospinal fluid and central venous catheter cultures, and the cerebral involvement was recorded by series head magnetic resonance imaging (MRI) with an appearance of special encephalitis demonstrated. The imaging studies played a critical role throughout the diagnosis and treatment. Familiarity with the imaging findings in the appropriate clinical setting may result in a heightened level of awareness of this infection and, consequently, in earlier diagnosis and treatment.

Unusual uptake of radioiodine in the small bowel in a patient with thyroid cancer and bowel malrotation.

Whole-body radioiodide scan is an essential procedure in the evaluation of radioiodine-avid metastatic foci in patients with differentiated thyroid carcinoma. Normal physiologic distribution of the radiotracer includes the stomach and colon, but the small intestine is not often seen. Gastrointestinal anatomic variants may be associated with atypical activity on I scan. We report here a case of intestinal malrotation with associated I uptake in the small intestine. Careful correlation of planar imaging with SPECT/CT revealed the likely malrotation as well as small bowel uptake superimposed over normal colonic uptake. This finding was occult on prior I scan.

Laparoscopic management of midgut malrotation and tuberculous peritonitis in an adolescent boy.

An unusual case of miliary plastic peritonitis associated with midgut malrotation in an adolescent in whom various diagnostic investigations, medical management and staged initial laparoscopic diagnostic followed by therapeutic procedure was curative have been presented. Tuberculous miliary plastic peritonitis causes dense adhesions between bowel loops and prevents midgut volvulus in a pre-existing malrotation. Diagnosis can be a challenge and threshold for diagnostic laparoscopy should be low. Interval correction of malrotation by laparoscopy after complete resolution of tuberculosis is easy, safe and effective, and cosmetically more pleasing. Midgut malrotation is a congenital anomaly referring to either lack of or incomplete rotation of fetal axis around the axis of superior mesenteric artery during fetal development. Most patients present with bilious vomiting in the first month of life because of duodenal obstruction or a volvulus. This is an unusual case of midgut malrotation in association with tuberculous peritonitis in an adolescent boy.

Bedside upper gastrointestinal series in critically ill low birth weight infants.

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.

Midgut malrotation with congenital peritoneal band: a rare cause of small bowel obstruction in adulthood.

Midgut malrotation commonly presents in the neonatal period, and rarely manifests its symptoms in adulthood with an estimated incidence of 0.2-0.5%. Nevertheless, the symptoms are non-specific with no strong pointers towards the clinical diagnosis. Consequently, the diagnosis is usually disclosed with imaging or surgery. We report a case of small bowel obstruction secondary to a congenital peritoneal band with underlying midgut malrotation in a 48-year-old man.

Malrotation of Intestine with Carcinoma Colon in Adult.

Malrotation of gut is a congenital anomaly and patients usually present in childhood. Occasionally it may present in adulthood. Patients are usually asymptomatic when malrotation of gut is detected during investigations or operation. Also, it can cause longstanding abdominal symptoms and volvulus of gut. Rarely malrotation of gut may present with gastrointestinal neoplasm. We present a 60 year old female with carcinoma caecum along with intestinal malrotation.

A case of cancer of the ampulla of Vater accompanied by malrotation.

Intestinal malrotation is caused by a developmental anomaly of the embryonic intestine. Most cases develop in neonates, and development in adulthood is rare and difficult to diagnose before surgery. Pancreaticoduodenectomy was performed for cancer of the ampulla of Vater accompanied by incomplete fixation in a 63-year-old male patient. A branch of the superior mesenteric artery was present on the resection line and was deemed likely to cause circulatory disorder in the small intestine, and the duodenum and jejunum were covered with a membranous structure making dissection, anatomical identification, and jejunectomy difficult. Herein, we report the case with a review of the literature.

Severely high serum unbound bilirubin level after abdominal surgery in a newborn.

We report a newborn with intestinal malrotation who developed a severely high serum unbound bilirubin level and a low serum albumin level without a marked increase in serum total bilirubin level after abdominal surgery, which required exchange transfusion and albumin supplementation. The serum unbound bilirubin level may be highly relative to the serum total bilirubin level in newborns who have undergone abdominal surgery soon after birth and are hypoalbuminemic after surgery.