Presentation
The infant will present with bilious vomiting in the first 24 hours. Xray of the abdomen reveals the classic double bubble. See associated PowerPoint presentation. [elearn.uta.edu]
Volvulus in a neonate or infant almost always presents as a midgut volvulus secondary to intestinal malrotation. [amboss.com]
In conclusion, we present a rare case of malrotation with midgut volvulus in an adult. Given his acute presentation, operative intervention was the natural trajectory in his management. [academic.oup.com]
NG tube is placed and met with resistance. > If a transesophageal fistula is present to the distal esophagus, gas will be present in the bowel. [quizlet.com]
Reported presentations are typically quite similar to the patient presented hereyears of episodic nonspecific symptoms, including crampy abdominal pain, nausea, and constipation. [facs.org]
Entire Body System
- Trisomy 21
Risk Factors: Associated Syndromes Familial Intestinal Malrotation Marfan Syndrome Prune Belly Syndrome Chromosomal abnormalities Trisomy 13 Trisomy 18 Trisomy 21 Other conditions (e.g. [fpnotebook.com]
We report a case of prolonged nonchylous ascites associated with intestinal malrotation in an infant with trisomy 21 who was eventually treated successfully with surgical intervention. [journals.lww.com]
Related conditions include cystic fibrosis, Trisomy 21 (Down syndrome), congenital heart disease, bowel rotational abnormality, annular pancreas, esophageal atresia, anorectal atresia and genitourinary conditions. [chop.edu]
Chromosomal abnormalities (mainly trisomy 18 or 21) are found in about 20% of fetuses. Other major defects, mainly cardiac, are found in about 50% of the cases. [sonoworld.com]
She had trisomy 21. She had been operated on for malrotation at 8 months (Valdoni’s procedure) and for duodenal stenosis at 9 months (duodenotomy and resection of a congenital web). [e-ce.org]
- Collapse
Other signs include a triangular loop, the beak sign, and the presence of two collapsed bowel loops adjacent to the obstruction site [ 55 ]. [uptodate.com]
[…] intraluminal bleeding and hematochezia, seen in 10% to 15% of patients with volvulus. 37 As ischemia of the midgut supervenes, the abdomen becomes distended and firm, with physical signs of peritonitis, and the patient will present in shock with cardiovascular collapse [clinicalgate.com]
The atretic region and the adjacent distended proximal and collapsed distal bowel are isolated with sterile moist swabs. [intranet.tdmu.edu.ua]
- Poor Feeding
Other symptoms include bowel obstruction with bilious vomiting, abdominal distention, poor feeding, and failure to thrive. Older children with Hirschsprung disease have usually had chronic constipation since birth. [intranet.tdmu.edu.ua]
Respiratoric
- Aspiration
Immediate management includes bowel decompression (by continuous nasogastric suction to prevent emesis, which can lead to aspiration pneumonia or further abdominal distention with respiratory embarrassment) and referral to a center for neonatal surgery [merckmanuals.com]
In some instances, duodenal stenosis goes unrecognized for long periods, leading to recurrent episodes of vomiting, failure to thrive, and aspiration pneumonia. [journals.lww.com]
Antenatal aspiration may be considered in cases of massive cysts resulting in thoracic compression. [sonoworld.com]
If esophageal atresia w/o tracheoesophageal fistula there is no air in bowel > Tx- NG tube in proximal pouch on intermittent suction to drain secretions and prevent aspiration, elevate head of bed (prevent reflux of gastric contents through the distal [quizlet.com]
This prevents vomiting and aspiration, as well as providing babies with some relief from the discomfort caused by a swollen abdomen. Nutrients are provided intravenously, to replace what was lost through vomiting. [chp.edu]
Gastrointestinal
- Failure to Thrive
Infants usually present with failure to thrive, recurrent vomiting, and diarrhea. [ijri.org]
Results: All three children presented late (median age 8 [range 2 – 9] years) with bilious vomiting, upper abdominal distension, and failure to thrive. One of them had associated Down syndrome and another had horseshoe kidney. [thieme-connect.com]
In some instances, duodenal stenosis goes unrecognized for long periods, leading to recurrent episodes of vomiting, failure to thrive, and aspiration pneumonia. [journals.lww.com]
Other less common presentations can include failure to thrive, solid food intolerance, malabsorption, chronic diarrhea Evaluation For children that are stable upon presentation, radiologic evaluation is indicated, including plain radiographs and an upper [pedclerk.bsd.uchicago.edu]
Consult a pediatrician right away for a diagnosis if your child shows symptoms such as: Vomiting green fluid Drawing up their legs Swollen belly Diarrhea Constipation Failure to thrive Fast heart rate Fast breathing Bloody stools Stomach pain Adults may [webmd.com]
- Chronic Abdominal Pain
Even more rare is to remain completely asymptomatic into adulthood, as majority of the cases typically present early on with acute bowel obstruction, intestinal ischemia, chronic abdominal pain or volvulus. [journals.lww.com]
Symptomatic patients may present with either acute abdominal pain due to midgut volvulus, or chronic abdominal pain due to proximal midgut partial obstruction in the presence of congenital bands. [smj.org.sg]
We describe a 20-year-old female patient with chronic abdominal pain and a disordered eating pattern that were interpreted as being psychosomatic despite extensive somatic evaluations. [ntvg.nl]
However, some may present with chronic symptoms of intermittent bowel obstruction or vague abdominal complaints. Even fewer may report acute episodes of agonizing abdominal pain [ 13 ]. [ispub.com]
Patients with malrotation can present in infancy or in adulthood with acute abdominal pain and bilious emesis, with an acute volvulus, with typical reflux symptoms, or with chronic abdominal pain. [merckmanuals.com]
Skin
- Erythema
Remarkable abdominal distention, tenderness and/or erythema of the abdominal skin indicates perforation, which requires immediate surgery. [aafp.org]
The presenting symptoms include the acute onset of bilious vomiting and abdominal distension, followed by abdominal wall erythema, rigidity and peritonitis if left untreated. [clinicaladvisor.com]
Complicated MI presents more dramatically at birth with severe abdominal distension, sometimes accompanied by abdominal wall erythema and edema. Abdominal distension may be severe enough to cause respiratory distress. [intranet.tdmu.edu.ua]
Musculoskeletal
- Leg Pain
When the intestine is obstructed by Ladd's bands or when the blood supply is twisted, symptoms may include: Vomiting bile (greenish-yellow digestive fluid) Drawing up the legs Pain in the abdomen (belly) Abdominal distention (swelling) Rapid heart rate [chp.edu]
Deficiency of vitamin D in children can result in rickets, a condition characterized by bow deformities of the legs, pain in the legs and progressive softening of the bone structure. [rarediseases.org]
Psychiatrical
- Withdrawn
Both malformations take place in the same period of embryologic development, at 12 th g.w., and it is possible to speculate that the same developmental hit affects both the withdrawn of the yolk sac into the cavity following the midgut, and the later [omicsonline.org]
The PN was withdrawn on the postoperative day 10 and she was discharged on day 11. At last follow-up, 4 weeks after discharge, the patient had gained 900 g and she fed without problems. [e-ce.org]
After the successful myotomy, the instruments are withdrawn under direct vision and the pneumoperitoneum is evacuated. The nasogastric tube is also removed after completing the surgery. [intranet.tdmu.edu.ua]
Neurologic
- Difficulty Concentrating
In severe cases, it can cause weakness, fatigue, difficulty concentrating and short of breath upon exertion (dyspnea). [rarediseases.org]
Workup
The diagnostic preoperative workup should include a plain abdominal radiograph, upper and/or lower gastrointestinal contrast studies, and ultrasonography (1). [journals.lww.com]
A thorough workup was notable for congenital bowel malrotation; symptoms were relieved following surgical treatment. [westjem.com]
When he lost his medical insurance, he was discharged and advised to have further workup performed elsewhere. [hindawi.com]
Ghassemian AJ, MacDonald KG, Cunningham PG, Swanson M, Brown BM, et al. (1997) The workup for bariatric surgery does not require a routine upper gastrointestinal series. Obes Surg 7: 16-18. [omicsonline.org]
They may also be encountered incidentally at the time of workup for other diseases. [liebertpub.com]
X-Ray
- Colonic Stricture
CMV infection should always be excluded in full-term infants presenting with colonic stricture or malrotation. [ijponline.biomedcentral.com]
Tuberculosis-associated left colon stricture has also been reported. [13] Patients who undergo bowel resection for any reason and have a segment of intestine removed and joined by anastomosis may develop stricture at the anastomotic site as a consequence [emedicine.medscape.com]
Serum
- Hypoglycemia
Also vital are maintenance of body temperature, prevention of hypoglycemia with IV 10% dextrose and electrolytes, and prevention or treatment of acidosis and infections so that the infant is in optimal condition for surgery. [merckmanuals.com]
- Hyponatremia
Although routine laboratory studies are not specific for a diagnosis of small bowel obstruction, these studies help assess the presence and severity of hypovolemia, leukocytosis, and metabolic abnormalities (hyponatremia, hypokalemia). [uptodate.com]
Treatment
At present, there is no consensus on the treatment of abdominal cocoon disease. [bmcsurg.biomedcentral.com]
Initial resuscitation (See “Treatment” of sigmoid volvulus above.) [amboss.com]
Treatment [ edit ] Early treatment includes removing fluids from the stomach via a nasogastric tube, and providing fluids intravenously. [1] The definitive treatment for duodenal atresia is surgery (duodenoduodenostomy), which may be performed openly [en.wikipedia.org]
Our renowned surgeons and faculty continue to push the frontiers of surgical science through innovative treatment and leading-edge research, changing lives and the future of surgical treatment, each and every day. [medicine.yale.edu]
Babies with birth defects often need special care and treatments. The treatments may include surgery, medicines, assistive devices, and therapies. [icdlist.com]
Prognosis
Prognosis Survival is primarily dependent on gestation at delivery and the presence of other anomalies. [sonoworld.com]
Abstract With advances in surgery and the use of the antibiotics, the prognosis of congenital obstruction of the duodenum has become much more favorable. [pubs.rsna.org]
Prognosis Survival in MMIHS seems to have improved, thanks to more specialized care, innovations in parenteral nutrition, and introduction of multivisceral transplantation. [orpha.net]
This classification generally guides prognosis and therapy (see the image below). Classification of jejunoileal atresias. [emedicine.medscape.com]
It is key to improve the prognosis and reduce complications. Keywords: acute abdomen, intestinal malrotation, volvulus. Get Full Access Gale offers a variety of resources for education, lifelong learning, and academic research. [go.gale.com]
Etiology
Keywords: Adult, Case Report, Duodenum, abnormalities,Female, Human, Intestinal Obstruction, etiology,Pregnancy, Pregnancy Complications, etiology, How to cite this article: Hardikar J V. Malrotation of the gut manifested during pregnancy. [jpgmonline.com]
Risk factors — Factors that increase the risk for the most common etiologies of small bowel obstruction are given in the table ( table 1 ). (See 'Specific etiologies' below.) [uptodate.com]
Etiology Etiology of MMIHS is unknown but various hypotheses have been proposed to describe the pathogenesis including genetic, neurogenic, myogenic and hormonal origin. The 15q11.2 region might be associated with MMIHS. [orpha.net]
Etiology Duodenal atresia is a sporadic abnormality, although, in some cases, there is an autosomal recessive pattern of inheritance. [sonoworld.com]
Etiology Multiple theories regarding the etiology of jejunoileal atresia have been studied in many animal models (eg, puppies, ewes, rabbits, and chick embryos). [22, 23, 24, 25, 26] Murine studies suggest that some forms of atresia may be hereditary [emedicine.medscape.com]
Epidemiology
Epidemiology References: [1] [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]
Summary Epidemiology MMIHS prevalence is unknown but the disease has been reported in 230 patients, of which 71% are females. Clinical description Enlarged and nonobstructed bladder is the first manifestation of MMIHS and can be detected prenatally. [orpha.net]
[…] intravenously. [1] The definitive treatment for duodenal atresia is surgery (duodenoduodenostomy), which may be performed openly or laparoscopically. [6] The surgery is not urgent. [1] The initial repair has a 5 percent morbidity and mortality rate. [2] Epidemiology [en.wikipedia.org]
Epidemiology In the United States, for example, asymptomatic GI malrotation occurs with an incidence somewhere between 1 in 200 to 1 in 500. [news-medical.net]
(See "Epidemiology, clinical features, and types of small bowel neoplasms" .) [uptodate.com]
Pathophysiology
Lower Digestive Tract, 2nd Edition, part 2 in the 3-book Digestive System volume, covers the small bowel and colon, and provides a concise and highly visual approach from normal anatomy and physiology through pathophysiology, diagnostics and treatment [books.google.com]
Risk factors for the development of a volvulus References: [2] [3] [4] [5] [12] [13] [14] [15] [16] Pathophysiology Sigmoid volvulus Initial resuscitation : IV fluids ; acid-base and electrolyte imbalance correction ; n il per oral ; placement of a nasogastric [amboss.com]
Pathophysiology In the scenario of little or no rotation, the duodenum does not completely rotate and cross posterior to the SMA to allow creation of a normal ligament of Treitz on the left side of the abdomen. [online.epocrates.com]
Pathophysiology GI malrotation, has been recently suggested to result from genetic mutations. The gene implicated is the fork head transcription factor (FOXF1) gene. [news-medical.net]
Pathophysiology Malrotation refers to impaired rotational process of the midgut as it returns from outside of the abdominal cavity (extracoelomic phase of development) to the abdominal cavity. [pedclerk.bsd.uchicago.edu]
Prevention
This study also shows that more awareness about intestinal malrotation is needed to proactively treat the disorder and prevent its potentially life-threatening complications. [newsroom.clevelandclinic.org]
[…] dehydration and antibiotics to prevent infection. [luriechildrens.org]
Also vital are maintenance of body temperature, prevention of hypoglycemia with IV 10% dextrose and electrolytes, and prevention or treatment of acidosis and infections so that the infant is in optimal condition for surgery. [merckmanuals.com]
Prevention There are no current guidelines to prevent volvulus. [azkrmc.com]
Children may be started on IV (intravenous) fluids to prevent dehydration and antibiotics to prevent infection. [stanfordchildrens.org]