Presentation
The purpose of this paper is to present 2 cases of extrinsic congenital obstruction of the duodenum, to discuss the diagnostic problems involved, and to emphasize the possibilities of early roentgen diagnosis of this condition. [pubs.rsna.org]
The clinical presentation, surgical management and review of literature are discussed. [ncbi.nlm.nih.gov]
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]
If a transesophageal fistula is present to the distal esophagus, gas will be present in the bowel. [quizlet.com]
The frequency of midgut volvulus among infants presenting in the neonatal period ranges from 45% to 80%. [annsaudimed.net]
Entire Body System
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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]
In a small number of cases, Hirschsprung’s disease is associated with trisomy 21. Diagnosis The aganglionic segment is unable to transmit a peristaltic wave, and therefore meconium accumulates and causes dilatation of the lumen of the bowel. [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]
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Severe Pain
Volvulus causes severe pain and progressive injury to the intestinal wall, with accumulation of gas and fluid in the portion of the bowel obstructed. [2] Ultimately, this can result in necrosis of the affected intestinal wall, acidosis, and death. [shortbowelfoundation.org]
Posterior gastric ulcers may involve the pancreas and the splenic artery, resulting in severe pain and bleeding. The gastro-esophageal orifice is the most fixed part of the stomach. The fundus fits into the curve of the left dome of the diaphragm. [dartmouth.edu]
A sudden onset of severe pain may suggest acute intestinal perforation. (See "Overview of gastrointestinal tract perforation" .) [uptodate.com]
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Feeding Difficulties
Most congenital GI anomalies result in some type of intestinal obstruction, frequently manifesting with feeding difficulties, distention, and emesis at birth or within 1 or 2 days. [merckmanuals.com]
difficulties Wide mouth Spasticity Polydactyly Long face Ventriculomegaly Hypospadias Hernia Heterogeneous Dystonia Tracheoesophageal fistula Renal insufficiency Hydrocephalus Anteverted nares Intellectual disability, severe Abnormality of the kidney [mendelian.co]
Respiratoric
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Pleural Effusion
Congenital nonchylous pleural effusion with Down's syndrome. J Med Genet 1987;24:567-71. Lippincott-Raven Publishers [journals.lww.com]
Gastrointestinal
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Regurgitation
She regurgitated each feeding and soon vomited bile-stained material. The family history was not contributory. Physical examination and laboratory studies were not remarkable. [pubs.rsna.org]
The mother complained that the baby had recurrent vomiting/regurgitation of breast milk from the fifth day of birth, with multiple episodes of diarrhea. On examination, the child appeared malnourished and weighed 2.2 kg. [ijri.org]
Broad forehead Pulmonary hypoplasia Vesicoureteral reflux Flat face Auricular pit Sagittal craniosynostosis High, narrow palate Diastasis recti Metaphyseal dysplasia Aortic aneurysm Hiatus hernia Narrow palate Bicuspid aortic valve Heterotopia Mitral regurgitation [mendelian.co]
The sphincters are under neural and hormonal control, and they help to prevent regurgitation of contents. [dartmouth.edu]
There is anorexia instead of voracious appetite; the vomiting is regurgitant rather than projectily, and so frequently is peristalsis normally visible. [intranet.tdmu.edu.ua]
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Severe Abdominal Pain
a 13-year-old white boy, was in normal health until three weeks prior to admission to the University Hospital, at which time severe abdominal pain developed, followed by vomiting. [pubs.rsna.org]
The son of a farmer started to complain of severe abdominal pain for the last 3 months. His pains used to commence post meals. [parashospitals.com]
This is his first presentation for such severe abdominal pain. On physical exam, he was afebrile and hemodynamically stable. His abdominal exam revealed a distended abdomen with significant voluntary guarding. [academic.oup.com]
Symptoms of volvulus include: yellow or green vomit (bilious vomiting) severe abdominal pain abdominal swelling or distention rapid heart rate rapid breathing bloody stools If your child exhibits such symptoms, you should seek immediate medical evaluation [childrenshospital.org]
Musculoskeletal
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Arthritis
[…] onset spinocerebellar ataxia Infantile recurrent chronic multifocal osteomyolitis Infantile sialic acid storage disorder Infantile spasms broad thumbs Infantile spasms Infantile spinal muscular atrophy Infantile striato thalamic degeneration Infectious arthritis [ipfs.io]
It is a commonly used anti-arthritis medicine called NSAID (nonsteroidal anti-inflammatory drug). [my.clevelandclinic.org]
Urogenital
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Enlarged Uterus
In view of the enlarged uterus and the need for retroperitoneal dissection to correct an anatomically anomalous lesion with uncertain vascularity, the surgical opinion was to try nonoperative management first. [degruyter.com]
Workup
The diagnostic preoperative workup should include a plain abdominal radiograph, upper and/or lower gastrointestinal contrast studies, and ultrasonography (1). [journals.lww.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
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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]
Pleura
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Pleural Effusion
Congenital nonchylous pleural effusion with Down's syndrome. J Med Genet 1987;24:567-71. Lippincott-Raven Publishers [journals.lww.com]
Treatment
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]
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]
Further Reading Symptoms and Diagnosis of Intestinal Malrotation Symptoms and Diagnosis of Intestinal Malrotation Treatment and Prognosis for Intestinal Malrotation [news-medical.net]
Kazil understands the important circumstances that drive the treatment with the accuracy and compassion that everyone expects from a consummate general surgeon. [floridasurgicalclinic.com]
Persistent symptoms after symptomatic treatment should raise the alarm. Plain abdominal X-ray and US-scans must be used in cases of doubt. [academic.oup.com]
Prognosis
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 is primarily dependent on gestation at delivery and the presence of other anomalies. [sonoworld.com]
This classification generally guides prognosis and therapy (see the image below). Classification of jejunoileal atresias. [emedicine.medscape.com]
These types of anomalies require a multidisciplinary approach in order to establish a prognosis and plan the perinatal management. [redalyc.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]
Etiology
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 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]
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]
DISCUSSION Malrotation remains the most common etiology of volvulus in neonates, though a rare cause in adults. It is due to an error in embryologic gut development. [academic.oup.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]
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]
[…] 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]
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]
(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]
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]
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 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
[…] dehydration and antibiotics to prevent infection. [luriechildrens.org]
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]
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]
The child may also be given intravenous (IV) fluids to help prevent dehydration and antibiotics to prevent infection. [kidshealth.org]