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of or relating to or inside the intestines; "intestinal disease" (同)enteric, enteral

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/12/23 06:40:05」(JST)

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Intestinal malrotation is a congenital anomaly of rotation of the midgut (embryologically, the gut undergoes a complex rotation outside the abdomen). As a result:

the small bowel is found predominantly on the right side of the abdomen
the cecum is displaced (from its usual position in the right lower quadrant) into the epigastrium - right hypochondrium
the ligament of Treitz is displaced inferiorly and rightward
fibrous bands (of Ladd) course over the vertical portion of the duodenum (DII), causing intestinal obstruction.
the small intestine has an unusually narrow base, and therefore the midgut is prone to volvulus (a twisting that can obstruct the mesenteric blood vessels and cause intestinal ischemia).

Associated conditions

This can lead to a number of disease manifestations such as:

Acute midgut volvulus
Chronic midgut volvulus
Acute duodenal obstruction
Chronic duodenal obstruction
Internal herniation
Superior mesenteric artery syndrome

Causes

The exact causes are not known. It is not associated with a particular gene, but there is some evidence of recurrence in families.^[1]

Presentation

Patients (often infants) present acutely with midgut volvulus, manifested by bilious vomiting, crampy abdominal pain, abdominal distention, and the passage of blood and mucus in their stools. Patients with chronic, uncorrected malrotation can have recurrent abdominal pain and vomiting.

Malrotation can also be entirely asymptomatic.

Diagnosis

With acutely ill patients, consider emergency surgery laparotomy if there is a high index of suspicion.

Plain radiography may demonstrate signs of duodenal obstruction with dilatation of the proximal duodenum and stomach but it is often non-specific. Upper gastrointestinal series is the modality of choice for the evaluation of malrotation as it will show an abnormal position of the duodeno-jejunal flexure (ligament of Treitz). In cases of malrotation complicated with volvulus, it demonstrates a corkscrew appearance of the distal duodenum and jejunum. In cases of obstructing Ladd bands, it will reveal a duodenal obstruction.

In equivocal cases, contrast enema, may be helpful by showing the caecum at an abnormal location.

It is usually discovered near birth, but in some cases is not discovered until adulthood.^[2] In adults, the "whirlpool sign" of the superior mesenteric artery can be useful in identifying malrotation.^[3]

Treatment

Treatment is possible and these are the steps taken: Resuscitate the patient with fluids to stabilize them before surgically

correcting the malrotation (counterclockwise rotation of the bowel)
cutting the fibrous bands over the duodenum.
widening the mesenteric pedicle by separation of the duodenum and cecum

With this condition the appendix is often on the wrong side of the body and therefore removed as a precautionary measure during the surgical procedure.

One surgical technique is known as "Ladd's procedure", after Dr. William Ladd.^[4]^[5]

Long term research on the Ladd procedure shows that even after the procedure, patients are susceptible to have complaints and might need further surgery. ^[6]

References

^ Stalker HJ, Chitayat D (1992). "Familial intestinal malrotation with midgut volvulus and facial anomalies: a disorder involving a gene controlling the normal gut rotation?". Am. J. Med. Genet. 44 (1): 46–7. doi:10.1002/ajmg.1320440111. PMID 1519649.
^ Dietz DW, Walsh RM, Grundfest-Broniatowski S, Lavery IC, Fazio VW, Vogt DP (2002). "Intestinal malrotation: a rare but important cause of bowel obstruction in adults". Dis. Colon Rectum 45 (10): 1381–6. doi:10.1007/s10350-004-6429-0. PMID 12394439.
^ Yeh WC, Wang HP, Chen C, Wang HH, Wu MS, Lin JT (1999). "Preoperative sonographic diagnosis of midgut malrotation with volvulus in adults: the "whirlpool" sign". Journal of Clinical Ultrasound 27 (5): 279–83. doi:10.1002/(SICI)1097-0096(199906)27:5<279::AID-JCU8>3.0.CO;2-G. PMID 10355892.
^ Ladd WE (1936). "Surgical Diseases of the Alimentary Tract in Infants". N Engl J Med 215: 705–8. doi:10.1056/NEJM193610152151604.
^ Bass KD, Rothenberg SS, Chang JH (1998). "Laparoscopic Ladd's procedure in infants with malrotation". J. Pediatr. Surg. 33 (2): 279–81. doi:10.1016/S0022-3468(98)90447-X. PMID 9498402.
^ Murphy FL, Sparnon AL (2006-04-01). "Long-term complications following intestinal malrotation and the Ladd’s procedure: a 15 year review". Pediatric Surgery International 22 (4): 326–329. doi:10.1007/s00383-006-1653-4.
^ Emanuwa; et al. (2011). "Midgut malrotation first presenting as acute bowel obstruction in adulthood: a case report and literature review" (PDF). World Journal of Emergency Surgery 6 (22). doi:10.1186/1749-7922-6-22.

External links

Macrorad Teleradiology Intestinal Malrotation of Adults

UpToDate Contents

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1. 腸回転異常 intestinal malrotation
2. 6ヵ月以下の乳幼児における体重減少の評価 evaluation of weight loss in infants six months of age and younger
3. 内臓逆位（心耳の構造異性）のマネージメントおよび転帰 management and outcome of heterotaxy isomerism of the atrial appendages
4. 消化管の発達に関する概要 overview of the development of the gastrointestinal tract
5. 輪状膵 annular pancreas

English Journal

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

Coe TM1, Chang DC2, Sicklick JK3.
American journal of surgery.Am J Surg.2015 Aug;210(2):201-210.e2. doi: 10.1016/j.amjsurg.2014.12.048. Epub 2015 Apr 30.
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
PMID 26002189

Possible Genetic Origin of Limb-Body Wall Complex.

Gajzer DC1, Hirzel AC, Saigal G, Rojas CP, Rodriguez MM.
Fetal and pediatric pathology.Fetal Pediatr Pathol.2015 Jun 25. [Epub ahead of print]
Limb body wall complex (LBWC) is characterized by multiple severe congenital malformations including an abdominal and/or thoracic wall defect covered by amnion, a short or absent umbilical cord with the placenta almost attached to the anterior fetal wall, intestinal malrotation, scoliosis, and lower
PMID 26111189

Intestinal malrotation in Rubinstein-Taybi syndrome.

Stevens CA1.
American journal of medical genetics. Part A.Am J Med Genet A.2015 Jun 19. doi: 10.1002/ajmg.a.37167. [Epub ahead of print]
Rubinstein-Taybi syndrome (RSTS) is a multiple congenital anomaly syndrome which may include malformations of the central nervous system, heart, genitourinary tract, and other organs. However, intestinal malrotation has not been previously known to be associated with RSTS. This report documents six
PMID 26097216

Japanese Journal

症例中腸軸捻転を合併した新生児腸回転異常症の術前診断に超音波カラードプラ法が有用であった4症例

池嶋由加里,久野克也,森沢猛 [他]
臨床病理 : 日本臨床検査医学会誌 62(3), 231-234, 2014-03
NAID 40020057032

特発性気腹症，縦隔気腫，皮下気腫を呈し，術後治療に難渋したCornelia de Lange syndromeの1症例

楯川幸弘
日本腹部救急医学会雑誌 34(1), 121-125, 2014
腹腔内遊離ガスを認めるが，明らかな消化管穿孔を有しないもの，または原因の不明なものは特発性気腹症と報告されている。症例は，Cornelia de Lange syndromeの13歳，男児。嘔気，下痢，熱発により外来を受診した。感染性腸炎による腸閉塞と診断し入院した。入院後，腹部膨満が著明になりCT を施行し，腹腔内遊離ガス，縦隔気腫，皮下気種を認めた。穿孔性腹膜炎を疑い緊急手術を施行し，膿性腹水 …
NAID 130004679043

Acute Appendicitis with Intestinal Malrotation: The Usefulness of Coronal Computed Tomography

Sonomura Tetsuo,Koyama Takao,Ishii Seigo,Takeuchi Taizo,Sanda Hiroki,Nakata Kouhei,Nakai Motoki,Minamiguchi Hiroki,Kishi Kazushi,Sato Morio
Internal Medicine 53(14), 1511-1513, 2014
… We herein present a rare case of acute appendicitis with intestinal malrotation. … Coronal images of contrast-enhanced computed tomography (CT) revealed the small intestine on the right side and the large intestine on the left side, thus indicating intestinal malrotation (non-rotation type). …
NAID 130004466393