Malrotation

Classification according to ICD-10
Q43.3	Congenital malformations that affect bowel fixation
ICD-10 online (WHO version 2019)

As Malrotation ( latin malus 'bad' , harmful ',' mean 'and Rotatio , rotation' ) is a disorder of the rotation of the small and large intestine in the embryonic development , respectively.

This positional anomaly can go completely unnoticed. B. lead to (intermittent) compression of the duodenum ( duodenal stenosis , superior mesenteric artery syndrome ).

Occurrence

The frequency is given as 1: 500 live births, twice as common in boys.

In the context of syndromes:

• Apple peel syndrome
• De Grouchy Syndrome
• Familial intestinal malrotation
• Ivemark symptom complex
• Kapur-Toriello syndrome , synonym : cleft lip and palate - cardiopathy - intestinal malrotation
• Martínez-Frías syndrome
• McKusick-Kaufman syndrome
• Mcpherson-Clemens syndrome , synonym: duodenal and extrahepatic biliary atresia - hypoplastic pancreas - intestinal malrotation
• Megacystis-microcolon-intestinal hypoperistalsis syndrome
• Patau syndrome
• Prune Belly Syndrome

In about half there is an association with other malformations:

• Duodenal atresia , duodenal stenosis
• congenital diaphragmatic hernia
• Gastroschisis , omphalocele
• Heterotaxy
• Choanal atresia

Classification

Forms according to Grob:

• Nonrotation = 90 ° rotation (failure of the 2nd and 3rd rotation)
• Malrotation I = 180 ° rotation (failure of the 3rd rotation)
• Malrotation II = inverse (lat. Inversio , conversion ' ,' reversal '/' reversible ',' reversed ') 2nd rotation with regular or misdirected 3rd rotation

Symptoms

Acute or recurring abdominal pain in toddlers and children with beginning or incomplete intestinal obstruction due to volvulus (lat. Volvere , 'to turn' ) of the intestine.

Sometimes symptoms only appear in adults.

therapy

Surgical solution of disruptive ligaments, adhesions and fixation Fixation of the intestine in the “Ladd procedure”.

literature

• M. Bettex, N. Genton, M. Stockmann (Eds.): Pediatric Surgery. Diagnostics, indication, therapy, prognosis. 2nd Edition. Thieme, Stuttgart 1982, ISBN 3-13-338102-4 .
• W. Schuster, D. Färber (Ed.): Children's radiology. Imaging diagnostics. Springer, Berlin et al. 1996, ISBN 3-540-60224-0 .

Web links

• emedicine

Individual evidence

1. ↑ S. Aslanabadi, A. Ghalehgolab-Behbahan, M. Jamshidi, P. Veisi, S. Zarrintan: Intestinal malrotations. A review and report of thirty cases. In: Folia Morphol. Volume 66, Warsaw 2007, pp. 277-282.
2. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
3. ↑ Familial intestinal malrotation. In: Orphanet (Rare Disease Database).
4. ↑ Kapur-Toriello syndrome. In: Orphanet (Rare Disease Database).
5. ↑ Cleft lip and palate - malrotation - cardiopathy. In: Orphanet (Rare Disease Database).
6. ↑ Radiopaedia
7. ↑ D. Perez, T. Frick, A. Schierz: Intestinal malrotation with small intestinal ileus in adults. In: Visceral Surgery. Volume 40, 2005, p. 141, doi : 10.1055 / s-2005-836398 .
8. ^ KD Bass, SS Rothenberg, JH Chang: Laparoscopic Ladd's procedure in infants with malrotation. In: Journal of Pediatric Surgery. Volume 33, No. 2, 1998, pp. 279-281 ( doi: 10.1016 / S0022-3468 (98) 90447-X , PMID 9498402 ).

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !