Meconium ileus

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Classification according to ICD-10
P75 Meconium ileus in cystic fibrosis
P76 Other intestinal obstruction in the newborn
ICD-10 online (WHO version 2019)

The meconium ileus is a special form of intestinal obstruction (ileus) in newborns, usually at the level of the ileum, caused by meconium (child spank ).

The first description comes from the year 1905 by Karl Landsteiner .

Spread and cause

Cystic fibrosis is present in 80-90 % with abnormally tough, sticky meconium. About 20% of children with cystic fibrosis have a meconium ileus after birth.

In contrast, in meconium plug syndrome, the meconium is not changed.

Other, rare causes are pancreatic atresia or stenosis of the pancreatic duct .

Further, there is a meconium ileus due to a congenital, autosomal - recessive inherited lack of guanylate cyclase-C ( meconium ileus by guanylate cyclases 2C deficiency )

Classification

Depending on the presence of further changes, a distinction can be made between:

Clinical manifestations

Clinical criteria are:

  • No meconium leakage within the first 24 to 48 hours after birth
  • Signs of constipation with a distended, distended abdomen
  • Vomit
  • mostly clinical signs of cystic fibrosis ( sweat test )

As a complication Ileusatresie or stenosis, can bowel perforation , meconium peritonitis and volvulus added.

diagnosis

The diagnosis can already be made intrauterine by sonography or magnetic resonance tomography , then also immediately after birth.

The air-filled loops of the small intestine can be seen in the X-ray image (abdomen overview) when the large intestine is evacuated. The colonic enema can show the collapsed or underdeveloped colon ( microcolon ) and the level of the obstruction.

Differential diagnosis

The following are to be distinguished:

therapy

Treatment depends on the underlying condition.

To relieve (and diagnose) the first step is the enema under fluoroscopy, previously with amidotrizoic acid ("gastrografin"), today with nonionic iodine-containing contrast media, which are considered more suitable because of the risk of perforation. If meconium is emptied in this way, the intestinal passage can be re-established in multiple enemas with warm, isotonic saline solution.

Otherwise, as well as in the event of complications, the meconium must be surgically removed.

Healing prospects

The prognosis depends on the underlying disease and the complications present.

literature

  • Marcel Bettex (ed.), Max Grob (introductory), D. Berger (editing), N. Genton, M. Stockmann: Pediatric Surgery. Diagnostics, indication, therapy, prognosis. 2nd, revised edition, Thieme, Stuttgart / New York 1982, p. 7.54, ISBN 3-13-338102-4
  • NS Parikh, R. Ahlawat: Meconium Ileus. In: StatPearls [Internet], 2019, PMID 30725693 [2]
  • JH Waldhausen, M. Richards: Meconium Ileus. In: Clinics in Colon and Rectal Surgery. Volume 31, number 2, March 2018, pp. 121-126, doi: 10.1055 / s-0037-1609027 , PMID 29487495 , PMC 5825880 (free full text) (review).
  • S. Stoll-Becker, I. Reiss, G. Hüls, A. Hauch, M. Hermsteiner, L. Gortner: Neonatal ascites: Meconium ileus with perforation in cystic fibrosis. In: Clinical Pediatrics. Volume 213, Number 1, 2001 Jan-Feb, pp. 26-27, doi: 10.1055 / s-2001-11269 , PMID 11225471 .
  • R. Murshed, L. Spitz, E. Kiely, D. Drake: Meconium ileus: a ten-year review of thirty-six patients. In: European journal of pediatric surgery: official journal of the Austrian Association of Pediatric Surgery .. [et al] = Journal for Pediatric Surgery. Volume 7, Number 5, October 1997, pp. 275-277, doi: 10.1055 / s-2008-1071170 , PMID 9402484 .
  • CA Del Pin, C. Czyrko, MM Ziegler, TF Scanlin, HC Bishop: Management and survival of meconium ileus. A 30-year review. In: Annals of surgery. Volume 215, Number 2, February 1992, pp. 179-185, doi: 10.1097 / 00000658-199202000-00014 , PMID 1546905 , PMC 1242407 (free full text).

Individual evidence

  1. ↑ Willibald Pschyrembel : Clinical Dictionary , 266th, updated edition, de Gruyter, Berlin 2014, ISBN 978-3-11-033997-0, keyword "Mekoniumileus"
  2. ^ A b W. Schuster, D. Färber: Pediatric radiology: imaging diagnostics. 2nd edition 1996, Springer, ISBN 3-540-15944-4
  3. ↑ a b M. Berger, D. von Schweinitz: Mekoniumileus. In: D. von Schweinitz, B. Ure (Ed.) Pediatric Surgery. Springer, Berlin, Heidelberg 2019, print ISBN 978-3-662-58201-5 Online ISBN 978-3-662-58202-2 [1]
  4. ↑ a b 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 .
  5. ↑ K. Landsteiner: Intestinal obstruction due to thickened meconium pancreatitis. in: Zentralblatt Allgemeine Pathologie 1905; Vol. 16, pp. 903-907.
  6. ↑ a b Emedicine Intestinal Obstruction
  7. ↑ a b c d Radiopaedia
  8. ↑ CFMedicine
  9. ^ RR Gorter, A. Karimi, C. Sleeboom, CM Kneepkens, HA Heij: Clinical and genetic characteristics of meconium ileus in newborns with and without cystic fibrosis. In: Journal of Pediatric Gastroenterology and Nutrition . Volume 50, Number 5, May 2010, pp. 569-572, doi: 10.1097 / MPG.0b013e3181bb3427 , PMID 20386322 .
  10. ↑ K. Fakhoury, PR Durie, H. Levison, GJ Canny: Meconium ileus in the absence of cystic fibrosis. In: Archives of Disease in Childhood. Volume 67, number 10 Spec No, October 1992, pp. 1204-1206, doi : 10.1136 / adc.67.10_spec_no.1204 , PMID 1444561 , PMC 1590459 (free full text).
  11. ↑ Intestinal obstruction of the newborn due to guanylate cyclase 2C deficiency. In: Orphanet (Rare Disease Database).
  12. ↑ meconium ileus.  In: Online Mendelian Inheritance in Man . (English)
  13. ^ A. Tal, R. Carmi, E. Chai-Am, H. Zirkin, J. Bar-Ziv, E. Freud: Familial meconium ileus with normal sweat electrolytes. In: Clinical pediatrics. Volume 24, Number 8, August 1985, pp. 460-462, doi: 10.1177 / 000992288502400809 , PMID 4006357 .
  14. ^ A b R. R. Gorter, A. Karimi, C. Sleeboom, CMF Kneepkens, HA Heij: Clinical and Genetic Characteristics of Meconium Ileus in Newborns With and Without Cystic Fibrosis. In: Journal of Pediatric Gastroenterology and Nutrition. 2010, p. 1, doi: 10.1097 / MPG.0b013e3181bb3427 .
  15. ↑ a b c Emedicine Meconium Ileus Imaging
  16. ↑ T. Riebel, A. Würfel, A. Gasiorek-Wiens: Sonography of congenital anomalies of the gastrointestinal tract. In: Ultrasound in Medicine. Volume 12, Number 6, December 1991, pp. 283-288, doi : 10.1055 / s-2007-1005945 , PMID 1796288 .
  17. ^ PF Gunderman, LA Shea, BW Gray, BP Brown: Fetal MRI in management of complicated meconium ileus: Prenatal and surgical imaging. In: Prenatal diagnosis. Volume 38, number 9, 08 2018, pp. 685-691, doi: 10.1002 / pd.5296 , PMID 29877592 .
  18. ↑ AAFP
  19. ↑ Megacystis-microcolon-intestinal hypoperistalsis syndrome. In: Orphanet (Rare Disease Database).
  20. ↑ WE Berdon, DH Baker, WA Blanc, B. Gay, TV Santulli, C. Donovan: megacystis-micro-colon intestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiological findings in five newborn girls. In: American Journal of Roentgenology . Volume 126, Number 5, May 1976, pp. 957-964, doi: 10.2214 / ajr.126.5.957 , PMID 178239 .
  21. ^ A b FHA Prüfer, K. Maurer, G. Schweigmann, I. Gaßner: Management of delayed meconium discharge in premature and newborn babies. In: Ultrasound in Medicine - European Journal of Ultrasound. 27, 2006, doi: 10.1055 / s-2006-953984 .
  22. ↑ M. Boczar, E. Sawicka, K. Zybert: Meconium ileus in newborns with cystic fibrosis - results of treatment in the group of patients operated on in the years 2000-2014. In: Developmental period medicine. Volume 19, Number 1, 2015 Jan-Mar, pp. 32-40, PMID 26003068 .
  23. ↑ M. Sathe, R. Houwen: Meconium ileus in Cystic Fibrosis. In: Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society. Volume 16 Suppl 2, November 2017, pp. S32-S39, doi: 10.1016 / j.jcf.2017.06.007 , PMID 28986020 (review).