Asymptomatic malrotation: Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review

Kathleen Graziano, Saleem Islam, Roshni Dasgupta, Monica E. Lopez, Mary Austin, Li Ern Chen, Adam Goldin, Cynthia D. Downard, Elizabeth Renaud, Fizan Abdullah

Research output: Contribution to journalArticle

Abstract

Objective Patients with malrotation, or an intestinal rotation abnormality (IRA), can experience serious adverse events. Increasingly, asymptomatic patients are being diagnosed with malrotation incidentally. Patients with symptomatic malrotation require surgery in an urgent or semiurgent manner to address their symptoms. The treatment of asymptomatic or incidentally discovered malrotation remains controversial. Methods Data were compiled from a broad search of Medline, Cochrane, Embase and Web of Science from January 1980 through January 2013 for five questions regarding asymptomatic malrotation. Results There is minimal evidence to support screening asymptomatic patients. Consideration may be given to operate on asymptomatic patients who are younger in age, while observation may be appropriate in the older patient. If reliably diagnosed, atypical malrotation with a broad-based mesentery and malposition of the duodenum can be observed. Regarding diagnostic imaging, the standard of care for diagnosis remains the upper gastrointestinal contrast study (UGI), ultrasound may be useful for screening. A laparoscopic approach is safe for diagnosis and treatment of rotational abnormalities. Laparoscopy can aid in determining whether a patient has true malrotation with a narrow mesenteric stalk, has nonrotation and minimal risk for volvulus, or has atypical anatomy with malposition of the duodenum. It is reasonable to delay Ladd procedures until after palliation on patients with severe congenital heart disease. Observation can be considered with extensive education for family and caregivers and close clinical follow-up. Conclusions There is a lack of quality data to guide the management of patients with asymptomatic malrotation. Multicenter and prospective data should be collected to better assess the risk profile for this complex group of patients. A multidisciplinary approach involving surgery, cardiology, critical care and the patient's caregivers can help guide a watchful waiting management plan in individual cases.

Original languageEnglish (US)
Pages (from-to)1783-1790
Number of pages8
JournalJournal of Pediatric Surgery
Volume50
Issue number10
DOIs
StatePublished - Oct 1 2015

Fingerprint

Evidence-Based Practice
Advisory Committees
Pediatrics
Duodenum
Caregivers
Observation
Watchful Waiting
Intestinal Volvulus
Mesentery
Diagnostic Imaging
Critical Care
Standard of Care
Cardiology
Laparoscopy
Heart Diseases
Anatomy
Education

Keywords

  • Asymptomatic malrotation
  • Atypical malrotation
  • Diagnosis and surgical management of asymptomatic malrotation
  • Heterotaxy syndrome
  • Intestinal rotation abnormalities
  • Key words Malrotation

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Asymptomatic malrotation : Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review. / Graziano, Kathleen; Islam, Saleem; Dasgupta, Roshni; Lopez, Monica E.; Austin, Mary; Chen, Li Ern; Goldin, Adam; Downard, Cynthia D.; Renaud, Elizabeth; Abdullah, Fizan.

In: Journal of Pediatric Surgery, Vol. 50, No. 10, 01.10.2015, p. 1783-1790.

Research output: Contribution to journalArticle

Graziano, Kathleen ; Islam, Saleem ; Dasgupta, Roshni ; Lopez, Monica E. ; Austin, Mary ; Chen, Li Ern ; Goldin, Adam ; Downard, Cynthia D. ; Renaud, Elizabeth ; Abdullah, Fizan. / Asymptomatic malrotation : Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review. In: Journal of Pediatric Surgery. 2015 ; Vol. 50, No. 10. pp. 1783-1790.
@article{030d5363010f477ca3e3965a442b9edf,
title = "Asymptomatic malrotation: Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review",
abstract = "Objective Patients with malrotation, or an intestinal rotation abnormality (IRA), can experience serious adverse events. Increasingly, asymptomatic patients are being diagnosed with malrotation incidentally. Patients with symptomatic malrotation require surgery in an urgent or semiurgent manner to address their symptoms. The treatment of asymptomatic or incidentally discovered malrotation remains controversial. Methods Data were compiled from a broad search of Medline, Cochrane, Embase and Web of Science from January 1980 through January 2013 for five questions regarding asymptomatic malrotation. Results There is minimal evidence to support screening asymptomatic patients. Consideration may be given to operate on asymptomatic patients who are younger in age, while observation may be appropriate in the older patient. If reliably diagnosed, atypical malrotation with a broad-based mesentery and malposition of the duodenum can be observed. Regarding diagnostic imaging, the standard of care for diagnosis remains the upper gastrointestinal contrast study (UGI), ultrasound may be useful for screening. A laparoscopic approach is safe for diagnosis and treatment of rotational abnormalities. Laparoscopy can aid in determining whether a patient has true malrotation with a narrow mesenteric stalk, has nonrotation and minimal risk for volvulus, or has atypical anatomy with malposition of the duodenum. It is reasonable to delay Ladd procedures until after palliation on patients with severe congenital heart disease. Observation can be considered with extensive education for family and caregivers and close clinical follow-up. Conclusions There is a lack of quality data to guide the management of patients with asymptomatic malrotation. Multicenter and prospective data should be collected to better assess the risk profile for this complex group of patients. A multidisciplinary approach involving surgery, cardiology, critical care and the patient's caregivers can help guide a watchful waiting management plan in individual cases.",
keywords = "Asymptomatic malrotation, Atypical malrotation, Diagnosis and surgical management of asymptomatic malrotation, Heterotaxy syndrome, Intestinal rotation abnormalities, Key words Malrotation",
author = "Kathleen Graziano and Saleem Islam and Roshni Dasgupta and Lopez, {Monica E.} and Mary Austin and Chen, {Li Ern} and Adam Goldin and Downard, {Cynthia D.} and Elizabeth Renaud and Fizan Abdullah",
year = "2015",
month = "10",
day = "1",
doi = "10.1016/j.jpedsurg.2015.06.019",
language = "English (US)",
volume = "50",
pages = "1783--1790",
journal = "Journal of Pediatric Surgery",
issn = "0022-3468",
publisher = "W.B. Saunders Ltd",
number = "10",

}

TY - JOUR

T1 - Asymptomatic malrotation

T2 - Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review

AU - Graziano, Kathleen

AU - Islam, Saleem

AU - Dasgupta, Roshni

AU - Lopez, Monica E.

AU - Austin, Mary

AU - Chen, Li Ern

AU - Goldin, Adam

AU - Downard, Cynthia D.

AU - Renaud, Elizabeth

AU - Abdullah, Fizan

PY - 2015/10/1

Y1 - 2015/10/1

N2 - Objective Patients with malrotation, or an intestinal rotation abnormality (IRA), can experience serious adverse events. Increasingly, asymptomatic patients are being diagnosed with malrotation incidentally. Patients with symptomatic malrotation require surgery in an urgent or semiurgent manner to address their symptoms. The treatment of asymptomatic or incidentally discovered malrotation remains controversial. Methods Data were compiled from a broad search of Medline, Cochrane, Embase and Web of Science from January 1980 through January 2013 for five questions regarding asymptomatic malrotation. Results There is minimal evidence to support screening asymptomatic patients. Consideration may be given to operate on asymptomatic patients who are younger in age, while observation may be appropriate in the older patient. If reliably diagnosed, atypical malrotation with a broad-based mesentery and malposition of the duodenum can be observed. Regarding diagnostic imaging, the standard of care for diagnosis remains the upper gastrointestinal contrast study (UGI), ultrasound may be useful for screening. A laparoscopic approach is safe for diagnosis and treatment of rotational abnormalities. Laparoscopy can aid in determining whether a patient has true malrotation with a narrow mesenteric stalk, has nonrotation and minimal risk for volvulus, or has atypical anatomy with malposition of the duodenum. It is reasonable to delay Ladd procedures until after palliation on patients with severe congenital heart disease. Observation can be considered with extensive education for family and caregivers and close clinical follow-up. Conclusions There is a lack of quality data to guide the management of patients with asymptomatic malrotation. Multicenter and prospective data should be collected to better assess the risk profile for this complex group of patients. A multidisciplinary approach involving surgery, cardiology, critical care and the patient's caregivers can help guide a watchful waiting management plan in individual cases.

AB - Objective Patients with malrotation, or an intestinal rotation abnormality (IRA), can experience serious adverse events. Increasingly, asymptomatic patients are being diagnosed with malrotation incidentally. Patients with symptomatic malrotation require surgery in an urgent or semiurgent manner to address their symptoms. The treatment of asymptomatic or incidentally discovered malrotation remains controversial. Methods Data were compiled from a broad search of Medline, Cochrane, Embase and Web of Science from January 1980 through January 2013 for five questions regarding asymptomatic malrotation. Results There is minimal evidence to support screening asymptomatic patients. Consideration may be given to operate on asymptomatic patients who are younger in age, while observation may be appropriate in the older patient. If reliably diagnosed, atypical malrotation with a broad-based mesentery and malposition of the duodenum can be observed. Regarding diagnostic imaging, the standard of care for diagnosis remains the upper gastrointestinal contrast study (UGI), ultrasound may be useful for screening. A laparoscopic approach is safe for diagnosis and treatment of rotational abnormalities. Laparoscopy can aid in determining whether a patient has true malrotation with a narrow mesenteric stalk, has nonrotation and minimal risk for volvulus, or has atypical anatomy with malposition of the duodenum. It is reasonable to delay Ladd procedures until after palliation on patients with severe congenital heart disease. Observation can be considered with extensive education for family and caregivers and close clinical follow-up. Conclusions There is a lack of quality data to guide the management of patients with asymptomatic malrotation. Multicenter and prospective data should be collected to better assess the risk profile for this complex group of patients. A multidisciplinary approach involving surgery, cardiology, critical care and the patient's caregivers can help guide a watchful waiting management plan in individual cases.

KW - Asymptomatic malrotation

KW - Atypical malrotation

KW - Diagnosis and surgical management of asymptomatic malrotation

KW - Heterotaxy syndrome

KW - Intestinal rotation abnormalities

KW - Key words Malrotation

UR - http://www.scopus.com/inward/record.url?scp=84956805117&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84956805117&partnerID=8YFLogxK

U2 - 10.1016/j.jpedsurg.2015.06.019

DO - 10.1016/j.jpedsurg.2015.06.019

M3 - Article

C2 - 26205079

AN - SCOPUS:84956805117

VL - 50

SP - 1783

EP - 1790

JO - Journal of Pediatric Surgery

JF - Journal of Pediatric Surgery

SN - 0022-3468

IS - 10

ER -