Malrotation in Older Children and Adults Patrick Phelan 12/3/2009

Malrotation “the condition is rare enough so that it is likely to escape the mind, and it is common enough to be important.” William Ladd 1932

Embryology Week 5: Gut herniates from abdominal wall Rotation: always CCW (looking at fetus) Duodenojejunal segment rotates 90° twice (180°) Cecocolic segment rotates 90° twice (180°) Week 10-11: Gut returns to abdominal cavity Duodenojejunal segment rotates 90° again (270°) Cecocolic segment rotates 90° again (270°)

Normal orientation of the mesentary!! Embryology 90° 90° 90° Normal orientation of the mesentary!!

Happy Blimp

Abnormalities of Rotation Nonrotation Bowel loops rotate less than 90° total Incomplete rotation Rotation occurs, but less than 270° total

Sad Blimp

Volvulus Mesenteric twisting results in transient or persistent vascular occlusion Ischemia Necrosis Perforation Sepsis

Ladd’s Procedure Detorsion of volvulus if present. Division of Ladd’s cecal bands Division of interloop bands – widen mesentery Appendectomy Relocation of the bowel

Clinical Settings We all know: What about: Infant with bilious emesis Emergent UGI (and Ladd’s procedure) What about: Adolescents/Adults with positive UGI/BE/CT w/ vague hx vomiting, abdominal pain, malabsorption Found at ex-lap for other reason Truly Incidental malrotation CT for abdominal trauma BE for colon cancer screening

Evidence Retrospective Case Series Administrative Database Data Decision Tree Modeling

Retrospective Case Series Prasil (2000): Single Institution retrospective case series Methods: all patients operated on for malrotation (as a primary condition) 1980 – 1998. Exclusion: high probability associations Incidental findings at laparotomy Comparison between those < 2 y/o and >2 y/o

Prasil 2000 Results: Table 2. Symptoms at presentation Table 1. Mode of Clinical Presentation Table 2. Symptoms at presentation

Prasil 2000 Table 3. Surgical Complications Conclusions: “Based on our review, we support the policy that regardless of age and even in asymptomatic patients, any malrotation diagnosed should have particular surgical attention.”

Malek 2005 Population based study of Administration Database Methods: KID 2000 (Kid’s Inpatient Discharge) 2.5M discharge summaries from 27 states Inclusion: Code for abnormality of intestinal rotation 1< Age <18 Ladd’s procedure component Exclusion: Discharge to short-term care facility (avoid double count) Discharge dx of post-operative small bowel obstruction

Malek 2005 Data extrapolated using US census data to predict national number of Ladd’s procedures Variables of interest Emergency admission Transfer Hospital location(urban/rural) Teaching status Dedicated Children’s hospital

Malek 2005 Results 771 cases, 219 > 1 year old

Malek 2005 75% 19% 4%

Malek 2005 25% admitted for treatment of other disease Conclusion: 8 anti-reflux 7 appendectomies 5 diaphragmatic hernias 4 trauma ex-laps Conclusion: Although less common in older children, volvulus can occur at all ages and therefore warrants surgical correction

Durkin 2008 Single Institution retrospective case series Methods: all patients seen for malrotation 2002 – 2006 24 patients – 23 underwent Ladd’s procedure >16 = adult <16 = pediatric Age Range: 10 days to 89 years

Durkin 2008 Results: No significant difference in chronicity of symptoms at presentation between children and adults Subgroup analysis of <2 y/o shows 100% of patients present acutely (50% >2 y/o) p = 0.03

Durkin 2008 Results: Practioners are better at diagnosing Malrotation in Children

Durkin 2008 Results: Re-ops: Postoperative complications are worse in Adults Re-ops: Duodenal obstruction 2/2 adhesion in 2 pts Others: ladd’s not initially performed

Malek 2005 (2) Decision analysis model Outcomes Goal: determine age at which observation is preferable to Ladd’s for ASYMPTOMATIC malrotation. Methods: Markov decision analysis with 1 year cycles for Asymptomatic malrotation patients >1 y/o Outcomes Life expectancy QALYs – projected decrease in quality of life from operative intervention

Malek 2005 (2) Age related mortality is estimated from patients with colon resection for benign cause.

Malek 2005 (2)

Malek 2005 (2) Conclusion: For truly asymptomatic adults with malrotation, observation is preferred over prophylactic Ladd’s procedure

JD 11y M Hx abd pain with N/V for years Presents with non-bilious vomiting and abdominal pain

References Durkin E. J Am Coll Surg (2008) 206: 658–663. Malek M. The American Journal of Surgery (2006) 191: 45–51 Prasil P. J Pediatr Surg (2000) 35: 756-758. Malek M. Journal of Pediatric Surgery (2005) 40, 285–289