INTESTINAL MALROTATION

14th January 2018

Dr. Maher Najm

IN OLDER CHILDREN: WHAT TO LOOK FOR?

DISCLOSURE

I do not have any relevant financial relationship with commercial interest to disclose.

Learning Objectives

Identify the features in clinical presentation that suggest malrotation in older children

Introduction

Malrotation

•Normalanatomy

Narrowbaseattachmentofsmallbowel

• Malrotation

Riskofvolvulus

What is known about malrotation?

1923: First description by Dott

1936: William Ladd wrote the classic article on the treatment

Most of literature on this condition focuses on neonates and infants

90% of patients with malrotation are diagnosed within the 1st year of life , 80% of these were neonates

Autopsy studies indicate that the incidence is 0.2-1% of population

The classical presentation is a sudden onset of bilious vomiting and abdominal distension

What is new ?

Age at Diagnosis

Nehra and Goldstein, Surgery 2011

0102030

Age at Diagnosis

31%

21%

48% Infants(18y)

Male: Female Ratio

0

0.5

1

1.5

2

Infants Children Adults

1.9

10.78

1 1 1

Male Female

Duration of Symptoms

0

20

40

60

80

Total 0-1year 1-18years >18years

32

64

1121

10 11 15 6

24 2515

2828

0

59% 32

Hours/days Weeks Months Years

Asymptomatic Patients

0% 20% 40% 60% 80%

100%

Infants Children AdultsAsymptomatic 15 5 17Symptomatic 85 95 83

15% 5% 17%

Clinical Presentations

0 20 40 60 80

Pain

Vomiting

Nausea

Diarrhea

Bloating

Failuretothrive

GIbleeding

Constipation

Percentageofpatients(%)

>18years

1-18years

Clinical Presentations

ClassicalpresentationRecurrentcolickyabdominalpain

NonbiliousvomitingFailuretothrive0%

20% 40% 60% 80%

100%

1-2years 2-18years

100%

25%

0%

62%

0%

38%

0%

33%

Clinical Presentations

9.5%

91.5% Percentageofmalrotationdiagnosedbydiagnosticlaparoscopyof73patients(7-12years)presentedwithacuteabdomen

Less Common and Rare Presentations

Gastroesophageal reflux Malabsorption Obstructive jaundice Recurrent idiopathic pancreatitisprotein-losing enteropathy

Incidence of Volvulus

0%

50%

100%

Infants Children Adults

63 7288

37% 22% 12%

Novolvulus Volvulus

MALROTATIONIS A TIMED BOMB LYING WITHIN

Take Home Message Intestinal malrotation can occur in patients of any age.

In contrast with traditional teaching, up to 70% of these patients may present after infancy.

An increased awareness of this entity and its varied presentation at different ages may reduce time to diagnosis and improve patient outcome.

References

1. Dott NM. Anomalies of intestinal rotation: their embryology and surgical aspects: with report of 5 cases. Br J Surg. 1923. 24:251-286.2. Ladd WE. Congenital Obstruction of the Duodenum in Children. N Engl J Med. 1932. 206:277-80.3. Palanivelu, C., Rangarajan, M., Shetty, A.R. et al, Intestinal malrotation with midgut volvulus presenting as acute abdomen in children: value of diagnostic and

therapeutic laparoscopy. J Laparoendosc Adv Surg Tech. 2007;17:490–492.4. Nehra, D., Goldstein, A.M. Intestinal malrotation: varied clinical presentation from infancy through adulthood. Surg. 2011;149:386–393. 5. Nilesh G. Nagdeve et.al , Malrotation beyond infancy. Journal of Pediatric Surgery,Volume 47, Issue 11, November 2012, Pages 2026-20326. Spitz, L., Orr, J.D., Harries, J.T. Obstructive jaundice secondary to chronic midgut volvulus. Arch Dis Child. 1983;58:383–385. 7. Kirby, C.P., Freeman, J.K., Ford, W.D.A. et al, Malrotation with recurrent volvulus presenting with cholestasis, pruritus, and pancreatitis. Pediatr Surg Int.

2000;16:130–131. 8. Sasaki, T., Soh, H., Kimura, T. et al, Recurrent acute pancreatitis caused by malrotation of the intestine and effective treatment with laparoscopic Ladd

procedure. Pediatr Surg Int. 2005;21:994–996.9. Durkin, E.T., Lund, D.P., Shaaban, A.F. et al, Age-related differences in diagnosis and morbidity of intestinal malrotation. J Am Coll Surg. 2008;206:658–663.10. Yanez, R, Spitz, L. Intestinal malrotation presenting outside the neonatal period. Arch Dis Child. 1986;61:682–685.11. Zellos A, Zarganis D, Ypsiladis S, Chatzis D, Papaioannou G, Bartsocas C. Malrotation of the intestine and chronic volvulus as a cause of protein-losing

enteropathy in infancy. Pediatrics. 2012 Feb. 129(2):e515-8.