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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.