HAEC

“Hirshcprung Associated
Enterocolitis
 DEFINITION
• Hirschsprung-associated enterocolitis (HAEC) is the most
severe and potentially lethal complication of HD
• Hirschsprung-associated enterocolitis (HAEC), an inflammatory
disorder of the bowel related to Hirschsprung disease

https://www.uptodate.com/contents/emergency-complications-of-hirschsprung-disease?csi=840fcbb7-1966-
41f6-b696-f74821d43a90&source=contentShare
 ANATOMY

Standring, S., Borley, N. R., & Gray, H. (2008). Gray's anatomy: the anatomical basis of clinical practice. 40th ed., anniversary ed. [Edinburgh]:
Churchill Livingstone/Elsevier.
ANATOMY

Heuckeroth RO (2018). Hirschsprung disease - integrating basic science and clinical

medicine to improve outcomes. Nat Rev Gastroenterol Hepatol. Mar;15(3):152-167..
 EPIDEMIOLOGY
• Hirschprung  most common cause LIO in neonates, 1:5000 newborn
• HAEC occurs preoperatively or at the time of HD diagnosis in 6–26 % of cases and post pull-through surgery
in 5–42 %

Demehri, F.R., Halaweish, I.F., Coran, A.G. et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment
and prevention. Pediatr Surg Int 29, 873–881 (2013).
https://www.uptodate.com/contents/emergency-complications-of-hirschsprung-disease?csi=840fcbb7-1966-
41f6-b696-f74821d43a90&source=contentShare
 PATHOGENESIS
• HAEC remains poorly understood, clinical entity of HAEC likely represents a common result of various
dysfunctions of intestinal homeostasis.
• Partial obstruction, which may lead to stasis, bacterial overgrowth and translocation of an anastomotic
stricture
• Abnormal enteric nervous system (ENS)  create an abnormal intestinal equilibrium, where can create
partial obstruction or bacterial overgrowth may lead to enterocolitis.
• Abnormal epithelial barrier function (EBF) a composite function of factors including mucin production,
intraluminal immunoglobulins, epithelial tight junctions and the ENS adherence of pathologic organisms to
enterocytes (i.e. enteroadhesion), a phenomenon demonstrated with the adherence of C. difficile,
Cryptosporidium, and E. coli
• Described above may create a dysfunctional environment in which the gut microbiome is susceptible to a
pathologic change in composition leading to HAEC

Demehri, F.R., Halaweish, I.F., Coran, A.G. et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment
and prevention. Pediatr Surg Int 29, 873–881 (2013).
 PATOPHYSIOLOGY

Demehri, F.R., Halaweish, I.F., Coran, A.G. et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment
and prevention. Pediatr Surg Int 29, 873–881 (2013).
CLINICAL PRESENTATION AND
PHYSICAL EXAMINATION

Elhalaby E, Coran A et al (1995) Enterocolitis associated with Hirschsprung’s disease: a clinical-radiological

characterization based on 168 patients. J Pediatr Surg 30(1):76–83
 CLINICAL PRESENTATION AND
PHYSICAL EXAMINATION

Pastor A, Osman F et al (2009) Development of a standardized definition for Hirschsprung’s-associated

enterocolitis: a Delphi analysis. J Pediatr Surg 44(1):251–256
Ankush G et Al (2017) Guidelines for the diagnosis and management of Hirschsprung-
associated enterocolitis Pediatr Surg Int.
 Workup
• Plain Radiograph Gaseous intestinal distension with abrupt cutoff at the level of
the pelvic brim—the intestinal cutoff sign
• rectal biopsy in the diagnosis of HAEC is controversial and not recommended
• A contrast enema should not be performed if HAEC is suspected, because
of the risk of intestinal perforation
• Computed uted tomography (CT) has been described as showing thickening of
the wall of the entire large bowel and terminal ileum, with enhancement of the
mucosa

Demehri, F.R., Halaweish, I.F., Coran, A.G. et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment
and prevention. Pediatr Surg Int 29, 873–881 (2013).
Sheth J, Nour S, Dickinson F. CT images of Hirschsprung's associated enterocolitis: a rare
finding. Arch Dis Child 2009; 94:816.
Elhalaby E, Coran A et al (1995) Enterocolitis associated with
Hirschsprung’s disease: a clinical-radiological characterization
based on 168 patients. J Pediatr Surg 30(1):76–83
 MANAGEMENT (GENERAL)
• Resucitation
• Decompression of GIT
• Medication
• Antibiotics
• Sodium Chromogylcate

Demehri, F.R., Halaweish, I.F., Coran, A.G. et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment
and prevention. Pediatr Surg Int 29, 873–881 (2013).
 MANAGEMENT
(OPERATIVE/INTERVENTON)
• Posterior myotomy/myectomy
(POMM)

Mark Daventport et al . Rob and Smith’s Operative Pediatric Surgery 8 th Edition . 2020.
CRC press.
 MANAGEMENT
(OPERATIVE/INTERVENTON)
• Sphincterotomy  post pull-through patients often have tight rectal
sphincters
• Ileostomy or colostomy  if HAEC not responsive to medical and
surgical intervention

Demehri, F.R., Halaweish, I.F., Coran, A.G. et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment
and prevention. Pediatr Surg Int 29, 873–881 (2013).
 MANAGEMENT (NON OPERATIVE)
• Antibiotics
• Sodium cromoglycate  a mast cell stabilizer
• Bowel Rest
• Decompression
• Rectal Washout  Rectal washouts with saline (10–20 mL/kg) using a large
bore soft tube repeated anywhere from two to four times per day until proper
decompression as determined by clinical examination.

Demehri, F.R., Halaweish, I.F., Coran, A.G. et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment
and prevention. Pediatr Surg Int 29, 873–881 (2013).
COMPLICATION
• Shock
• Peritonitis

Ankush G et Al (2017) Guidelines for the diagnosis and management of Hirschsprung-

associated enterocolitis Pediatr Surg Int.
ALGORITHM MANAGEMENT

Ankush G et Al (2017) Guidelines for the diagnosis and management of Hirschsprung-associated

enterocolitis Pediatr Surg Int.
THANK YOU