Hirschsprung_Disease_Lecture_Final_Year_MBBS.pptx

Final Year MBBS lecture according to KMU i.e. Khyber medical university Peshawar module. it is from Pediatric surgery chapter. This pptx is prepared according to Ganges's nine events. you can add quiz at the start of the lecture to assess their pre-lecture knowledge and then same quiz at the end of the lecture as a formative assessment. we use LMS system so my quiz is now uploaded separately in exam pattern.

Ref Bailey and Love's

1. Hirschsprung Disease Final Year MBBS Lecture Dr. Abdullah Khan Senior Registrar, PIMC/PIMS

2. Introduction - Congenital disorder of the colon - Due to absence of ganglion cells in submucosal & myenteric plexus - Leads to functional intestinal obstruction - Commonly affects rectosigmoid region

3. Learning Objectives - Understand etiology and pathogenesis - Recognize clinical presentation - Know diagnostic modalities - Discuss management options - Identify complications

4. Etiology & Pathogenesis - Arrest of neural crest cell migration - Aganglionic segment → tonic contraction - Male predominance (4:1) - Associated with Down syndrome

5. Clinical Features - Neonates: Failure to pass meconium > 48h - Abdominal distension, bilious vomiting - Older children: Chronic constipation, growth retardation - Enterocolitis: life-threatening complication

6. Investigations - Plain X-ray: dilated bowel loops - Contrast enema: Transition zone - Anorectal manometry: Absence of rectoanal inhibitory reflex - Gold standard: Rectal biopsy (absence of ganglion cells)

7. Management - Initial stabilization: IV fluids, NG tube decompression - Definitive: Surgical resection of aganglionic segment - Pull-through procedures (Swenson, Duhamel, Soave) - Laparoscopic/minimally invasive approaches increasingly used

8. Complications - Enterocolitis (most serious, cause of mortality) - Anastomotic leak/stricture - Persistent constipation/incontinence

9. Summary - Hirschsprung disease: congenital absence of ganglion cells - Presents with neonatal obstruction, chronic constipation - Diagnosis: rectal biopsy is gold standard - Definitive treatment: surgical resection and pull-through

10. Post-Lecture Quiz LMS POLL