Anorectal management med students
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Anorectal management med students
- 1. Management of Anorectal Problems in Paediatrics By Rae Oranmore-Brown FCS, MMed
- 2. Learning Outcomes • To know how to assess a neonate with an ano- rectal malformation • To recognise the signs and symptoms of Hirschsprungs Disease • To be aware of methods of diagnosis • To be aware of when to form a colostomy • To understand the principals of definitive surgery
- 3. What are the anorectal problems affecting the paediatric population? • Congenital anorectal malformations (ARM) • Hirschsprung’s Disease
- 4. ARM • ARM: congenital malformation in which the terminal part of the hindgut is abnormally placed and lies outside (partially or completely) the sphincter mechanism • Incidence: 1 in 4000 • Male:Female 60:40 • There is usually a connection between the distal rectum and genitourinary tract • 5% have no fistula
- 5. Associated congenital abnormalites VACTERL • Vertebral • Anorectal • Cardiac • Tracheoesophageal • Renal (genitourinary) • Limb abnormalities
- 6. Male ARM
- 7. Female ARM
- 9. Types of ARM
- 10. Management of male ARM
- 11. Management of female ARM
- 13. High imperforate anus with rectourethral fistula
- 16. Definitive Management • Posterior Saggittal Anorectoplasty (PSARP) • [May require abdominal approach in cases of high fistula (pull-through)] • Sphincter complex always identified by nerve stimulator at onset of surgery • Urethral catheter • Midline incision to find rectum and identify fistula • Pull mobilised rectum through sphincter muscles, reconstruct as necessary • Anocutaneous anastomosis
- 18. Supportive structures for continence
- 21. Rectum attached to prostatic bed
- 22. Post op
- 23. Outcome in ARM Type Faecal Continence (%) Constipation (%) Female Rectoperineal 100 55 Rectovestibular 95 55 Rectovaginal 70 30 Cloaca 55 30 Male Rectoperineal 100 55 Rectobulbar 85 55 Rectoprostatic 60 40 Rectobladder neck 20 15
- 24. Hirschsprung’s Disease • A motor disorder of the gut, which is caused by the failure of neural crest cells (precursors of enteric ganglion cells) to migrate completely during intestinal development during fetal life. The resulting aganglionic segment of the colon fails to relax, causing a functional obstruction.
- 25. Clinical presentation • Failure to pass meconium / delayed passage • Chronic constipation with abdominal distention • Squirt sign / blast sign
- 27. Investigations
- 28. Management • Decompress bowel – rectal washout, saline / soap enemas • Antibiotics if complicated by enterocolitis • Surgery: colostomy • Pull-through
- 29. References • J Neonat Surg. 2015 Jan 10; 4(1): 7,Bhatnagar. Anorectal Malformations part 1 • UCSF (University of California San Francisco) Medical Centre, Pediatric Surgery • Key Clinical Topics in paediatric surgery: M Pachl, MN de la Hunt & G Jawaheer (2014) • Handbook of Pediatric Surgery: CK Sinha, M Davenport (2010) • Congenital aganglionic megacolon (Hirschsprung disease): David E Wesson, MD (2016)
- 30. Questions
Editor's Notes
- Embryology Day 21: common chamber (cloaca) occluded by a membrane but visible from outside as an ectodermal pit – the proctodaeum Day 33: posterior hindgut separated from anterior urogenitial sinus by mesenchymal ingrowth of the urorectal septum Day 46: cloacal membrane breaks down Gene expression of Sonic Hedgehog (SHH) and other target genes (BMP44 & HOX5) regulate the process
- Defunctioning divided colostomy of the proximal part of the sigmoid colon: Separate two ends of colon, bridged by skin Distal loop: wash out to remove any trapped faeces Proximal stoma: project above skin surface – prevent spillover and faecal impaction of distal colon and rectum Distal stoma: small enough to prevent prolapse, but able to do distal wahout and imaging
- Post op care: anal dilation starting 2-3 weeks post op to prevent anal stricture for 3-6 months, depending on when stoma is reversed. (Stool passage prevents stricturing). Stoma can be reversed when neoanus is healed 2 weeks to 3 months post PSARP
- The higher the ARM, the lower the achieved continence Sacral anomalies are associated with poor continence Lower malformations tend to suffer more from constipation
- In about 80 percent of patients, the disorder affects the rectosigmoid colon (known as short-segment disease). In 15 to 20 percent of patients, the aganglionosis extends proximal to the sigmoid colon (known as long-segment disease). Hirschsprung disease (HD) occurs in approximately 1 in 5000 live births with an overall male:female ratio of 3:1 to 4:1; when the entire colon is involved, the gender ratio more nearly approaches 1:1
- The majority of patients with Hirschsprung disease (HD) are diagnosed in the neonatal period. Patients present with symptoms of distal intestinal obstruction: bilious emesis, abdominal distension, and failure to pass meconium or stool. The diagnosis can be suggested by a delay in passage of the first meconium (greater than 48 hours of age). By 48 hours of life, 100 percent of normal full-term neonates will pass meconium. In contrast, 50 to 90 percent of infants with Hirschsprung disease will fail to pass meconium within the first 48 hours of life. However, passage of stool within the first one to two days of life does not exclude the diagnosis. There may be an explosive expulsion of gas and stool after the digital rectal examination (squirt sign or blast sign), which may relieve the obstruction temporarily.
- AXR, Contrast enema Submucosal and rectal biopsy
- Affected infants also may present initially with enterocolitis, a potentially life-threatening illness in which patients have a sepsis-like picture with fever, vomiting, diarrhea, and abdominal distension, which can progress to toxic megacolon. Patients with enterocolitis require fluid resuscitation, intravenous (IV) antibiotic therapy including coverage for anaerobic bacteria, rectal irrigations, and, in rare cases, an emergency colostomy. Types of pull-thoughs: Swensons Duhamels Soaves Laparoscopy-assisted transanal pull through Transanal endorectal pull through