neonatal Intestinal obstruction; etiology, diagnosis and management.pptx

neonatal intestinal obstruction is one of the major causes of neonatal surgical mortality. knowledge of the possible etiologies, diagnosis and management are essential in reducing this mortality.

1. MAKERERE UNIVERSITY SCHOOL OF MEDICINE DEPARTMENT OF ANESTHESIA $ CRITICAL CARE MEDICNE NEONATAL INTESTINAL OBSTRUCTION Supervisor: Dr. Phyllis Kisa Paediatric surgeon Paul Ssempebwa Mmed surgery 2 Tuesday 30 th may 2023

2. Little is much... Kittie Louise Suffield 1884 - 1972

3. outline Background and epidemiology Embryology Aetiology Clinical presentation Investigations Management Follow up and prognosis

4. Background Most common cause of surgical emergency in the newborn period. Wide spectrum of uncommon etiologies Incidence 1:2000 live births(Bishop JC, et 2020) In Uganda ARM are the most common etiology. (Otim P et al,2022). 37% mortality of neonatal surgical mortality is attributed to ARM.(Ullrich.et al, 2020,) Associated with other anomalies Predicators of mortality: prematurity fever at admission breastfeeding status mother’s parity cadre of healthcare providers Associated congenital anomalies Duration of symptoms

5. Embryology 3 to 4 th week formation of the embryonic disk. Cephalo caudal and lateral folding of the embryo. The endoderm lined yolk sac that is part of the embryo forms the primitive GIT. The other portions of the endoderm lined cavities form the yolk sac and the allantois . cephalad to the primitive gut is the foregut , caudal is the hindgut, in between is the midgut that remains attached to the yolk sac by the vitelline duct/yolk stalk .

6. Continued.. 5 th week; tabularization of the gut. Endoderm forms: epithelial lining, glands and organs like the liver and pancreas Mesoderm is divided into somatic and visceral mesoderm Somatic mesoderm forms; parietal peritoneum. Visceral mesoderm forms: mesentery and visceral peritoneum Foregut derivatives: esophagus to ligament of tretz (esophagus,stomach,liver,pancreas, duodenum) Midgut derivatives: jejunum,ileum,colon to the proximal 2/3 of the transverse colon Hindgut derivatives: distal 1/3 of the t.verse colon to the proximal rectum.

7. Etiology Intrinsic : atresia, stenosis & membranes Extrinsic causes : malrotations, Ladd’s bands Functional : Hirchsprung’s disease, NEC, Meconium illeus According to the level : High : above the ligament of tretz(TEF,HPS,Duodenal atresia/membranes) Medium : from the ligament of treitz to the ICJ.( jejunal illeal atresia,NEC,Meconium illues,malrotations) Low : beyond the ICJ: (HD,ARM,total colon aganglia,left colon syndrome)

8. Otim P,et al,2022

9. Clinical presentation Maternal polyhydramnios 3o% associated with anomalies(Tato s, et al,2016) Vomiting Diff. from regurgitation? Color of the vomitus? Gastric aspirates >20ml/ hr (Britton JR, et.al,1995) Abdominal distention Failure to pass meconium

10. Case 1 1/7 neonate Excessive frothing and difficulty in breathing Can hardly suckle. Prenatally : delivered by svd maternal history of polyhydramnios Has chest crepitations bilaterally

11. Case 2 Sudden onset of abdominal pain Wretching and vomiting of non billous content Abdominal distension

12. Case 3 2 week old neonate Initiated on cow’s milk due to the demise of the mother. Non billous vomiting Abdominal distension o/e: palpabe mass in the epigastrium

13. Case 4 2/52/M neonate Coffee brown projectile vomiting 0/E: dehydrated, irritable Scaphoid abdomen with a palpable epigastric round mass

14. Case 5 1/7 female neonate, delivered by SVD to a P4+0 mother Projectile bilious vomiting Failure to pass stool and abdominal distension

15. Intestinal malrotation Normally, intestinal herniation and counterclockwise bowel rotation by a 270°about the SMA while returning to the peritoneal cavity by 12 wks. There is bowel fixation. cecum in the right lower quadrant ,the duodenojejunal junction in the left upper quadrant. Broad mesenteric base, which inhibits volvulus or twisting of bowel about the vascular pedicle. Intestinal malrotation may result in an abnormal position of the cecum and duodenojejunal junction in close proximity, thereby creating a narrow mesenteric base which predisposes to twisting of bowel loops around the SMA – known as midgut volvulus. Spectrum of malrotation: No rotation Reverse rotation Incomplete rotation

16. Incidence 1: 5000 live births 1% in the general population(from autopsy reports) Incidence is greater with syndromes e.g.: heterotaxy,trisomy 21,gastroschisis,omphalocele, congenital diaphragmatic hernia among others. 75% of malrotations with mid gut volvulus occur in the neonatal period

17. clinical presentation Range: Asymptomatic(malrotation) to Severe symptoms(midgut volvulus) Symptoms: Abdominal pain, bilious vomiting,scaphoid abdomen with increasing swelling and tenderness. ischemia: abdominal wall erythema, melena, hematemesis, toxic appearance. Investigations: anemia, thrombocytopenia,leukocytosis,hyperkalemia, hypoalbuminemia. Imaging: Babygram : no diagnostic UGI contrast series:confirmatory , corkscrew duodenum, bird beak sign, Z shaped duodenum Ultrasound scan: duodenal dilatation with distal tapering whirl pool sign, due to the twisting of the mesentery around the SM vessels.

18. Ladd’s procedure Entry into abdominal cavity and evisceration (open) 2. Counterclockwise detorsion of the bowel (acute cases) 3. Division of Ladd cecal bands 4. Broadening of the small intestine mesentery 5. Incidental appendectomy 6. Placement of small bowel along the right lateral gutter and colon along the left lateral gutter

19. Intussusception Rare cause of neonatal i.o Occurrence is 0. to 1.3% non classical presentation of bilious vomiting, failure to pass stool and abdominal distension Preterm neonates it mimics NEC but no air within the biliary tree or pneumatosis intestinalis on X ray. Associated with a pathological lead point.

20. Jejunal illeal atresia Most common cause of neonatal I.O globally Jejunal_illeal : 1-3/5000 live births, 1/3 of whom are premature Incidence in men = females Survival rates >90% globally( Vechia.D et al,1998) ; 67% Uganda (Cairo.S, et al,2017) : Aetiology: Intrauterine ischemic events ? Genetic component, type 3b and worse prognosis Association: extra abdominal < 10%; intraabdominal – gastroschisis, omphalocele, malroatations, internal mesenteric hernias.

21. Grosfeld classification Why?

22. Meconium disease Spectrum; meconium illeus and meconium syndrome Meconium illeus 9 _33% of neonatal I.O Aetiology: viscid-thick, protein rich meconium that plugs the intestine esp. at ICJ causing I.O Associated with CF in 10 to 20% of the cases Cystic fibrosis: Autosomal recessive up to 5% cases Mutation CFTR, thus defective transmembrane transport Defective clearance of secretions

23. Meconium illeus Intestinal dysmotility due to thick, viscid, protein rich meconium in the immature bowel. Starts in utero. Classification: Simple meconium illeus Complicated meconium illeus Simple MI: Present after 24 hours of delivery Abdominal distension,failure to pass stool and bilious vomiting p/a: doughy/soft palpable mass with indention. Anus/rectum may feel atretic but its normal DDx : colonic atresia, HD meconium plug

24. Complicated MI: Early presentation within the first 24 hours of birth Complicated by gangrene, perforation, meconium peritonitis o/e: Neonates appear toxic, fevers, abdominal distension, abdominal edema May have a giant palpable pseudocyst

25. Investigations Prenatal USS cystic masses Babygram: dilated bowel loops ?air /fluid levels multiple calcifications pneumoperitoneum Fluoroscopy water soluble enema microcolon of misuse inspissated meconium diagnostic as well as therapeutic CF: sweat sodium > 60mmol/L in 100mg of swear CFTR mutations, F508 del is + ve 70%

26. Management Non Operative Management : Resuscitation Isotonic water soluble enema(sometimes N acetyl salicylate is used) Followed by NGT 5 to 10ml of N acetyl salicylate to liquify the meconium Repeat control imaging. Success rates are < 83% Complications< 23% cases of perforation Operative Management For simple Ml failed non operative management complication of non operative management like a perforation Laparotomy/laparoscopy: manual evacuation of meconium with 2% to 4% N acetyl Ceistene wash ** appendectomy Enterotomy +/- enterostomy Histology Prognosis 85-100% simple MI ; >95% Complicated MI

27. Hirchsprung’s disease Congenital absence of ganglion cells in the myenteric or submucosal plexus. Aetiopathogensis: failed migration/differentiation of neurocrest cells, Incidence: 1: 5000 live births 80% rectal sigmoid junction 10% proximal large colon 5-10% total colon aganglia. Associated: trisomy 21 congenital central hypoventilation syndrome, Goldberg–Shprintzen syndrome, Smith–Lemli– Opitz syndrome neurofibromatosis, and neuroblastoma Clinical Features: Delayed passage of meconium>90% cases Bilious vomiting Abdominal distention May be peritonitic in cases of cecal perforations Ix: Barium enema(helps to rule out a meconium plug) Transition zone in 90% of the cases Reverse Rectal anal ratio RAIR Histology: full thickness rectal biopsy; aganglia, hypertrophied nerve trunks

28. Operative management HD Swenson Duhamel Soave *one stage procedure safe, less invasive and low incidence of Hircshprung Associated EnteroColitis .( Ruttenstock,et al 2010)

29. Anal Rectal Malformations Epidemiology 1: 400,000, 1:500,000 live birth M:F 1.2:1 1% Chance of 2 nd child having ARM in affected couples. Associations: Downs’ Syndrome with ARM without a fistula VACTERL IVF Maternal Diabetes thalidomide

30. Classification

31. Algorithm for decision making in ARM

32. PSARP

33. complications Anal continence Superficial SSI Anal Stenosis Fecal Incontinence Rectal Mucosal Prolapse

34. Pre operative considerations Prompt diagnosis(includes prenatal screeing ) Adequate resuscitation Ngt Catheter Fluid and electrolyte replacement Analgesia Minimal invasive surgical options Multidisciplinary team Professional expertise Prompt referrals Family conferences

35. Post Operative considerations Resuscitation Fluid and electrolyte replacements Adequate safe analgesia Nutrition consideration Prevent risks to complications Prompt management of complications Rehabilitation

36. Thank you!

37. References 1. Bishop JC, McCormick B, Johnson CT, et al. The double bubble sign: duodenal atresia and associated genetic etiologies.  Fetal Diagn Ther . 2020. 47 (2):98-10 2. Sarah J. Ullrich, Nasser Kakembo, David F. Grabski, Maija Cheung, Phyllis Kisa, Mary Nabukenya, Janat Tumukunde, Tamara N. Fitzgerald, Monica Langer, Martin Situma, John Sekabira, Doruk Ozgediz, Burden and Outcomes of Neonatal Surgery in Uganda: Results of a Five-Year Prospective Study,Journal of Surgical Research,Volume 246,2020,Pages 93-99,ISSN 0022-4804, https://doi.org/10.1016/j.jss.2019.08.015 . ( https://www.sciencedirect.com/science/article/pii/S0022480419306213 ) 3. Otim P, Elobu EA, Mbiine R, et al. The etiological spectrum of bowel obstruction and early postoperative outcome among neonates at a tertiary hospital in Uganda. World Jnl Ped Surgery 2022;5:e000377. doi:10.1136/wjps-2021-000377 4. Tato S, Cabezas N, Gómez JA, Martínez JA. Diagnóstico prenatal de atresia ileal asociada a peritonitis meconial . Prog Obstet Ginecol . 2016;59(1):21-23. 5. Britton JR, Britton HL. Gastric aspirate volume at birth as an indicator of congenital intestinal obstruction. Acta Paediatr . 1995 Aug;84(8):945-6. doi : 10.1111/j.1651-2227.1995.tb13800.x. PMID: 7488825. 6. Dalla Vecchia LK, Grosfeld JL, West KW, et  al. Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg. 1998;133:490–496 7. Sarah Cairo1 • Nasser Kakembo2 • Phyllis Kisa2 • Arlene Muzira2 • Maija Cheung3 • James Healy3 • Doruk Ozgediz3,4 • John Sekabira Disparity in access and outcomes for emergency neonatal surgery: intestinal atresia in Kampala, Uganda, pediatr Surg int. 2017 8.Ruttenstock, E. and P. Puri (2010). "Systematic review and meta-analysis of enterocolitis after one-stage transanal pull-through procedure for Hirschsprung’s disease." Pediatric Surgery International 26 (11): 1101-1105