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Case Presentation

Case Presentation. Abdullah Almaghraby, MBBS. History. A 16 year old male with Cystic Fibrosis has End stage lung disease on O2. Pancreatic insufficiency. On PEG tube for feeding. Presented to ER with epigastric pain,vomiting and no bowel motion for 2 days. History.

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Case Presentation

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  1. Case Presentation Abdullah Almaghraby, MBBS

  2. History A 16 year old male with Cystic Fibrosis has • End stage lung disease on O2. • Pancreatic insufficiency. • On PEG tube for feeding. Presented to ER with epigastric pain,vomiting and no bowel motion for 2 days.

  3. History - The epigastric pain: Started gradually, aching in nature, progressing with time radiated to the back, and relieved with leaning forward. - The vomiting: Started with the onset of pain. He vomited twice; food content, non projectile, and non bilious. - The Constipation: Started before the onset of pain. His usual bowel habit is one bowel motion per day, but for the last couple of days no motion has occurred.

  4. History • The patient denies any history of: • Fever • Contact with ill person. • Jaundice. • Heartburn. • Previous gall-stones. • Urinary symptoms. • Worsening of his respiratory effort. • His feeding tube is patent according to the mother. • His appetite decreased . • He had similar episode 2 months ago.

  5. History Past Surgical History: • Initial insertion of percutaneous endoscopic gastrostomy [PEG] tube in 2010 at 14 Years.

  6. History • Neonatal History: • Product of full term without neonatal complications. • Nutrition: • His growth parameters are far below the 5th Centile, on PEG tube feeding • Immunization schedule: • up to date • Allergies: • Negative

  7. History Medications: • Budesonide (budesonide nebulization) • Albuterol (albuterol respiratory sol) • Gentamycin (inhaled) • Dornase alfa • Ceftazidime • Pancrelipase

  8. Summary A 16 year old with end-stage lung disease related to his cystic fibrosis, pancreatic insufficiency, presented with abdominal pain, vomiting and constipation for 2 days prior to admission.

  9. Physical exam • Vital signs: • Temperature: 36.8 C • Pulse: 124 bpm • Blood Pressure: 100/71 mmHg • Oxygen saturation: 84% on room

  10. His current weight is 21 Kg

  11. His current height is 133 cm

  12. Physical exam • General:alert and oriented • Hands: clubbing • HEENT: negative • Respiratory: • Sternal retractions • Chest wall:Pectus carniatum • Auscultation:decreased air entry on the right with bilateral crepitations • Cardiovascular: • RRR S1, S2 no M

  13. Physical exam • Abdomen: • Soft and lax with mild epigastric tenderness. • No masses or organomegaly • PEG tube on the left upper quadrant • Increased intensity of bowel sounds

  14. Investigations CBC • WBC 4.11 10^9/L • RBC 4.61 10^12/L • Hemoglobin 122 g/L LO • Hematocrit 0.395 L/L • MCV 85.7 fL • RDW 16.0 % HI • Platelet 184 10^9/L

  15. Investigations Electrolytes • K 4.5 mmol/L • Na 141 mmol/L • Cl 97 mmol/L LO • PO4 1.64 mmol/L HI • Mg 0.86 mmol/L • CO2 36 mmol/L HI • Ca, Total 2.35 mmol/L

  16. Investigations Chemistry • Glucose, Random 4.9 mmol/L • Albumin 38 g/L LO • Urea 3.0 mmol/L • Creatinine 35 umol/L LO • ALT 8 U/L • AST 18 U/L • Alk Phos 134 U/L • GGT 25 IU/L • Lipase Level 70 IU/L HI

  17. Investigations • Blood culture: –ve • Resp culture: +ve • Many mucoid Pseudomonas aeruginosa • Scant Candida Albicans • Moderate Normal respiratory flora

  18. Investigations Abdominal X ray Chest X ray

  19. Investigations • The abdomen is distended. • The liver is enlarged. • Gastrostomy tube in position. • There is no definite sign of intestinal obstruction or perforation. • The ground-glass appearance of the abdomen may raise the possibility of free fluid within the abdomen.

  20. Investigations • Extensive fibrotic densities with cystic changes, right much more than the left. • Hyperinflated left lung. • Loss of volume of the right lung. • Heart and mediastinum shifted to the right side. • There is no evidence of pleural effusion or pneumothorax. • Few air-fluid levels seen within the cystic changes on the right side

  21. Differntial diagnosis • Pancreatitis • Deudenitis • Cholecystitis • Adhesions from previous surgery • Intussusception • Appendicitis • Hernia • Volvulus

  22. Provisional diagnosis Distal Intestinal Obstruction Syndrome (DIOS)

  23. Distal Intestinal Obstruction Syndrome (DIOS) • Is a problem with the intestine. • The food and mucus partially or completely block the intestine causing pain. • About 1 out of 10-20 people with CF get DIOS. • Prevalence is highest in the 2nd and 3rd decades of life.

  24. Clinical presentation • Stomach aches • Periumbilical pain • Lower abdominal pain • Loss of appetite • Early satiety • Vomiting • Constipation • Hard, immobile mass is often felt on the right side of the abdomen.

  25. Risk factors • Born with a meconium ileus • Have had gut surgery • Have had episodes of DIOS in the past. • Not taking the enzymes. • The enzymes is not working (pH effect) • Dehydration • Interestingly, the incidence of DIOS is increased after lung transplantation !!

  26. Distal Intestinal Obstruction Syndrome (DIOS) • Clinical findings may mimic those of appendicitis. • Despite the common distension of the appendix by inspissated secretions, the reported prevalence of acute appendicitis in CF patients is lower than that in the general population.

  27. Diagnosis • Clinical + Radiological

  28. Radiographic features Abdominal radiograph • small bowel obstruction • bubbly soft tissue mass in the right lower quadrant • The gut filled with large amounts of impacted feces at the end of the small intestine and/or caecum and ascending colon. • It may also show dilated loops of the small intestine.

  29. Radiographic features Gastrografin enema • May help to find the level of obstruction • Aids in treatment / reduction of obstruction

  30. Radiographic features CT • Typically seen to affect the right colon • Colonic wall thickening • Mesenteric soft-tissue infiltration • Increased pericolonic fat • The appendix is routinely distended (> 6 mm) in the absence of appendicitis resulting from mucoid impaction, • Therefore the diagnosis of appendicitis should not be made unless secondary signs are present.

  31. complications • Hypovolemic shock • Intestinal ischemia • Intestinal perforation • Chemical peritonitis • Recurrent DIOS

  32. Treatment • Golytely (an intestinal lavage solution) is used orally, via a nasogastric tube or button. • This uses osmotic pressure to draw fluid into the gut to dislodge impacted stool.

  33. Treatment • This treatment is accompanied with extra oral or IV fluid. • Significant recurrent abdominal symptoms require full investigation by a gastroenterologist.

  34. Clean-prep Do NOT give in the presence of bile stained vomiting. • Add contents of 1 sachet to 1 litre water – can be flavoured with a clear juice. • Can be given orally or via NG tube (usually latter) a single dose of domperidone 30 minutes before starting can increase gastric emptying. • Do not administer just before bedtime due to risk of aspiration. • Start at 10ml/kg/hour for 30 mins then 20 ml/kg/hour for 30 mins.

  35. Clean-prep • If well tolerated rate can go up to 25 ml/kg/hour. • Maximum volume is 100 ml/kg or 4 litres (whichever is smaller) over 4 hours. • Patients must be reviewed after 1st 4 hours. • If not passing essentially clear fluid per rectum then a further 4 hours treatment can be given. • Maximum daily dose should be 200 ml/kg or 8 litres • Monitor for hypoglycaemia, which can occur with CF diabetics undergoing this regimen.

  36. Treatment • Surgery is usually not required. • colonoscopy is rarely necessary

  37. Back to Our patient

  38. Treatment • KFSH&RC Current medications: • Polyethylene glycol 3350 with electrolytes (GoLYTELY) 4L, through gastrostomy tube over 4hours, till the rectal effluent is clear • budesonide (budesonide nebulization) 0.5 mg, NEB, BID • albuterol (albuterol respiratory sol) 2.5 mg, NEB, q4hr • gentamicin (gentamicin injectable) 80 mg, NEB, q12hr • dornase alfa, 2.5 mg, Inhalation, BID • omeprazole, 20 mg, IV Piggyback, q12hr • ceftazidime, 1,000 mg, 5 mL, 100 mL/hr, IV Piggyback, q8hr • Pancrelipase:20 cap OD • Phytonadione;10 OD • Multivitamine: ADEK 1 tab OD

  39. prevention • Ensure adequate enzyme is taken for all foods and fluids, but avoid excessive doses of enzyme. • Ensure adequate fluid (milk, water, salt replacement drink etc) and salt intake, especially when playing sport, in hotter climates, and during illness.

  40. prevention • Eat lots of fruit, vegetables and fiber containing breads and cereals in addition to a high calorie, high salt diet. • Take regular stool softening medication. • Omeprazole may be prescribed in order to alkalinize the medium for the pancreatic enzymes.

  41. Emergency colonoscopy for DIOS • This technique proved safe and was relatively well tolerated in this cohort. • Emergency colonoscopy, undertaken early in patients with progressive symptoms who prove refractory to medical therapy, is a novel and effective modality of therapy and avoids the need for surgical intervention.

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