CASE PRESENTATION

Abdullah Almaghraby, MBBS

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

- 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.

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.

History

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

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

History

Medications:

1. Budesonide (budesonide nebulization)2. Albuterol (albuterol respiratory sol)3. Gentamycin (inhaled)4. Dornase alfa5. Ceftazidime6. Pancrelipase

History

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.

Vital signs:

Temperature: 36.8 CPulse: 124 bpmBlood Pressure: 100/71 mmHgOxygen saturation: 84% on room

Physical exam

His current weight is 21 Kg

His current height is 133 cm

General: alert and oriented Hands: clubbingHEENT: negativeRespiratory:

Sternal retractions Chest wall: Pectus carniatum Auscultation: decreased air entry on the right with bilateral crepitations

Cardiovascular: RRR S1, S2 no M

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

Physical exam

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

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

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

Investigations

Blood culture: –ve

Resp culture: +veMany mucoid Pseudomonas aeruginosa Scant Candida Albicans Moderate Normal respiratory flora

Investigations

Abdominal X ray

Chest X ray

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.

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

Investigations

Pancreatitis Deudenitis Cholecystitis Adhesions from previous surgery Intussusception Appendicitis Hernia Volvulus

Differntial diagnosis

PROVISIONAL DIAGNOSIS

Distal Intestinal Obstruction Syndrome (DIOS)

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.

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.

CLINICAL PRESENTATION

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 !!

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.

DISTAL INTESTINAL OBSTRUCTION SYNDROME (DIOS)

DIAGNOSIS

Clinical + Radiological

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.

RADIOGRAPHIC FEATURES

Gastrografin enema May help to find the level of obstruction Aids in treatment / reduction of obstruction

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.

COMPLICATIONS Hypovolemic shock Intestinal ischemia Intestinal perforation Chemical peritonitis Recurrent DIOS

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.

TREATMENT This treatment is accompanied with

extra oral or IV fluid. Significant recurrent abdominal

symptoms require full investigation by a gastroenterologist.

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.

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.

Clean-prep

TREATMENT Surgery is usually not required.  

colonoscopy is rarely necessary

Back to Our patient

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, BIDalbuterol (albuterol respiratory sol) 2.5 mg, NEB, q4hrgentamicin (gentamicin injectable) 80 mg, NEB, q12hrdornase alfa, 2.5 mg, Inhalation, BIDomeprazole, 20 mg, IV Piggyback, q12hrceftazidime, 1,000 mg, 5 mL, 100 mL/hr, IV Piggyback, q8hrPancrelipase: 20 cap ODPhytonadione; 10 ODMultivitamine: ADEK 1 tab OD

Treatment KFSH&RC

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.

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.

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.