Morning Report

Shannon Yonts, MD January 18, 2012

HPI
3 year old male Developed fever, vomiting, and abdominal pain 2 days ago Vomiting has been increasing
now up to 10 times per day

Vomit has been non-bloody non1 episode of green emesis today Fever up to 101 today

HPI (cont.)
Pain seems constant Decreased PO intake 1 wet diaper in last 24 hours Other symptoms:
Increased fussiness Decreased energy No diarrhea Last stool 3 days ago

No one else has been sick at home

HPI (Cont.)
Taken to OSH:
Given NS bolus 20 mL/kg, morphine 0.5 mg, Zofran Transferred to PCMC

Past Medical History

Cystic Fibrosis
Pulmonary and GI manifestations Past cultures positive for MRSA Diagnosed at 7 months of age
Had vomiting and hyponatremia Found to be malnourished History of loose stools

Past Surgical History

No surgeries

Immunizations
Up to date

Medications
Miralax 1 cap qday Claritin Phytonadoine Albuterol Pulmozyme Vitamin D MVI Iron Flonase Prevacid Creon 12, 2 caps with every meal AquADEKs Hypertonic saline

Allergies NKDA Family history

Mother with possible rheumatoid arthritis Father Crohns disease No family history of CF

Social History
Lives with parents Only child

Review of Systems
Positive as in HPI

Physical Exam
Vitals: T 38.3, HR 135, RR 30, BP 104/57, O2 sat 94% on room air General: Awake, very fussy, appears uncomfortable HEENT: PERRL, TM clear, No nasal congestion, oropharynx normal, moist mucous membranes CV: Tachycardic. No murmur. Pulses appropriate. Cap refill 2-3 seconds 2-

Physical Exam (cont.)

Lungs: Clear to auscultation. No increased work of breathing. Abdomen: Distended. Bowel sounds present, but hypoactive. Diffusely tender to palpation. No guarding. No rebound tenderness. No masses. Rectal: Small amount of soft stool Skin: Normal. No rashes Extremities: Warm. Good pulses. Neuro: Fussy. Awake and alert. Grossly normal strength.

Differential
Constipation Distal intestinal obstruction syndrome Intussusception Appendicitis Volvulus Obstruction Gastroenteritis C. difficile

Labs
CMP: Na 140, K 4.5, Cl 101, CO2 27, BUN 10, Cr 0.35, Glucose 94, LFTs - normal CBC: WBC- 12.1, Hct- 43, Plt 313 WBCHct Diff: Neutrophils 73%, Bands 0%, Lymphs 17%, Mono 9%.

Lactate: 2 Lipase: 38

Imaging
U/S:
No evidence of intussusception

KUB:
Dilated small bowel loops with air-fluid levels. airConsistent with bowel obstruction or focal ileus.

Brief Hospital Course

Mineral oil enema no return Consulted surgery Bilious emesis placed Anderson tube Given morphine for pain Desat placed on blow-by O2 blowContrast enema no return
Incomplete opacification of ascending colon

C. diff + Started IV Flagyl Started mucomyst PO and enemas

After 2 mucomyst enemas, had multiple stools and improved pain

After improved exam and stool output, started GoLytely clean out

Distal Intestinal Obstruction Syndrome (DIOS)

Formerly known as meconium ileus equivalent Acute Complete or partial obstruction of the small intestinal lumen by thick intestinal contents
Usually ileocecal junction

Epidemiology of DIOS
1010-47% of CF patients Most common > age 20 Uncommon under age 5 Tends to recur More common if history of meconium ileus

Risk Factors for DIOS

Severe genotype (delta F508) Pancreatic insufficiency Poorly controlled fat malabsorption Dehydration History of meconium ileus Prior episodes of DIOS s/p organ transplant

Pathogenesis
Not fully understood Pancreatic insufficiency
Altered fat absorption

Intestinal dysmotility Abnormal mucins, water, and electrolytes in stool content

Clinical Manifestations
Crampy abdominal pain Usually right lower quadrant Acute onset
Differentiates from constipation

Symptoms progressively severe May have constipation, diarrhea, or normal stool pattern Vomiting after complete obstruction May palpate mass in right lower quadrant

Diagnosis
Triad
Abdominal pain Right lower quadrant mass Stool in distal small intestine and right colon on x-ray x-

Management
Largely empirical few trials Incomplete
Oral rehydration Osmotic laxatives

Complete
Surgery consult Place NG IV hydration Correct electrolyte abnormalities Gastrografin enema

Management
If not vomiting
Miralax GoLytely Gastrografin PO

If vomiting
Consider gastrografin enema (in radiology)
Hyperosmolar Could lead to hypotension, shock, bowel perforation

Mucomyst (N-acetylcysteine) (NMucolytic

Surgery

Mineral oil enema and Fleet enemas usually not effective

References
Colombo, C. Ellemunter, H. et al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. Journal of Cystic Fibrosis. 2011 Jun;10 Suppl 2:S24-8. Fibrosis. 2:S24Van Der Doef, H., Kokke, F. et al. Intestinal obstruction syndromes in cystic fibrosis: Meconium ileus, distal intestinal obstruction syndrome, and constipation. Current Gastroenterology Reports. 2011 Jun;13(3):265Jun;13(3):26570. Katkin, J., Schultz, K. Cystic fibrosis: Overview of gastrointestinal disease. UpToDate. Oct. 11, 2011. UpToDate.