Massive Gastrointestinal Bleeding from a Dieulafoy Lesion in a Seven Year Old Boy

1. Massive Gastrointestinal Bleeding from a Dieulafoy Lesion in a Seven Year Old Boy Amana N. Nasir, Carolyn M. Wilhelm, Joel A. Levien, John N. Udall, Jr.

2. History of Present Illness • A 7yo boy was transferred to WCH from an outside hospital with a right lung pneumonia and pleural effusion. • He had received amoxicillin, azithromycin and 5 days of high dose ibuprofen prior to being hospitalized at the outside facility.

3. Past Medical History • Unremarkable for chronic illnesses • No chronic medications • There had been no hospitalizations or surgeries • No known drug allergies

4. Admission Chest Radiographs

5. Hospital Day 1 • Hemoglobin 11.3gm% • Hematocrit 32.3 % • Started on IV ceftriaxone and vancomycin

6. Hospital Day 2 • Right chest tube placed • He vomited 15cc of blood and passed melanotic stools during the night • Transferred to the PICU • His H/H fell to 7.4 gm% / 21.7% (admission H/H were 11.3 gm% / 32.3%) • Two units PRBCs and 1 unit FFP were given • Started on IV pantoprazole

7. Hospital Day 3 • Pediatric GI service consulted • Pediatric GI examination • Tachypneia, tachycardia and normal BP • Tenderness in the epigastrium • Rectal examination was followed by the passage of grossly bloody stool • Impression- gastritis and/or stress ulcer • Plan- close observation, consider EGD

8. Hospital Day 4 • H/H increased to 10.3 gm% / 29.5% • Sucralfate slurries were added • Decrease in melanotic stools • No additional hematemasis • Continued epigastric discomfort

9. Hospital Day 9 • The pt. had a 2nd episode of hematemesis (40-50ccs) • H/H dropped to 8.5gm% / 25.3 % • EGD performed (1st EGD) • Blood clots throughout the stomach but no active bleeding • 2 moderate sized duodenal ulcers (one with a white eschar base and one with an overlying clot) • Started on IV pantoprzole and octreotide drips • Transfused 3 units PRBCs & 1 unit of FFP

10. Cardia of stomach and pylorus(1st EGD)

11. Ulcer eschar and ulcer with clot

12. Hospital Day 10 • The patient became pale, diaphoretic and hypotensive • NG tube placed and blood suctioned • The patient was taken for emergency EGD (2nd EGD)

13. Hospital Day 10 • At EGD the same clean based ulcer with an eschar was seen in the duodenal bulb and in the duodenal sweep a blood clot overlying a moderate sized blood vessel was noted • The area around the blood vessel was injected with 2.5mL of 1:10,000 epinephrine • The area and ulcer base was then gently cauterized with a Gold heater probe

14. Cautery with Gold heater probe(2nd EGD)

15. Hospital Day 11-16 • Following the 2nd EGD the patient was transfused with 4 more units of PRBCs. He remained stable with no signs of bleeding. • On the 16th day the patient had a third episode of hematemesis (400cc) that required 2 units of PRBCs. • A fasting serum gastrin level was normal. • Possible surgical intervention was discussed with the family. However, there was no additional evidence of active bleeding.

16. Hospital Day 21 • Prior to discharge another endoscopy (3rd EGD) was performed. There was no active bleeding, no blood clots and both duodenal ulcers appeared to be healing. • Biopsies from the gastric antrum showed chronic gastritis but no Helicobacter pylori. • The patient was discharged on high doses of pantoprazole, ranitidine and sucralfate.

17. Pylorus and healing Diuelofy lesion(3rd EGD)

18. Summary • During his WCH stay our patient received a total of 11 units of PRBCs and 2 units of FFP • On discharge his H/H was 12.6gm%/ 36.8%

19. Follow up • At a clinic visit two weeks after discharge he was stable. There had been no further hematemesis or melena . The H/H was 14.2 gm% / 42%. He was taking pantoprazole 20 mg tid, ranitidine 75 mg bid and sucralfate 500 mg qid. The same medications and doses were continued except for the sucralfate which was discontinued. • At a clinic visit six weeks after discharge he remained asymptomatic. The H/H was 13.2 gm% / 38.5%. The ranitidine was discontinued at the six week visit and the pantopazole was decreased to 20 mg bid.

20. Dieulafoy lesion • First described by T. Gallard in 1884 and later by G. Dieulafoy in 1896 • Proposed etiology- an unusually large and tortuous artery that runs in the submucosa • massive bleeding occurs when the vessel is exposed or erodes as it approximates the mucosa • Most common in the lesser curvature of the stomach, but reported to occur in bronchi and in the esophagus, small and large intestine

21. Accounts for less than 2% of all upper GI bleeds • May be underestimated due to difficulty in diagnosis • Diagnosis may be complicated due to the intermittent nature of the bleeding • Found primarily in adults • Twice as common in men as women

22. Rarely reported in the pediatric population • In the English literature, there have been 8 reported pediatric cases, ranging in age from 13 months to 15 years • To our knowledge, this is the third pediatric case in the English literature of a small intestinal Dieulafoy lesion.

23. Diagnosis • The diagnosis is established by endoscopy but the lesion can be difficult to identify • The lesion may be noted as a bleeding arteriole or noted as a clot overlying a vessel (our case) • In most cases the surrounding mucosa is normal • Multiple endoscopic procedures may be necessary before the lesion is found • The diagnosis in a few cases has been established by capsule endoscopy, arteriography or endoscopic ultrasound

24. Treatment • Endoscopic interventions (most commonly employed) • injection of epinephrine or sclerosing agents, thermocoagulation, photocoagulation or band ligation • In our case epinephrine injection and electrocaudery were used • Surgical interventions (less commonly employed) • Reserved when endoscopic intervention fails • Includes over-sewing of the lesion or wide resection. • Associated with more postoperative complications • Angiography with embolization has also been used when the lesion is found in the jejunum

25. Conclusion • Dieulafoy lesions are rare in the pediatric age group and can be difficult to diagnose. • Our case illustrates the success of endoscopy for diagnosis and treatment.

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