Dieulafoy lesion (DL) refers to gastrointestinal (GI) ulcers associated with erosion of a superficial large-caliber artery and massive bleeding. It accounts for 0.5% to 14% of upper GI bleeding in adults and is extremely rare in children (1). It occurs mostly over the lesser curvature of the stomach. An increasing number of cases have been reported in children in the last 10 years, and to the best of our knowledge, we report the first case of a sigmoid DL associated with a juvenile rectal polyp in an obese 6-year-old child. We also reviewed GI DL in children, discussing their epidemiology, etiology, location, clinical presentation, diagnosis, treatment, and frequency of recurrent bleeding.
A 6-year-old girl, previously healthy, presented to the emergency department with painless fresh blood per rectum 1 hour before presentation. She gave no history of vomiting, diarrhea, constipation, or of a similar condition. On physical examination she was an obese child with a body mass index of 21 kg/m2 (>97th percentile for age), afebrile, and hemodynamically stable. She had no rash or petechiae. Her abdomen was soft, nontender, and nondistended with normal bowel sounds and no hepatosplenomegaly. Her rectal examination showed no perianal lesions, adequate anal tone, and no palpable rectal lesions. Laboratory studies showed hemoglobin of 13.9 g/dL; white blood cell count of 9700/mm3 with 49% segmented, 40% lymphocytes, 9% monocytes, and 3% eosinophils; and a platelet count of 296 × 103/mm3. Prothrombin and partial thromboplastin times were normal. Stool for Wright stain showed red blood cells but no ova or parasites, and stool culture was negative.
The patient was hospitalized for additional investigation and management. Rectal bleeding decreased in amount during the next hospital day, and repeat hemoglobin level was stable. The Meckel radionuclide scan was negative. Colonoscopy on the following day showed a small nonbleeding rectal polyp and a bleeding submucosal vessel in the sigmoid colon, 25 cm from the anal verge, which is consistent with DL (Fig. 1). The polyp was resected and the DL was cauterized using a 7-F (2.3 mm) gold probe electrohemostasis catheter with Endostat II cautery machine (Boston Scientific, Boston, MA) with good hemostasis. Pathology of the resected lesion was compatible with a juvenile rectal polyp. The patient was asymptomatic 24 hours after endoscopy. She remained symptom free with stable hemoglobin level 1 year after discharge.
DL is named after the French surgeon Paul Dieulafoy, who reported in 1888 3 cases of gastric ulcers in which the ulcer progression was interrupted by massive bleeding secondary to erosion of a large submucosal arterial vessel in its base. He called those lesions exulceration simplex. It was speculated that the presence of large superficial arteriole causes continuous pressure on the overlying mucosa, leading to its thinning and rendering it more susceptible to traumatic ulceration and vessel erosion with time, which probably explains its frequency in adults and its association with massive bleeding. In addition, adult studies suggest that exposure to certain irritating agents such as alcohol and nonsteroidal anti-inflammatory drugs in gastric lesions and hard fecal material in colonic lesions increase the likelihood of DL in adults who are more exposed to these agents compared with children. It was suggested that the condition may be either acquired or congenital (2).
To better understand DL in children, we performed a literature search through PubMed, MEDLINE, Embase, and SCOPUS search engines, using the MeSH terms Dieulafoy Lesion, Dieulafoy ulcer, Dieulafoy disease, Dieulafoy lesion, Dieulafoy ulcer, Dieulafoy disease, caliber persistent artery, and exulceration simplex. The same terms were then used with the age limit of 18 years to identify pediatric cases. In addition, the references of the retrieved articles were screened for information relevant to our search objectives. Our search identified 30 case reports of pediatric DL compared with hundreds of adult cases during the same period of time. Three pediatric case reports (3–5) could not be retrieved and were not included in our discussion. The characteristics of the remaining 27 retrieved cases in addition to our case are presented in Table 1.
The first pediatric case of DL was reported in 1968 by Rossi et al (6) in a 20-month-old girl who had Christmas disease, and who presented with massive upper GI bleeding requiring surgical intervention. In 1986, Veldhuyzen et al published the first review article on 101 DL cases, among which 2 were reported in children (6–8). The male:female ratio of gastric lesions in that review was 2:1. Since then, isolated pediatric case reports have been published, the youngest of which were 2 neonates, 19 and 22 hours old, who presented with hematemesis (9). More than half of all pediatric DL cases (16/28) were reported after the year 2000, reflecting an increase in the number of DL in children during the last 10 years (Table 1). The lesions occurred most frequently in boys (male: female ratio of 1.5:1) presenting at any age starting from birth, which supports the theory of congenital presence of a superficial submucosal large-caliber arteriole that erodes easily upon ulceration of the overlying mucosa leading to massive bleeding. The number of reports increases with advancing age. The reported pediatric cases clustered around 3 age periods; 1 year and younger (6 cases), between 3 and 7 years (7 cases), and older than 10 years (14 cases) (Table 1). Earlier presentations were associated with pathological conditions such as hemophilia in 2 cases; however, the precipitating factor was unidentified in the rest of the cases (6,10).
Its clinical presentation varied according to the location of the lesion. Gastric and duodenal lesions presented with massive upper GI bleeding, small intestinal lesions presented with upper GI bleeding and/or hematochezia, whereas colonic lesions presented with fresh blood per rectum as in our case. In adults, around 70% of DLs are located in the stomach mostly over the lesser curvature within 6 cm of the cardia, which may be explained by the fact that submucosal arterial branches in that region arise directly from the left gastric artery, whereas in the rest of the GI tract they arise from serial branches. Other adult GI locations include esophagus, small bowel, rectum, and colon. Occasionally extragastrointestinal lesions have been reported in the bronchial tree where they present with hemoptysis (2). In children, the stomach was also the most common location in 57% of cases (16/28), followed by the jejunum (5/28 cases), the colon (4/28 cases), the duodenum (3/28 cases), and finally the ileum (1/28 cases). In addition, 1 child had 2 simultaneous lesions in 2 different GI locations: stomach and jejunum (11). Our case is the fourth reported case of colonic DL in children and the first case reported outside the rectum.
After 1995, endoscopy became the method of choice for diagnosis of pediatric DL of the gastroduodenal and rectosigmoid locations. Recent reports from adult patients indicate that diagnosis may be established after the first endoscopy in 70% to 97% of gastric lesions (12,13). Failure of diagnosis is attributed to the heavy bleeding, masking the lesion during endoscopy. That was also true in pediatric cases in which the initial endoscopy established the diagnosis in 8 of 10 gastroduodenal cases and in all of the colonic cases. In the remaining 2 gastric cases the diagnosis was missed because of heavy bleeding or because of the intermittent nature of the arterial bleeding; both cases were diagnosed after a second endoscopy. Jejunoileal lesions were the most difficult to diagnose, requiring different diagnostic methods including exploratory laparotomy, angiography, 99mTc-labeled red blood cell scan, or endoscopic capsule (Table 1).
As for treatment, endoscopic hemostasis was first used in adults to control the bleeding lesions in 1991. It includes a variety of hemostatic methods such as hemoclipping, thermocoagulation, hypertonic saline-epinephrine injection and/or pure ethanol injection, or sclerosis (12,13). Those same interventions were reported in children since 1995 for treatment of gastroduodenal and rectosigmoid lesions (Table 1). The most common modality used for hemostasis in gastric lesions was injection therapy (6/8) cases; hemoclipping was used in 1 case and band ligation in another case. All of the small bowel lesions required surgical intervention for hemostasis. Colonic lesions were treated with endoscopic thermocoagulation (13). Two complicated cases required surgical intervention; the first case was a child with a gastric lesion and hemophilia (10), and the second case was a girl with a rectal lesion and extensive burns (14).
Recurrence of bleeding is reported in >10% of adult cases, and it depends on the hemostasis method used. Mechanical methods such as band ligation and hemoclipping achieve permanent hemostasis in almost 100% of adult cases (12,15). In addition, administrations of nonsteroidal anti-inflammatory drugs or anticoagulants after endoscopic hemostasis increase the risk of rebleeding (1). In children, of the 8 gastric cases treated endoscopically, rebleeding occurred in 2 patients (25%). In the first case, the initial bleeding was controlled by endoscopic injection and rebleeding with band ligation, whereas the second case had 2 simultaneous lesions in the stomach and in the jejunum, and required surgical intervention (11).
Colonic DLs are less commonly reported in the literature. Forty-two cases of colon DL cases were reported in our literature review, 3 of them were described in children. Colon lesions were most frequently located in the right colon and rectum. The reported pediatric cases include a 5-year-old girl who presented with acute rectal bleeding requiring blood transfusion and was treated by endoscopic thermocoagulation (16), a 7-year-old boy with recurrent episodes of small amounts of painless rectal bleeding who was also treated by endoscopic thermocoagulation (17), and an 18-year-old girl with 45% total body surface area burn who developed sudden massive rectal bleeding (14) and required surgical intervention to control her bleeding. Our case is the fourth case of colon DL and is the first case described in the sigmoid colon in a child, a location that has been previously reported in 1 adult case (18).
Our case is also the first case of DL described in association with a juvenile rectal polyp, which is the most common GI tumor in children. It is present in 1% to 3% of population younger than 18 years of age and is diagnosed most often in the first decade of life, with a peak incidence between 2 and 6 years of age. Juvenile polyps are usually isolated and benign. Their etiology is unknown, yet they are considered developmental inflammatory malformations. Clinically, they present with intermittent rectal bleeding, whereas massive rectal bleeding is a rare presentation (19). Whole colon endoscopy, polypectomy, and pathology examination to rule out malignancy are the methods of choice for diagnosis and treatment. The association between a rare condition such as sigmoid DL and a more common condition such as juvenile polyp as in our case may be coincidental, unless one considers the probability of a chronic local irritation of the colonic mucosa leading to the development of inflammatory polyp and to mucosal ulceration eroding the wall of superficial submucosal artery in the sigmoid colon causing DL.
DL is a rare condition increasingly recognized as a cause of GI bleeding in children. The growth may be due to increasing awareness, increasing use of diagnostic endoscopy in children, and some unrecognized conditions to be identified. Endoscopy is the method of choice for diagnosis and treatment of gastroduodenal and colon lesions. Ileal and jejunal lesions require a high index of suspicion and more complex diagnostic techniques. Its association with juvenile polyp may be coincidental.
1. Lim W, Kim TO, Park SB, et al
. Endoscopic treatment of dieulafoy lesions and risk factors for rebleeding. Kor J Intern Med 2009; 24:318–322.
2. Jain R, Chetty R. Dieulafoy disease of the colon. Arch Pathol Lab Med 2009; 133:1865–1867.
3. Sarin YK, Sharma AK. Dieulafoy's lesion in a child. Indian Pediatr 1993; 30:545.
4. Robson ME, McLennan J. Dieulafoy's vascular malformation and primary pulmonary hypertension in a 10 year-old girl. Histopathology 1994; 25:279–281.
5. Goenka AS, Dasilva MS, Cleghorn GJ, et al
. Therapeutic upper gastrointestinal endoscopy in children: an audit of 443 procedures and literature review. J Gastroenterol Hepatol 1993; 8:44–51.
6. Rossi NP, Green EW, Pike JD. Massive bleeding of the upper-gastrointestinal tract due to Dieulafoy's erosion. Arch Surg 1968; 97:797–800.
7. Veldhuyzen van Zanten SJ, Bartelsman JF, Schipper ME, et al
. Recurrent haematemesis from Dieulafoy vascular malformations: a review of 101 cases. Gut 1986; 27:213–222.
8. Gough MH. Submucosal arterial malformation of the stomach as the probable cause of recurrent severe haematemesis in a 16-year-old girl. Br J Surg 1977; 64:522–524.
9. Koo YH, Jang JS, Cho JH, et al
. Endoscopic injection treatment for gastric dieulafoy lesion in two newborn infants. Kor J Gastroenterol 2005; 46:413–417.
10. Pitcher GJ, Bowley DM, Chasumba G, et al
. Life-threatening haemorrhage from a gastric Dieulafoy lesion in a child with haemophilia. Haemophilia 2002; 8:719–720.
11. Marangoni G, Cresswell A, Faraj W, et al
. An uncommon cause of life-threatening gastrointestinal bleeding: 2 synchronous Dieulafoy lesions. J Pediatr Surg 2009; 44:441–443.
12. Sone Y, Kumada T, Toyoda H, et al
. Endoscopic management and follow up of Dieulafoy lesion in the upper gastrointestinal tract. Endoscopy 2005; 37:449–453.
13. Linhares MM, Filho BH, Schraibman V, et al
. Dieulafoy lesion: endoscopic and surgical management. Surg Laparosc Endosc Percutan Tech 2006; 16:1–3.
14. Guy RJ, Ang ES, Tan KC, et al
. Massive bleeding from a Dieulafoy-like lesion of the rectum in a burns patient. Burns 2001; 27:767–769.
15. Alis H, Oner OZ, Kalayci MU, et al
. Is endoscopic band ligation superior to injection therapy for Dieulafoy lesion? Surg Endosc 2009; 23:1465–1469.
16. Tooson JD, Maranon LS, Gates LK Jr. Pediatric rectal Dieulafoy's lesion. Am J Gastroenterol 1995; 90:2235–2243.
17. Meister T, Varilec G, Marsanao LS, et al. Endoscopic management of rectal Dieulafoy like lesions. A case series and review of the literature. Gastrointest Endosc
18. Dharia T, Tang SJ, Lara L. Bleeding sigmoid colonic Dieulafoy lesion. Gastrointest Endosc 2009; 70:1028, 1028–9.
19. Wang LC, Lee HC, Yeung CY, et al
. Gastrointestinal polyps in children. Pediatr Neonatol 2009; 50:196–201.