Ten- Year-Old Female with Dieulafoy Lesion Treated with Therapeutic Endoscopy And Literature Review


 Background There are many causes of sudden gastrointestinal bleeding in children, Dieulafoy lesion is very rare. This vascular abnormality can be fatal without appropriate treatment.Case presentation Retrospective analysis of the clinical manifestations, endoscopic features and treatment of a Chinese child with Dieulafoy lesion and review relevant literatures.Result A 10-year-old girl was admitted to hospital with sudden massive hematemesis and melena. Abdominal CT revealed suspected submucosal bleeding in the stomach. Finally the disease was diagnosed due to the typical manifestations with endoscopy. Meanwhile, we used electrocoagulation and hemoclips to hemostasis under endoscopy. No recurrence of hematemesis identified during the 4-weeks follow-up.Conclusion Dieulafoy lesion in children is rare cause of sudden gastrointestinal hemorrhage. But differential diagnosis cannot ignore it. Endoscope is the optimize choice for diagnosis and treatment.


Background
Dieulafoy lesion (DL),also known as Dieulafoy disease, is a rare but fatal cause of gastrointestinal hemorrhage [1]. Early diagnosis and appropriate treatment are vital. We report a ten-year-old female, presenting with hematemesis and melena, who was diagnosed with Dieulafoy lesion and treated with electrocoagulation and hemoclips successfully.

Case Presentation
A 10-year-old Chinese girl, with history of hematemesis, was admitted to pediatrics intensive care unit(PICU) of Shengjing hospital of China Medical University with massive hematemesis and melena. She denied surgical history and was not taking any medicine. No known signi cant gastrointestinal (GI ) conditions within the family. On admission physical examination, her temperature was 36.5℃, heart rate of 134 beats per minute, blood pressure was 95/56 mmHg, respiratory rate of 25 per minute. Her body weight was 40 kg. She was pale, and abdominal examination was unremarkable. No other phycial abnormalities were noted. Her initial laboratory testing revealed white cell count of 12.7 × 10 9 /L(normal: 4-10 × 10 9 /L) hemoglobin(HB) of 7.8 g/dL (normal: 12-15.5 g/dL), platelet count of 134 × 10 9 /L (normal: 100-300 × 10 9 /L). The results of other laboratory tests (including coagulation function test) were unremarkable. An abdominal computed tomography (CT) revealed suspected mucosal hemorrhage in the cardia of gastric, and slight dilation of small submucosal blood vessels.
On the day of admission, she was managed with proton pump inhibitor (1 mg/kg.d), intravenous uids and nutritional support, and diet resistance. However, she developed another massive melena and her HB had decreased to 6.9 g/dl. She was subsequently transferred to our department and two units of packed red blood cells were transfused. An gastroscopy with a transparent hood over the head was urgently performed, and bright red blood was noted in the stomach. A careful examination revealed the presence of an actively bleeding protruding vessel in the posterior wall of the body of stomach (Fig. 1).We treated it with a cautery probe, during which the lesion initially oozed. Two endoscopic hemoclips were applied with full control of bleeding (Fig. 2). The rest of the stomach mucosa did not show any other bleeding lesion and ulceration. The patient had no further complaints at her follow-up at the outpatient clinic 4 weeks later.

Discussion And Conclusions
Dieulafoy lesion(DL), described by French pathologist Dieulafoy rst, manifests itself with spontaneous recurrent gastrointestinal bleeding (GIB). It is observed in about 0.3-6.7% percent of the causes of upper GIB [2]. There is no accurate statistics on the incidence of this disease in children. DL is a large penetrating artery which is a normal vessel with an unexpectedly large diameter, the vessel caliber is 1-3 mm. This penetrating artery creates a small wall defect with brinoid necrosis found at the base. It does not known clearly about the mechanisms of the pathologic bleeding of DL in date. Newborn cases suggest DL can be congenital and a congenital anomaly may develop acute ruptures. The mechnical friction, chemical corrosion or drugs can induce protruding vessel ruptured and massive bleeding [3,4]. In adult, DL is more common in men than women and middle aged and old people have more cases [5].
Unlike adult patients, the pediatric cases do not appear to have a gender preference [6].
The small nature of the lesion and the special sites of the hemorrhage are the two features of DL. Most lesions are in the proximal stomach, particularly within 6-10 cm of the lesser curvature of the stomach, where blood supply comes directly from the arteriae gastrica sinistra [7]. Nongastric sites are also involved in DL such as the duodenum, jejunum, ileum, rectum and even in the bronchus [8].
It is challenging to diagnose DL exactly because of the features of the disease. Endoscopy, angiography and surgical search are the primary diagnostic modalities. RBC scintigraphy can also detect the site of bleeding. Undoubtedly, endoscopy is the most feasible method. Initial endoscopies can diagnose precisely over 71% cases [9]. As the subtle lesions exist, multiple endoscopies are needed in some patients.
The endoscopic visual criteria of DL includes: (A) active arterial spurting or micro pulsatile streaming from a mucosal defect < 3 mm, (B) visualization of a vessel protruding from a slight defect or normal mucosa, or (C) a fresh blood clot adherent to a minute mucosal defect or a normal-appearing mucosa [10,11].
To diagnose a Dieulafoy lesion clearly and safely, these principles should be included in endoscopic exam [12]: (1) During period of active bleeding, emergency endoscopy should be performed under the anti-shock therapy. (2) The lesion may be exposed by cleaning gastral cavity with moderate endoscopic perfusion. Timely endoscopy and treatments can decrease the mortality of DL sharply [13]. Endoscopy is recommended as the rst-line method of treatment [14]. Endoscopic treatments include thermal electrocoagulation, heat probe coagulation, laser photocoagulation, regional injection-epinephrine, sclerotherapy, norepinephrine injection, band ligation and hemoclips [15,16]. Mechanical banding and hemoclips are more effective than thermal electrocoagulation and injection [17]. The combination of electrocoagulation and hemoclips may be more reliable. As in our case the procedure was adopted.
To patients who failing endoscopic therapy, angiography with gel foam embolization should be suggested [18]. Surgical management, regarded as the only treatment initially available before, is reserved for patients who are refractory to endoscopy and angiography. Endoscope combined with laparoscopic surgery is a new attempt, which is less invasive than traditional surgery and easy to be accepted by patients [19].
In general, DL as a cause of life-threatening bleeding is rare occurrences in the pediatric population. But pediatrician should be aware of it as a differential diagnosis of pediatric GIB. Endoscopy is still the primary diagnostic tool and the rst-  Hemostasis controled after electrocoagulation and hemoclips.