Case Report: A Case of Infant Bronchial Dieulafoy's Disease and Article Review

Background: Bronchial Dieulafoy's disease (BDD), characterized by constant diameter arterial malformation, is rare, especially among infants. The pathogenesis and clinical features of pediatric patients are unknown. Misdiagnosis and biopsy operations may lead to potential massive hemorrhage, which endangers the patient's life.Case Presentation: Here, we present a case of a 9-month-old boy who was diagnosed with BDD with massive hemoptysis. The boy was cured by embolization of the bronchial artery and was in good health at the 1-year follow-up. In addition, we searched PubMed, Google Scholar, and Web of Science databases using keyword “Bronchial Dieulafoy's Disease (BDD)” and found six additional cases of pediatric BDD.Conclusion: It is still insufficient to draw a conclusion about the origin of the disease. Bronchial angiography and endobronchial ultrasonography are considered promising methods to diagnose Dieulafoy's disease of the bronchus. Bronchoscopy with transbronchial biopsy should not be deployed due to the high risk of fatal hemorrhage. Explicit clinical case reports of BDD are needed to enhance the understanding of this rare disease.

DIEULAFOY’S DISEASE IN FORENSIC PRACTICE

Dieulafoy’s disease is a genetically determined lesion manifested in a developmental malformation of the vessels in the submucosal layer of the stomach with arrosion of an abnormally large artery. Dieulafoy’s ulcer is relatively uncommon and causes 0.4–1 % of all acute gastric bleedings, twice as often in men than in women. With the advent of endoscopy, its mortality rate decreased from 80 to 20 %. At the macroscopic scale, Dieulafoy’s arrosion is oval or star-shaped, with the mucous membrane “raised” above the bleeding vessel in the form of a polyp. In 80 % of the cases, the haemorrhage occurs at a 5–6 cm distance from the oesophageal-gastric anastomosis, most commonly in the lesser curvature. Microscopically, the wall of the arrosed artery is affected by proliferation and sclerosis of the intima, degeneration of the middle layer and disappearance of elastic fibres. This article describes a case from forensic practice that may be of interest to doctors of various specialities. During autopsy of citizen F., his stomach was found to contain two litres of black-brown clotted blood. Examination of the gastric mucosa revealed a lesion in the lesser curvature 5 cm below the oesophageal aperture with characteristic histological markers of Dieulafoy’s disease. Accordingly, death of citizen F. was caused by a massive gastric haemorrhage of a mucosal lesion developed in progression of Dieulafoy’s disease. This case highlights the risks associated with this pathology, as a massive gastric bleeding without proper timely surgery is potentially lethal.

A rare and fatal respiratory disease: bronchial Dieulafoy’s disease

A 66-year-old woman had two severe episodes of massive hemoptysis without any premonitory symptoms, with approximately 400–500 ml blood each time. Bronchoscopic exam revealed a smooth and pulsatile protrusion that was approximately 8–10 mm in diameter found at the beginning of the right middle lobe bronchus in the bronchial lumen. The protrusion arose from the surface with absolutely normal mucosa. Selective bronchial arteriography showed that elongated, tortuous, and dilated branches of the bronchial artery in the region of the middle lobe bronchus. Further bronchial arterial embolization (BAE) is recommended, although the patient currently has no active bleeding. Bronchial Dieulafoy’s disease (BDD) is a rare and life-threatening disease. Selective bronchial arteriography is a diagnostic tool to detect and locate abnormal arteries. There is no unified guideline or expert consensus on the treatment of BDD. Selective BAE or surgical resection is usually used as a first-line treatment to control hemoptysis. The reviews of this paper are available via the supplemental material section.