A rare anomaly: duodenal Dieulafoy’s lesion in a preterm baby

Abstract

Neonatal Dieulafoy’s lesion is a rare but serious condition that can be life-threatening if not diagnosed and intervened in a timely manner. It presents with episodes of sudden acute gastrointestinal haemorrhage in the form of blood in vomit and/or blood in stool. In general, most of the lesions are successfully treated with endoscopic or angiographic intervention. Surgery is usually reserved for cases that fail endoscopic or angiographic intervention. We present a neonatal case of duodenal Dieulafoy’s lesion that occurred in a 29-week-old male baby with birth weight of 1.2 kg. He developed melena and haematemesis at 4 weeks of life. He required normal saline boluses and transfusion of blood products for acute blood loss. The lesion was successfully treated with endoscopic intervention.

Background

Dieulafoy’s lesion is the developmental vascular malformation where normal artery with large diameter is situated under mucous membrane and protrudes into the gastric lumen through a small defect. The presenting signs are similar to many other conditions that occur in early infancy and prematurity. Dieulafoy’s lesion is not common in infancy and is very rare in neonatal population and if not diagnosed and treated in time can cause significant morbidity. Rupture can also result in a massive haemorrhage, which, if not promptly treated, can be fatal. We report a case of Dieulafoy’s lesion in a preterm baby who was successfully treated with endoscopic intervention.

Case presentation

A male baby with birth weight of 1.2 kg was born at 29 weeks of gestation by lower segment caesarean section in view of pregnancy-induced hypertension. The mother is 26 years old primigravida with normal antenatal screening blood tests and antenatal scan. After birth, the baby received positive pressure ventilation by self-inflating bag and mask for 15 s and started on non-invasive ventilation support. He received intramuscular vitamin K soon after birth.

His early neonatal period was uneventful and required minimal respiratory support with continuous positive airway pressure and high-flow nasal cannula oxygen for 1 week.

Feeds were introduced on day 4 and were gradually increased to reach full feeds by day 18 of life. He developed features of sepsis on day 20. His C reactive protein was elevated, measuring 167 mg/L, and blood culture grew Serratia marcescens. Lumbar puncture showed 500 White blood cell count/mm³ of cerebrospinal fluid (CSF) and neutrophil count was 80%. CSF culture was negative. He received antibiotics for 21 days.

At 4 weeks of life, the baby had episode of sudden pallor, melena and blood-stained aspirates in the orogastric tube. His haemoglobin dropped to 50 g/L, requiring normal saline bolus and packed red cell transfusion. His coagulation profile and platelet counts were normal. Abdominal X-ray was normal except mildly distended bowel loops. He was treated conservatively for necrotising enterocolitis. One week later, he had a second episode of melena with a drop in haemoglobin to 55 g/L, requiring blood transfusion and he was transferred to our hospital for further evaluation and management. Within 48 hours of admission to our Neonatal intensive care unit, he developed three episodes of sudden pallor with haematemesis of approximately 10 mL each time and two episodes of melena required repeated normal saline boluses, packed red cells, fresh frozen plasma and platelet transfusion. Sepsis screen was normal. X-ray of the abdomen showed distension of bowel loops with bowel wall oedema. Abdominal ultrasound was reported normal. Upper gastrointestinal (GI) endoscopy revealed a duodenal ulcer with pulsatile artery protruding into the lumen of first part of duodenum (figure 1). All relevant investigations are shown in table 1.

Figure 1.

Small pink-coloured mass can be seen protruded in the lumen.

Table 1.

Relevant investigations

Investigations	Timeline
	During third week	At 4 weeks	At 5 weeks	At 5–6 weeks
C reactive protein	167 mg/L	Normal	Normal	Normal
White blood cells in CSF	500/mm3
CSF culture	No growth
Blood culture	Serratia marcescens	Sterile	Sterile
Coagulation profile		Normal	Normal
Urine culture		No growth
Blood in vomit				3 episodes
Blood in stool (melena)		Frank blood in stools	Frank blood in stools	Frank blood in stools: 2 episodes
Stool for occult			Present
Blood
Haemoglobin (Hb)	Normal	Hb dropped to 5 gm%	Hb dropped to 5.5 gm%	Hb dropped to 5–5.5 gm%
Platelet count	Normal	Normal	Normal	Low
X-ray abdomen		Mildly distended bowel loops only	Mildly distended bowel loops only	Mildly distended bowel loops and bowel wall oedema only
Abdomen ultrasound		Normal		Normal
Brain ultrasound	Normal	Grade 1 germinal matrix haemorrhage+grade 1 periventricular leukomalacia	Grade 3 changes periventricular leukomalacia	Grade 3 changes periventricular leukomalacia
Endoscopy (upper gastrointestinal)				Duodenal ulcer with pulsatile artery

APC, Argon plasma coagulation; CSF, cerebrospinal fluid; WBC, White blood cells.

Differential diagnosis

The baby received vitamin K at birth so haemorrhagic disease of newborn was a rare possibility. Septic markers were already improved, platelet count and coagulation profile were normal, so sepsis and necrotising enterocolitis were ruled out, and coagulopathy was unlikely.

There was no episode of sudden excessive cry or jelly-like stools, and the baby was passing regular stools without any obstruction or tenderness of the abdomen. Hence, the possibility of intussusception, volvulus and Meckel’s diverticulum was unlikely. The baby was not sick looking in between the bleeding episodes, and bleeding was sudden and in significant quantity, so the presentation was like rupture of blood vessel with some vascular malformation. To rule out ulcer which was a rare possibility and vascular malformations including Dieulafoy’s lesion, GI endoscopy was performed.

Treatment

During the upper GI endoscopy, epinephrine was injected locally around the lesion. During the procedure, bleed was managed with packed red cell transfusion and fresh frozen plasma. Octreotide infusion therapy was started. Following the local epinephrine injection, blood-stained aspirates had subsided. His blood sugar remained normal. He had second endoscopy with GIF XP190 video gastroscope within 48 hours after the first intervention. The endoscope was advanced to the level of duodenum and deployed the argon plasma coagulation catheter (30W 1 L/m), and sporadic argon plasma coagulation was done around the site successfully (figure 2).

Figure 2.

The protruded mass in the lumen has shrunk in size and the colour has changed from pink to greyish creamy.

Outcome and follow-up

There was no recurrence of GI bleed following the endoscopic haemostatic procedure. The baby was transferred back to the referring hospital on full feeds, in room air within a week.

Discussion

Dieulafoy’s lesion is the presence of a histologically normal artery with a large diameter which runs in the submucosa and protrudes into the gastric lumen through a small mucosal defect.¹ Dieulafoy’s lesion is also termed ‘calibre persistent artery’. Primarily described by Gallard in 1884, it was more precisely distinguished by the French surgeon Georges Dieulafoy in 1898 after his study of fatal gastric haemorrhage in three asymptomatic young men. Dieulafoy’s lesions typically present as sudden massive haemorrhage, which is often recurrent without treatment.^{1 2}

Dieulafoy’s lesion accounts for 0.3%–6.7% of the upper GI tract bleeding cases in adults. The prevalence in paediatric population is unknown with approximately 28 reported cases in English literature and it may be due to under-reporting or undiagnosed cases. Dieulafoy’s lesion is rare in neonatal population and even rarer in preterm population. To our knowledge, ours is the third case described in premature babies.^2–4

Dieulafoy’s lesion is an extremely rare cause of sudden pallor, gastric haemorrhage and melena in the neonatal population. However, it is a serious condition that can be catastrophic if remain undiagnosed and not intervened in a timely manner. These lesions are commonly found in the gastric mucosa. Their presence in duodenum as seen in our case is uncommon.

Dieulafoy’s lesions are typically managed with endoscopic interventions, with high success rates reported with various modalities such as thermocoagulation, band ligation, administration of sclerosants and hemoclip application, thereby limiting the need for surgical intervention.^4–6

In our case, prematurity and very low birth weight at 1.2 kg caused significant size limitation when using neonatal endoscope. Due to this reason, Argon plasma coagulation was considered the most appropriate treatment option as other treatment options such as thermocoagulation or clips would have increased the risk of complications such as perforation due to narrow working channel. When the initial endoscopic examination revealed the lesion, local epinephrine injection was used as a temporising measure. The second endoscopic intervention with APC was planned after making sure that necessary surgical expertise was available during the procedure. The need for surgical intervention has been reported in very few newborns who continued to have upper GI bleeding secondary to a Dieulafoy’s lesion.⁷ Our case is the only reported case of successful endoscopic management of Dieulafoy’s lesion located in the duodenum in a preterm baby with very low birth weight. As in our case, replacement of blood products with packed cell transfusion, fresh frozen plasma and platelets may be required.

Common causes of GI bleed in preterm babies with very low birth weight are necrotising enterocolitis, vitamin K deficiency, deranged coagulation, Meckel’s diverticulum and volvulus. Other causes include swallowed blood due to intraoral or intranasal injury and gastric perforation. Dieulafoy’s lesion should be considered in the differential diagnosis in sudden, massive and/or recurrent GI bleeding.

This report demonstrates that Dieulafoy’s lesions can be managed effectively with endoscopic approach in preterm babies with very low birth weight. However, it must be remembered that the lack of fine endoscope and the tiny size of the stomach and duodenum can pose a challenge for endoscopic procedures in extreme preterm babies.

Patient’s perspective.

We as a parent were worried about our baby because he was bleeding intermittently without any detectable cause. Medical team was doing their efforts and finally they diagnosed it and our baby was saved. Thanks to the medical team and the doctors.

Learning points.

• Sudden pallor and drop in haemoglobin, negative sepsis screen, normal coagulation profile and non-significant radiological investigation are the clues for heading to gastrointestinal endoscopy for further diagnosis.

• Dieulafoy’s lesion can be treated successfully by endoscopy with argon plasma coagulation.

• Bleeding during endoscopy can be controlled by octreotide infusion hours before procedure and epinephrine injection during treatment.

Ethics statements

Patient consent for publication

Obtained.