Case Report

Hemobilia Induced by Gall Bladder Polyps

Reza Nazarzadeh¹, Saeedeh Majidi¹*, Parya Dehghanian², Ali Alamdaran³ and Mehran Hiradfar⁴

¹Department of Pediatric Surgery, Mashhad University of Medical Science, Iran

²Department of Pathology, Akbar Hospital & Mashhad University of Medical Science, Iran

³Department of Radiology, Akbar Hospital & Mashhad University of Medical Science, Iran

⁴Endoscopic and Minimally Invasive Surgery Research Center, Mashhad University of Medical Science, Iran

Submission: June 18, 2019;; Published: July 11, 2019

*Corresponding author: Saeedeh Majidi, Department of Pediatric Surgery, Mashhad University of Medical Science, Mashhad, Iran

How to cite this article: Reza Nazarzadeh, Saeedeh Majidi, Parya Dehghanian, Ali Alamdaran, Mehran Hiradfar. Hemobilia Induced by Gall Bladder Polyps. JOJ Case Stud. 2019; 10(3): 555787. DOI: 10.19080/JOJCS.2019.10.555787.

Abstract

Hemobilia is an aberrant connection of hepatic biliary and vascular systems. It usually occurs post traumatic, blunt trauma or iatrogenic. Non traumatic hemobilia is a rare occurrence which there are few case reports in children which were due to pancreatic pseudocyst, choledochal cyst, hepatic abscess, hepatic fascioliasis and Meta chromatic leukodystrophy. All reported cases are female except one with von Willebrand’s disease. In few patients Meta chromatic leukodystrophy (MLD) was the cause of hemobilia. MLD is a rare neurological disease which is caused by Sulfatids deposition in nervous tissue and it has three subtypes, late infantile, juvenile and adult type. The gall bladder may be involved in the disease course and presents with different symptoms like abdominal pain or hemobilia. We present an eight-year-old girl with late infantile MLD who presented with upper GI bleeding and in evaluation the diagnosis of hemobilia and gall bladder polyps was confirmed.

Keywords: Hemobilia; Tissue hyperplasia; Metachromatic leukodystrophy; Blunt trauma; Abdominal pain

Introduction

Gall bladder polyps are tissue hyperplasia, which protrude inside the gall bladder from the inner surface. They may be found accidentally or present with vague abdominal pain and vomiting. GBPs are rare in general population and are even less common in pediatric age group [1].

The most important cause of gallbladder polyps in children is Metachromatic Leukodystrophy (MLD). MLD is a lysosomal storage disease, which progressively affects central and peripheral nervous system. It has three sub types based on the age of manifestation onset. In late infantile subtype, the child development is normal in the first 2 years. Neurological impairment and developmental delay manifest after this period [2].

Gallbladder involvement in MLD is a known condition, which may have no significant symptoms or present with different manifestations such as abdominal pain, vomiting or upper GI bleeding. Hemobilia due to MLD is a rare entity. We present a child with MLD with neurological impairment who was admitted with massive hematemesis due to Gallbladder polyps.

Case

An eight-year-old girl referred to emergency ward of Akbar Hospital with melena and massive hematemesis, which started since 12 hours ago. She was a known case of Metachromatic Leukodystrophy (MLD) since the age of 4 years.

Physical examination revealed Pale conjunctiva. No abdominal tenderness or mass was palpable. Vital signs at the time of admission were: Blood pressure= 100/60mmHg, pulse rate = 120/min with normal respiratory rate and temperature. The hemoglobin dropped to 7.5 from 11.5gr/Dl during transporting from first local hospital.

She was the first child of consanguineous parents with history of term delivery and her birth weight was 3900 grams. She experienced normal development until about two years of age, then she gradually showed developmental delay, regression of speech and ability of walking and seizure attacks occurred.

At the age of three, she was not able to walk due to muscle spasm with loss of DTR and no Babinski reflex. Verbal skill was lost, and hearing tests showed auditory impairment. Sensorimotor (demyelinating) type of peripheral neuropathy was shown in EMG study. In brain MRI there was extensive signal abnormality in both centrum semi-oval with sparing subcortical U-fibers. Typical involvement of the corpus callosum and brain stem was present (Figure 1A). In MR Spectroscopy, increase in Choline was suggestive of active myelin breakdown, which is compatible with the diagnosis of MLD. In addition, lactate rise was reported in MR spectroscopy, which was related to ischemia and necrotic component.

US showed thickened calcified irregular gallbladder wall and multiple polypoid lesions in gallbladder. GB was filled with blood. Two intraluminal cystic lesions were also reported in gallbladder fundus (Figure 1B & 1C).

After stabilizing the vital signs and transfusion of blood, upper GI endoscopy was performed which showed active bleeding coming through the orifice of ampulla of Vater. Due to persistent bleeding and hematochezia, exploratory celiotomy and cholecystectomy was performed. External biliary tract was washed-out by-passing catheter through cystic duct that was filled with blood.

Gross pathology examination revealed that the gallbladder was distended with multiple intramural yellowish lesions in cut surface of GB (Figure 1D & 1E). Microscopic study revealed mucosal papillary thickening and presence of foam cells in subepithelial stroma with focal low-grade intraepithelial dysplasia (Figure 1F).

Oral intake was started the day after operation. Melena stopped about 4 days later. Patient discharged home with no episode of recurrent bleeding.

Discussion

Hemobilia is a rare condition in general population which occurs in 2.5% of patients after blunt liver trauma and in 3%- 7% after hepatobiliary [3]. Hemobilia in children is even rarer and usually occurs after blunt abdominal trauma. This is due to communication between aneurysm in hepatic arteries and the biliary tract and may become a life-threatening condition with 25% mortality rate. Selective hepatic artery embolization is an appropriate treatment for this condition [4].

Other causes of hemobilia in adults include infection or stone [5]. There are some case reports of non-traumatic hemobilia in children that are mentioned in Table 1. All of them except one with von willebrand disease were female. Severe anemia reported as a hallmark of clinical presentation in nearly all of cases. Due to continuous and profuse bleeding, blood transfusion was done in most of cases.

Metachromatic Leukodystrophy (MLD) is a main cause of gallbladder polyps and non-traumatic hemobilia in children, which is a rare lysosomal disease that has autosomal recessive inheritance. MLD is due to deficiency in Arylsulfatase A (ARSA) enzyme which causes accumulation of sulfatides especially in nervous tissue. This induces progressive lethal demyelinating process [13].

MLD should be suspected in a patient with progressive neurological disorder and developmental retardation. Brain MRI findings show evidence of leukodystrophy that become more prominent culminating in cerebral atrophy with progression of the disease. Confirmation of diagnosis is by detection of biallelic Arylsulfatase A (ARSA) pathologic variant on genetic test, increased sulfatide excretion in urine or detecting metachromatic deposition in tissue biopsy of nervous system or affected viscera.

Based on the time of occurrence of the first symptoms, MLD has three subtypes. It consists of “late infantile”, “juvenile” and “adult” forms. Patients in the late infantile subgroup, like the reported case, show neurologic symptoms usually before 30 months of age [2]. This subgroup involves 50%- 60% of all affected individuals and has the worst course and prognosis. Most patients (61%) of this group present with gait abnormalities and seizures (39%) [14].

Association of MLD and gallbladder abnormalities has been reported in literature and Gallbladder involvement seems to be a rule. In addition, it can present before the main neurological symptoms and may be key to the diagnosis of MLD [15,16]. Pathologic evaluation shows different abnormalities such as hyperplastic polyps, metaplasia, dysplasia, sulfatides accumulation and metachromatic stain [17], villous hyperplasia [18] and even neoplastic changes.

Conclusion

Gallbladder involvement MLD disease usually presents with abdominal pain, vomiting or jaundice. Data search revealed hemobilia in only four cases of MLD [6,10,19,20]. Presence of GIB in MLD cases mandate evaluation of the biliary tract.GB is the first culprit in this scenario.

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