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.
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.
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 .
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 .
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.
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.
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 . 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 .
Other causes of hemobilia in adults include infection or stone
. 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
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 . 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%) .
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 , villous hyperplasia
 and even neoplastic changes.
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.