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Indian Pediatr 2016;53: 833-834 |
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Ruptured Hepatocellular
Carcinoma in a Child with Budd-Chiari Syndrome
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Anandini Suri, Varun K Sharma, Prajakta R Ranade,
#Shaji Marar and *Aabha
Nagral
From Departments of Pediatrics, #Interventional
Radiology and *Gastroenterology, Jaslok hospital and Research Centre,
Mumbai, India.
Correspondence to: Dr Aabha Nagral, 7, Snehasagar,
Prabhanagar, Prabhadevi, Mumbai 400 025, India.
Email: [email protected]
Received: August 04, 2015;
Initial review: October 20, 2015;
Accepted: July 09, 2016.
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Background: Hepatocellular carcinoma is an uncommon complication
described in patients with Budd-Chiari syndrome. Case characteristics:
A 12-year-old boy with Budd-Chiari syndrome, who was earlier treated
with Transjugular intrahepatic porto-systemic shunt (TIPS), presented
with acute onset hemoperitoneum and hypotension. Outcome: It was
diagnosed to be a case of ruptured hepatocellular carcinoma. Message:
Successful TIPS may not prevent the development of hepatocellular
carcinoma, and children with Budd Chiari syndrome should be monitored
for the same.
Keywords: Complication, Hemoperitoneum, Liver tumor, TIPS.
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Budd-Chiari Syndrome (BCS) is a progressive
disease leading to portal hypertension, liver dysfunction,
hepatopulmonary syndrome, and cirrhosis. Hepatocellular carcinoma (HCC)
is a rare but potential complication of BCS. It has been reported in few
adult patients despite undergoing radiological intervention like
Transjugular Intrahepatic Porto-systemic Shunt (TIPS) [1]. We present a
12-year-old boy, a known case of BCS treated successfully with TIPS, who
presented with hemoperitoneum due to a ruptured HCC.
Case Report
A 12-year-old boy, known case of BCS, presented with
an acute onset abdominal pain with hypovolemic shock. He had
tachycardia, tachypnea and hypotension, and was pale. His abdomen was
distended along with tenderness and guarding.
His illness first manifested at the age of four years
with abdominal distension (ascites) and hematemesis. At 6 years, he
presented to our institute with failure to thrive, refractory ascites
and firm hepatosplenomegaly. Complete blood count showed evidence of
hypersplenism. Endoscopy revealed small esophageal varices. There was no
coagulopathy or liver dysfunction. Diagnosis of BCS was confirmed on the
basis of ultrasound (USG) Doppler and computed tomography (CT)
angiography which revealed complete occlusion of the middle and left
hepatic veins and short segmental occlusion of the right hepatic vein
(RHV) ostium. A combined percutaneous- transjugular approach was used to
stent the RHV ostium. He showed good clinical response, but after 6
months developed stent occlusion. He underwent TIPS procedure with 10 mm
× 60 mm GORE VIATORR stent. The portosystemic gradient reduced from 28
mmHg to 3 mmHg. There were no immediate complications. On follow-up,
ascites subsided and weight improved (from <5 th
centile
to 25th
centile). He had no further variceal bleeds. The patient was put on
anticoagulation with warfarin. Thrombophillia workup (Protein C, Protein
S, Anti thrombin III, Lupus anticoagulant, Anti cardiolipin antibodies)
and JAK2 mutation studies were negative.
Three-monthly USG Doppler studies were done to
evaluate the TIPS patency. Three years after insertion of TIPS, USG
Doppler showed a shunt block (proximal end) following which hepatic
venogram and balloon angioplasty was done (gradient reduced from 23 mmHg
to 3 mmHg). Patient remained asymptomatic since then till the current
episode.
Investigations during the current episode revealed
hemoglobin: 2.8 g/dL, total leucocyte count: 3x10 9/L,
platelets count 120×109/L,
prothrombin time: 21.0 seconds, INR: 1.9, AST: 29 IU, ALT: 41 IU/L, TSB:
2.1mg/dl, GGT: 195U/L, Alkaline phosphatase: 301 IU/L. USG abdomen
showed a mass in the right lobe of liver with breach of liver capsule,
and hemorrhagic fluid in the peritoneum. CT abdomen showed a large
(9.2cm × 4.5cm) ill defined, mixed density lesion along segment 8, 5, 7
extending into the perihepatic space suggestive of ruptured HCC (Web
Fig. 1a). Tumour with characteristics of a malignancy was seen
in the lower lobe of the right lung along with right-sided pleural
effusion suggesting metastasis to lungs. Alphafeto protein (AFP) was
2380 µg/L. Hepatic arteriogram (Digital subtraction angiography) was
done which revealed a hyper vascular mass in the segment 7, segment 8 of
the right lobe of liver, showing neovascularization supplied by multiple
branches of the right hepatic artery, with contrast extravasation in
delayed phase. (Web Fig. 1b). Hepatic artery embolization
(super selective embolization with PROGREAT microcatherter and injecting
200-300 PVA particles) was done on emergency basis to control bleeding.
Shock was corrected with intravenous fluids and packed cell
transfusions.
A diagnosis of ruptured HCC in the setting of BCS was
made. Due to advanced nature of the illness, he was managed
conservatively. Palliative care in the form of recurrent blood
transfusions was given. He succumbed 7 months later due to progressive
abdominal distension, jaundice and anemia.
Discussion
Hepatocellular carcinoma is an uncommon complication
of BCS and has been only reported in adults. Our patient developed BCS
at the age of 4 years and with radiological intervention his clinical
course improved, but after 6 years he succumbed due to HCC.
Park, et al. [2] published their experience
with 67 patients of BCS from Korea, where 17 developed HCC on long term
follow up. Median time lag between diagnosis of BCS and HCC was 51
months [2].
BCS leads to chronic congestion of liver due to
impaired drainage. Long standing fibrosis and dysplastic regenerative
nodules may predispose to HCC [3]. Our case had underlying cirrhosis of
liver; nodules seen on screening USG were thought to be regenerative and
the possibility of development of malignancy was unforeseen. The size of
regenerative nodule larger than 4 cm may be helpful in suspecting HCC
but larger size with benign etiology has been reported [4,5]. Contrast
enhanced USG with a cut-off size of 4 cm may be helpful in screening
children with BCS [6].
The modality of treatment of spontaneous rupture of
HCC is not well-defined in children as only one case has been reported
[8]. As per adult studies, transarterial embolization/chemoembolization
and resection, if possible, are two approved treatment options for
ruptured HCC; though long term outcome remains poor [9]. Our patient
underwent hepatic artery embolization to control bleeding as a
palliative management in view of lung metastasis.
As more children with BCS are being diagnosed at an
early age and managed with radiological intervention, they need to be
screened for hepatocellular carcinoma on long term follow-up. Early
diagnosis may provide a chance for curative treatment.
Contributors: AS, VS and PR: drafted the case
report and initial manuscript; VS: contributed to discussion and
literature review; SM: gave intellectual input on radiological
intervention and diagnosis; AN: drafted and approved the final
manuscript and will act as a guarantor.
Funding: None; Competing interests: None
stated.
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