Spontaneous Rupture of Liver Haemangioma — A Case Report & Review of Literature

On physical examination revealed a young female in mild painful distress. Her mucous membrane was pink and moist. She was warm to touch with a temperature of 38.4°C and her vitals were within normal limit. Abdominal examination revealed moderate tenderness in right upper quadrant with mild guarding but no rebound tenderness.An USS of abdomen revealed a normal gall bladder with no peri-cholecystic fluids or gall stones with a normal CBD. However, a 7.77 x 9.42 cm cystic mass was noted in right lobe of liver and there was no free fluid (Figure 1). A CXR revealed right basal consolidation. Her WBC was elevated 26.2 and she was also anaemic with an Hb of 11 mg/dl. Her renal function as well as electrolytes were within normal limit, however her liver enzymes were elevated AST-600, ALT-1900, ALP-238, GGT166 with normal serum bilirubin and amylase level.

After 24 hours her pain became severe and she became tachycardic with a pulse of 130 bpm with persistently elevated temperature (38.0°C). Her blood pressure and Spo2 was normal. There was decreased air entry at right lung base. Abdominal examination revealed marked tenderness with guarding but no rebound tenderness and there was sluggish bowel sound. Patient Hb had dropped from 10 gm/dl to 8 gm/dl. An Urgent CT scan of abdomen and pelvis with intravenous contrast revealed that the cyst (haemangioma) has ruptured with free fluid (blood) in Morrison pouch and in right Para-colic gutter ( Figure 2). Subsequently patient developed moderate respiratory distress and was reviewed by medicine specialist. A Spiral CT of the chest-revealed mild right pleural effusion with fluid in oblique fissure but no evidence of pulmonary embolism. An MRI of abdomen was also performed which reconfirm the ruptured hepatic haemangioma. Patient was admitted in HDU and was managed successfully and discharged home on day-12.Patient had a high ESR of 39 and her liver function test became normal. Her serum alpha fetoprotein, CEA, Ca 19-9, anti-nuclear DNA levels were within normal limit.

Discussion
Hemangioma is the most common benign tumour affecting the liver [1,2]. Hepatic haemangioma are mesenchymal in origin and usually are solitary. Haemangioma are composed of masses of blood vessels that are atypical or irregular in arrangement and size. Exact aetiology remains unknown [3][4][5].
Several pharmacologic agents have been postulated to promote tumour growth. Steroid therapy [6], oestrogen therapy, and pregnancy [7,8] can increase the size of an already existing hemangioma. Our patient was on inhalational steroid therapy for her asthmatic condition and was also on oral contraceptive pills for her polycystic ovarian disease.
Incidence rate 2% in USA & the prevalence rate at necropsy is 7.4%, usually see in multipara women, more female predominance and occur most frequently at age 30-50 years [1,2]. Our patient is 31years old and she has no children.
Hepatic haemangioma can occur as part of well-defined clinical syndromes. In Klippel-Trenaunay-Weber syndrome. In Kasabach- Routine laboratory tests -are usually normal. There could be thrombocytopenia or hypo-fibrinogenemia. Normal alpha-fetoprotein, CA 19-9, and carcinogenic embryonic antigen (CEA) levels bolster clinical suspicion of a benign hepatic mass lesion. Serum alpha fetoprotein, CA-19-9, CEA levels were normal in our patient.
Most hepatic haemangioma are small and asymptomatic at the time of diagnosis, and they are likely to remain that way. Hence long term follow up radiograph probably not necessary providing that no change in haemangioma size has occurred. Finally, patients with large haemangioma (i. e, >10 cm) may deserve long-term follow-up radiologic studies, perhaps annually, because of their probable increased risk of complications [1,6,8,19].
Treatment option includes surgical resection [8,17,27,[31][32][33] surgical enucleation, arterial embolization [7,8,15,18,27,34,35], radiofrequency ablation, hepatic irradiation, and orthotopic liver transplantation. However surgical resection and enucleation are the treatments of choice. It has been reported that patient with hemoperitoneum who underwent surgical intervention the mortality rate was 36.4% [1]. In rare instances, once patient remained hemodynamically stable can be managed conservatively like ours. Patient are usually followed up with serial ultrasound. Our patient was followed up with serial USS and after two years of follow up there was no increase in size and she remained asymptomatic throughout her follow up.

Conclusion
Although hepatic haemangioma is common, however spontaneous ruptures are very rare and associated with a very high mortality rate. High index of suspicion should be kept for prompt diagnosis of this rupture. Early intervention should be taken to prevent a fatal outcome.
Merritt syndrome, Osler-Rendu-Weber disease, Von Hippel -Lindau disease, multiple hepatic haemangioma [9] have been reported in patients with systemic lupus erythematous. Suzuki T, Tsuchiya N, Ito K. Multiple cavernous haemangioma of the liver in patients with systemic lupus erythematous.
Haemangioma are more common in the right lobe of the liver, usually small and asymptomatic can be larger and multiple usually presents as Right upper quadrant pain or fullness, may presents as an enlarged liver or the presence of an arterial bruit over the right upper quadrant. Rarely, haemangioma may present as a large abdominal mass or other atypical presentations like 1) cardiac failure from massive arterio-venous shunting [10], 2) jaundice from compression of the bile ducts, 3) gastrointestinal bleeding from hemobilia [11] and 4) fever of unknown origin (12).
An illness that resembles a systematic inflammatory process has been described with findings of fever, weight loss, anaemia, thrombocytosis, increased fibrinogen level, and elevated erythrocyte sedimentation rate. Our patient presents with RUQ pain & fever, with elevated WBC, ESR, low Hb and she was not icteric and auscultation revealed no arterial bruie.
Large tumours very rarely rupture spontaneously. Patients may present with signs of circulatory shock and hemo-peritoneum [12,13]. Our patient initially present as an un ruptured hepatic haemangioma as confirmed by initial USS (Figure 1) and spontaneously ruptured on day -1 of admission without any historyof traumaand confirmed radiologically by CT scan & MRI (Figures 2 and 3).