Surgical treatment of giant cavernous hepatic haemangiomas

Surgical treatment of giant cavernous  hepatic haemangiomas

Ibrahim  Abdelkader Salama,a MD; Mohammed Hussein  Abdullah,b MD; Mohammed Houseni,c MD

a) Department of Hepatobilary Surgery, Menophyia University, Shebin Elkom, Egypt.

b) Department of Anesthesia, Menophyia University, Shebin Elkom, Egypt.

c) Department of Radiology, National Liver Institute, Menophyia University, Shebin

Elkom, Egypt.

Co"espondence:e-mail: ibrahim_salama@hotmail.com

Abstract

Background: Haemangiomas are the most common benign liver tumors. Treatment is indicated for   symptomatic tumors, rapid increase  in  size, rupture or  doubt in  diagnosis.

Objective:Evaluation the efficacy of surgical treatment of giant cavernous hepatic haemangioma in tertiary hepatobiliary center.

Patients and methods: Retrospective study of 34 patients with giant hepatic haemangioma operated on. The diagnosis was  proved  preoperatively in 27 patients  and  confirmed by histopathology postoperatively in all patients. The indication of surgery was abdominal pain with large sizes tumors, rapid growth, and spontaneous rupture with haemoperitoneum.Surgical treatment either liver resection or living liver transplantation.

Results:  33 cases (67.6%)  were females &11cases  (32.4%)  were males, median age 38.8 years. Haemangiomas were located in right lobe in 19 patients(55.9%), left lobe in 12 patients (35.4%) in both lobes in 2 patients (5.8%), scattered all over  both lobe in one patient(2.9%), solitary  in 27 cases (79.4%)  and multiple in 7 patients (20.6%).The diameter  was 8-27 em, mean 18.6 em in diameter. 33 patients had liver resection (29 elective resection and 4 emergent resection for rupture). One patient had haemangioma irresectable,· the living liver transplant was peiformed. No mortality occurred during 18 months follow up and complications occurred in 8 patients out of 34 patients.

Conclusion: Hepatic resection is an effective treatment option for giant cavernous hepatic haemangioma and in selected patients, living related liver transplantation may be the only therapeutic option.

Key Words: Liver neoplasms, haemangioma, hepatectomy.

Introduction:

Haemangiomas are  the  most  common

benign tumors of the liver.l The lesions, which may be single or multiple, are thought to be vascular malformations that enlarge by means of ectasia  rather  than neoplastic  growth.2

Those with a diameter greater than 4 em have been refereed to as giant haemangiomas which may present as a symptomatic abdominal mass.3  Although  most haemangiomas are asymptomatic  and may be managed safely with observation alone, larger lesions may produce a variety  of symptoms and signs,

including pain (abdominal, back or shoulder), fullness, early satiety, nausa, vomiting, and fever or more rarely haemangioma may rupture or be associated with Kasabach-Merritt Syndrome with thrombocytopenia secondary to platelet trapping within the haemangioma, or abscess formation.4

Strategies for the management of liver haemangioma have ranged from selective observation to a variety of radiological and surgical interventions.5 While there is general agreement that small asymptomatic lesions should be managed conservatively, the surgical

resection provides the only consistently effective method of treatment and is indicated for symptomatic lesions in patients with an acceptable surgical risk and lesions for which a diagnosis is equivocal despite appropriate preoperative evaluation.2 The unknown natural history and the possibility of complications of larger  or  giant  haemangiomas may  pose therapeutic dilemma.4

The aim of this present study is to evaluate

the efficacy of surgical treatment of giant cavernous haemangioma of the liver in our tertiary hepatobiliary center.

Patients and methods:

This is a retrospective study on 34 patients

with giant haemangiomas of the liver who underwent surgical operation at National liver Institute (Tertiary hepatobiliary center), Menophyia University between March 2005 to March 2010. The following investigations

were performed for all patients: laboratory tests (complete blood pictures, liver function tests, coagulation profiles, CA 19-9 and alpha­ fetoprotein) imaging studies includes abdominal ultrasonography, Triphasic Spiral Computed Tomography (C T), Figure(l), A,B and sometimes Magnetic Resonance Imaging (MRI) (in 8 patients only). The current MRI protocol for  haemangioma includes T2- weighted images with short and long echo times, multiphasic dynamic contrast-enhanced Tl-weighted images and delayed fat-saturated dynamic contrast-enhanced Tl-weighted images Figure(l), A,B.

The diagnosis was proved preoperatively

in  27 patients  by combination of  typical findings  in  imaging  studies and  clinical symptoms and signs. Post-operatively, the diagnosis was confirmed by gross  and histopathological examination in all patients who    underwent surgical  resection.

Figure(1):Non-enhanced CT imageof giant hepatic haemangioma, (A.)Right lobe, (B)Left lobe.

Figure(2): MRIimageof giant hepatic haemangioma, (A.)Right Lobe, (B)Left Lobe.

The indication for surgery was abdominal pain with large sized tumors, rapid growth or increase in size, and spontaneous rupture with haemoperitoneum and  shock (4  cases).

A  variety of  surgical procedures were

employed, designed to  minimize the unnecessary loss of normal liver  tissue. Surgical  treatment consisted of either  liver resection or living related liver transplantation.

The formal liver resection started in non­

urgent cases  with  abdominal exploration through a Chevron incision. The liver  was mobilized by  division of ligamentous attachments; care was taken to preserve  the left  triangular ligament in  case  of  right hepatectomy. Thehaemangioma was identified and a decision as to the type of resection was made, based on the location of the lesion(s) and size in relation to the volume of normal parenchyma. To  reduce bleeding in large resection, a Pringles' maneuver was performed

by  placing  a non  crushing vascular clamp across the porta hepatis. Inflow was occluded for no more than 20 minutes, at which point the clamp was released and the liver perfused for 5 minutes,ifnecessary, the clamp was then reapplied After identification and subsequent ligation and division of the portal structures at the hilum of the liver (hepatic  artery, portal veand hepatic duct) of segement, that had to  be   resected  was   done. Therafter, identification and ligation of the hepatic vein(s) was  performed, with  identification of  the transection plane guided by the discoloration of  the  devascularized parts of  the  liver. Transection of  the  liver  parenchyma was undertaken either by a Cavitaron Ultrasound Surgical Aspirator (CUSA) (Valley Lab, Boulder, Colo, USA) with bipolar diathermy or  Harmonic Scalpel (Harmonic Scalpel, Ethicon Endo-Surgery, CineOH, USA) Figure(3), A,B.

Figure (3): Intraoperative giant hepatic haemangioma, (A)Right Lobe, (B)Left Lobe.

After completion of the operative resection the haemostasis was done to raw surface  of the liver (by organ beam or diathermy cautry) and  small  bile  ducts  were  closed and  the abdomen closed with drain in most of cases.

Four patients underwent emergency operations because of  shock and haemoperitoneum due  to rupture of  giant haemangioma of the liver. One patient from those 4 emergency patients was referred from other hospital  after exploration (this patient experienced sudden onset of abdominal pain and tenderness in the right hypochondrium, there was no history of trauma, or significant disease in the  past. The  patient  underwent laparotomy owing to a misdiagnosis of rupture

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liver abscess;on exploration haem.operitoneum with active bleeding from the  liver haemangioma was  found. Due  to  lack  of experience, perihepatic packing was done with sponges and the patient  was referred  to our tertiary care  institute). After the  patient stabilized haemodynamically, the  patient underwent spiral CT  which revealed a peripherally enhancing lesion in right lobe of the liver located  in segments VI, Vll &VDI and  the  patient underwent formal right hepatectomy Figare(4), A,B.

In one case  (child  girl)  underwent total hepatectomy with left lateral segment living related liver 1ransplantation from her mother Figure(S), A, B.

Figure (4):A case of rupture cavernous right lobe hepatic haemangioma, (A)Intraoperative image, (B)Excised haemangioma.

Figure(5):A case of multiple cavernous haemangioma in 1.5 year-old girl, (A)Intraoperative image, (B)Excised liver.

Follow-up:

Postoperative examination in the patients consisted of  physical  examination, liver function  tests  and ultrasonography of the remnant liver.The patient follow up was at 6 months interval for 18 months after liver resection. The  patient   with  living  liver transplantation was seen each month according to the protocol of liver transplantation unit in the institute.

Statistical analysis:

Statistical analysis were performed using the Student-t test  for  continuous variables, continuous variables were  expressed as mean±SD. A P value of less than 0.05 was considered significant.

Results:

Patient demographics:

During  the  study  period. 34  patients

underwent surgical removal of giant cavernous hepatic haemangiomas,23 cases (67.6%) were females and 11cases (32.4%) were males with age range from1.5- 55 years and median 38.8 years.

Clinical findings:

The majority of patients were symptomatic (27 cases) complaining of upper abdominal pain, pressure symptoms due to increased size of the tumors such as fullness and dyspepsia. The remaining 7 patients were asymptomatic,

4 out of them presented with acute abdomen and   shock  as   the   first  presentation.

Physical examination revealed hepatomagly in 22 cases and hepatomagly with palpable liver mass in the remaining cases. Laboratory tests were normal in all but 5 patients (anemia was found in 3 patients and abnormal liver function test in 2 patients).

None of patients had  consumptive coagulopathy based on  the routine haematological parameters. Detailed coagulation parameters  were not assessed in the    absence  of   specific  symptoms.

Eleven patients had associated co-morbid conditions, including hypertension in 4 patients, diabetes in 3 patients, coronary artery diseases in 2 patients, gall stone and renal calculus in one each.

Characteristics of haemangiomas:

Giant Cavernous hepatic haemangiomas were located in the right lobe in 19 patients (55.9%), in the left lobe in 12 patients (35.4%), in both lobe in 2 patients (5.8%) and scattered all over both right and left lobe of the liver in one patient (2.9%) Haemangiomas were identified as solitary lesion in 27 cases (79.4%) and multiple in 7 patients (20.6%). The size

of detected haemangioma was ranging from 8 to 27 em in its largest diameter with a mean diameter of 18.6 em.

Operative procedures:

30 patients underwent elective surgery while in 4 patients an emergency partial hepatectomy had to be performed because  of a ruptured haemangioma (3 in the  Right  lobe  (Right hepatectomy was done) and one in the left lobe (left lateral segmentectomy was performed).

In one patient (1.5 years girl) had multiple haemangiomas in both lobes of the liver which made the patient unable to move or lie down without  pain  due  to a large  mass  in  upper abdomen. These multiple irresectable haemangiomas in both lobes  with  these symptoms were  indicated for  living liver transplant  (mother donated to her daughter).

Table (1): Types of liver resection performed among patients in the study group.

LRLTx: Living Related Liver Transplantation

The types  of liver resection performed among patients in the present study group are shown in Table(l).

Inmultiple haemangiomas only the lesions responsible  for the symptoms were resected. In all patients this appeared  to be the largest lesion. In one patient with giant haemangioma, another haemangioma in the ipsilateral lobe was resected with the giant haemangioma. In one  patient only  the  largest lesions were resected and the  additional smaller haemangiomas were located in the contralateral lobe and were left in-situ.

Pringle's inflow occlusion was performed during transection of the liver in 12 patients.

The  median  total  inflow  occlusion by the Pringles maneuver was 20 (ranging 10 to 37) minutes, median operative blood loss was one liter (ranging  from 500 ml to 2.5 liters) and the  mean  postoperative stay  was  7  days

{ranging 4 to 16 days).

Mortality and morbidity:

There were no early or late deaths in this study.

Complications occurred in 8 resected patients; minor biliary leakage (3 patients), pleural effusion (2 patients), wound infection

{2 patients) and one patient had postoperative bleeding treated conservatively.

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Postoperative  follow-up:

All patients were followed up for 18 months. No recurrence ofhaemangioma was observed. In the patient with haemangioma left in-situ, no notable changes in the size of the remaining haemangioma in the liver remnant was found during the follow up period. The pediatric patient,  who underwent living  related  liver transplantation recovered  uneventfully, and was followed up at outpatient clinic for liver transplantation at monthly interval without any significant complication.

Discussion:

Cavernous haemangiomas occur  at a prevalence of0.4% to 7.3% in autopsy series.l Cavernous haemangiomas are  the most common benign neoplasm of the liver and are often incidentally detected during abdominal imaging done for unrelated clinical indications.6

The lesions have female predominance with sex ratio 1.5:1 consistent with the present study. The female predominance (females  were 23 cases (67.6%), and male 11 cases (32.4%) with ratio 1.8:1). These lesions are seen in all age groups  but most frequently in middle age.7

Diagnosis is confirmed by results of noninvasive radiological studies. Dynamic contrast-enhanced magnetic resonance imaging is the best  study, as it will identify  smaller and/or multiple lesions.8

The  management of  haemangioma is controversial  and is intimately  related to the size, symptoms and associated co morbidities of the  patients who harbor these  benign tumors.9

Symptomatic haemangiomas are large and associated with a constellation of vague upper abdominal complaints including pain, masses, distention, early  satiety and  weight  loss.9

Symptomatic giant haemangiomas require some form of treatment as radiation, arterial embolization, surgical resection, or  liver transplantation.I0,11

The wide variability of symptoms in patients with haemangioma can be explained by known spontaneous regression, growth and rupture.12

In the present study, giant haemangiomas were identified as solitary lesion in 27 patients (79.4%)  and multiple  haemangiomas in the remaining 7 patients (20.6%). The size  of

detected  haemangioma  ranged between 8 to

27 em with a mean diameter of 18.6 em. The majority of patients were  symptomatic, complaining of upper abdominal pain, also symptoms due to fullness and dyspepsia. These results  compare  favorably with  Mergo  and

Ross.12

Indications for operation have traditionally been the  presence of  symptoms, the development of symptoms and  the need  to establish a definite diagnosis when radiological and histological studies were inconclusive.4

Inthe present study, the indications for surgical

operations were due to symptomatic large­ sized  tumors, rapid  increase in size and development of complication (spontaneous rupture).

Liver haemangiomas have  also  been resected because of a perceived risk  of spontaneous or trawnatic rupture and possibility of the Kasabach-Merritt syndrome, a consumption coagulapathy with low platelet counts and hypofibrinogenomia.4

Intraoperitoneal bleeding from  rupture, which can either occur spontaneously or due to trawna, is a rare life-threating complication.5

It occurs in about 4% of patients but is fatal

in 60-75%13. In a recent review of literature, the operative mortality rate  of ruptured haemangioma was reported  to be 36.4%.14

In the current study, 4 patients  out of 34 patients  with giant haemangioma presented with acute abdomen, shock and haemoperitoneum due to ruptured giant hepatic haemangiomas, 3 patients in right lobe (21, 32 and 27 em in diameter  respectively) and one in the left lobe measuring 18 em in diameter; all  managed by emergency liver  resection successively.

Warmann and associates15 do not consider

the risk of rupture by itself an indication  for resection. However, because of the high risk of a fatal  outcome after  rupture of such haemangiomas and in view of the low operative risks  of  partial liver  resection, Zeng  and associates16 believe that  the  operation is indicated even  for  asymptomatic giant haemangioma. This is particularly the case for patients with risk factors for rupture, such as superficial localization of the lesion in the liver haemangioma  of   large  size,  use    of

antithrombitic medication and the profession of the patient.I?

Specific feature of  cavernous liver haemangioma may be apparent with a variety of imaging techniques such as ultrasonography, dynamic contrast-enhanced CTand MRI, with ultrasonography. A typical haemangioma is characterized as a sharply marginated, lobulated, perdomintelly hyperechoic lesion. However,  utrasonography is an operator­ dependent technique and the acquisition  of satisfactory images  in  obese  patients is technically difficult.12

Multiphasic C T scanning should include arterial, portal venous phase and delayed imaging to ensure visualization of vascular structures. On non-enhanced C T  scans, haemangimas appeared hypoattenuating relative to the adjacent liver.During the arterial­ dominant phase, haemangiomas demonstrated a characteristic peripheral nodular and intense enhancement18, In patient with unclear diagnosis and in those with a known colorectal malignancy or  cirrhosis, MRI  was recommended because of its high specificity in   the   diagnosis  of   haemangioma.19

The results of MRI in present study, although obtained only in 8 patients with haemangioma, are in accordance with other previous report.20 The use of other invasive imaging methods, such as scientigraphy and technetium-99m labeled red blood cell scanning, has also  been  reported  for  the diagnosis of benign liver lesions especially haemangiomas.20 The risk of needle induced bleeding during ultrasographic or CT guided biopsy  in  benign  hpervascular tumors  is reported  to   be   low   {0.03-0.04%).17

In this study none of our patients had liver biopsy preoperatively.

There is considerable controversy regarding the ideal treatment of giant haemangiomas of the liver. Some authors, on the basis of long­ term follow up of such lesion, propose that most of these lesions do not require any treatment.21,22 Another has treated these lesions surgerically, citing symptoms of the patient, increase  in size and an occasional  case of rupture,  or   diagnostic  uncertainty.23

Several therapeutic modalities are available for the treatment of haemangioma such as:

steroids, interferon  alfa-2 a, embolization, radiotherapy and  surgery or liver transplantation. Resection of a hepatic haemangioma was first reported by Hermann Pfannestiel in 1898 and remains  the only consistently effective method oftreatment24.

Irradiation therapy has been reported to provide partial reduction in size and relief of symptoms, but has risks, including radiation hepatitis, vena-occlusive disease, and hepatoma4. Because long-term effects of radiation therapy on the liver and adjacent structures are well documented, recommended radiation therapy is only for patients who are unfit for or refuse surgery. Steroids have been used with some success in infants but their effectiveness in adults is not known.16 Hepatic artery embolization has been used rarely for poor operative candidates, with some success in   reducing  the   size   of   the   lesion.25

Arterial embolization should be considered for symptomatic patients in which resection is contraindicated or before operation in patients with a ruptured haemangioma, or even in elective cases, to reduce bleeding during resection.15

The  surgical  options  available for  the treatment of giant haemangioma are hepatic artery ligation and resection either  by enucleation or  by formal  liver  resection. Ligation of the hepatic artery can replace resectional treatment if the resection is considered too  difficult or  too  risky.26

Although most patients with haemangiomas of the liver may be safely followed up, some patients need surgery for symptoms{pain, etc), large or increasing size, uncertain diagnosis and complication {consumptive coagulapathy, rupture etc).

Yoon and associates27 reported that the commonest indication for surgery was the presences of symptoms (60%) other were an uncertain diagnosis (29%) and large or increasing size {11%). In another series from the US,3 the indications of surgery were pain or increase in size (68%), uncertain diagnosis

{25%) and rupture {7%).In present series, the indications were pain (44%), increasing in size

{23.5%) and uncertain diagnosis{20.5%) and rupture (12%).

Absolute  surgical  indications for hepatic haemangioma are spontaneous or traumatic rupture with haemoperitoneum, intratumoral bleeding, and  consumptive coagulapathy (Kasabach-Merritt syndrome). Persistent abdominal pain, obstructive jaundice, portal hypertension, superficial location  of tumors larger than five em with a risk of trauma, and an uncertain diagnosis are  relative indication.28,29

Rupture of  haemangioma with haemoperitoneum, is the most  dreaded complication  and often fatal if not promptly managed.30 The first case of spontaneous rupture of a hepatic haemangioma was described by Van Haefen in 1898 in an autopsy case.31 In 1961, Swell and Weiss32 reviewed

12 cases of  spontaneous rupture of haemangioma from literature and reported the mortality  rate   to  be   as   high  as  75%.

In the present study, 4 cases of rupture haemangioma received emergency liver resection to  control the  bleeding with  no mortality.

In the current study, formal liver resection was  chosen in 31 out of 34 patients, enucleatition  in 2 patients and living related liver  transplantation was  selected for  one patient: 1.5 years  old, the size  of multiple irresectable haemangiomas in both lobes was the indication for liver transplantation in this patient. No mortality and no recurrence was observed  and the morbidity  was comparable to that described by Imamura and associates.33

An interesting fmding in the present series was the absence  of growth  or complication from the haenmangiomas left in-situ  in the liver remnants  after resection,  during  an 18 months follow-up period. This underlines the possibility of a tailored treatment for patients with symptomatic multiple haemangiomas in the liver.

In conclusion, hepatic resection is a useful and effective  treatment for giant cavernous haemangioma of the liver. More  careful management to reduce intraopeative hemorrhage  is recommended  to increase the safety of surgery. In selected  patients living related liver transplantation may be the only therapeutic option.

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