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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 4  |  Issue : 4  |  Page : 268-270

Left hepatectomy in a 3-month-old infant with congenital hepatoblastoma


Department of Pediatric Surgery and Pediatrics, B.J. Government Medical College, Pune, Maharashtra, India

Date of Web Publication16-Oct-2015

Correspondence Address:
Minakshi Bhosale
G/101, Sudarshan Apartments, Behind Spencer's Daily, Karvenagar, Pune - 411 052, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2249-4847.165694

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  Abstract 

Hepatoblastoma, the most common malignant liver tumor of infancy is known to present at a median age of 18 months. However, we happened to manage a case of a 3-month-old male infant with hepatoblastoma involving the left lobe of the liver. He underwent complete primary surgical excision of the tumor (left hepatectomy) and received six cycles of adjuvant chemotherapy thereafter. At a follow-up of 2 years, he is tumor-free as diagnosed by radiological investigations and follow-up serum alpha-fetoprotein (AFP) levels. Even on a thorough literature search, we did not find a similar report of an infant who has undergone elective primary surgical excision, except for the instances requiring emergency hepatectomy for hemoperitoneum resulting from perinatal tumor rupture. This is probably one of the youngest children in world literature with congenital hepatoblastoma, undergoing successful hepatectomy at presentation.

Keywords: Adjuvant chemotherapy, congenital hepatoblastoma, elective primary surgical excision, hepatectomy, infant


How to cite this article:
Bhosale M, Khadse S, Kulkarni R, Singh D. Left hepatectomy in a 3-month-old infant with congenital hepatoblastoma. J Clin Neonatol 2015;4:268-70

How to cite this URL:
Bhosale M, Khadse S, Kulkarni R, Singh D. Left hepatectomy in a 3-month-old infant with congenital hepatoblastoma. J Clin Neonatol [serial online] 2015 [cited 2019 Oct 22];4:268-70. Available from: http://www.jcnonweb.com/text.asp?2015/4/4/268/165694


  Introduction Top


Although uncommon in the perinatal period, hepatic tumors are associated with significant morbidity and mortality in affected patients.[1] Complete resection with adjuvant chemotherapy is the accepted modality of treatment for hepatoblastoma worldwide.[2] Though few discrete reports in the literature describe infants younger than 3 months having diagnosed to have hepatoblastoma, unlike our patient, they all have received up to four cycles of neoadjuvant chemotherapy before undergoing surgical excision.[3]


  Case Report Top


A 2½-month-old male infant, born at term by LSCS in view of fetal distress presented with recurrent vomiting of one-month duration. The antenatal period was uneventful, and no mass was detected on antenatal ultrasonography. On examination, a firm mass was palpable in right hypochondrium, 5 cm below the right costal margin. Abdominal ultrasound showed a 78 mm × 74 mm × 65 mm multiseptate mass with cystic spaces within the left lobe of the liver. Computed tomography demonstrated a heterogeneously enhancing mass lesion of 7.8 cm × 7.5 cm × 6.9 cm with multiple nonenhancing hypodense necrotic areas within [Figure 1]. There were no metastases. His hemoglobin was 6 gm% and received two rations of 50cc packed red blood cell transfusion preoperatively. Liver function tests (LFTs) were normal. β-human chorionic gonadotropin was normal. AFP was 99,971 IU/ml. Echocardiography was normal. He weighed 5 kg at the time of surgery.
Figure 1: Computed tomography showing mass lesion in left lobe of liver with multiple hypodense areas suggestive of tumor necrosis

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Upon complete workup, he was posted for exploratory laparotomy. The parents were counseled in detail, explained about the rarity of the condition, extent of the tumor, and need for hepatectomy-supramajor surgical procedure. They were also informed about the possible intra-operative complications including hemodynamic compromise from massive blood loss, leading to on-table cardiac arrest. A well-documented, written informed consent was obtained. Intravenous (IV) access was obtained using two wide-bore IV cannulas. Antibiotics were given at induction. Nasogastric tube was inserted and Foley catheterization was done. A bolster roll was placed to elevate the right costal margin. Abdomen was opened via Chevron incision, and the liver was taken down. On opening the abdomen, a large exophytic growth ~8 cm × 8 cm × 7 cm in size emanating from the left lobe of the liver was noticed [Figure 2]. It was tense cystic in consistency. We purposefully and stringently followed technical steps (intricacies) detailed below for carrying out the liver resection.
Figure 2: Large exophytic growth emanating from the inferior margin of left lobe of liver

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  • Dissection at the porta hepatis (hilar dissection) was startedfirst. Vascular control of the left hepatic duct, left branch of the portal vein, and the left hepatic artery was achieved using ligating sutures of 3-0/4-0 silk, before starting the actual tumor excision
  • Tumor excision was done very gently starting from below up. Going up from the inferior margin of liver, just lateral to the falciform ligament, gradual separation of the tumor from the liver could be achieved by use of finger dissection (finger fracture technique), and cauterization of small vessels running across the substance of liver by means of ball-point electrocautery with minimum settings (since we did not have more sophisticated equipments like cavitron ultrasonic surgical aspirator (CUSA) or the hand-held harmonic scalpel-stapler device available in our setting)
  • Once, about 60–70% of the tumor was excised, we concentrated on control of the hepatic veins. The left and middle hepatic veins were approached from the posterio-superior aspect of the liver. They were dissected off and divided between ligatures. On the body side, one transfixation and one simple ligation suture (using 5-0 prolene) was taken. It took about 30 min for isolation and complete control of the hepatic veins
  • To place the vascular ligatures correctly and tighten them optimally, avoiding undue pull/traction - which can be disastrous; taking complete control of the situation, all the vascular ligatures were placed and tied by the operating surgeon herself, rather than allotting the job to a junior assistant. This ensured gentle handling of the vessels and prevented sudden massive blood loss from rents/tears in the delicate vessels.


Complete gross resection was achieved. Operative time was 180 min. The excised tumor weighed 200 g. Hemostasis was re-confirmed. Tube drain was kept. Blood loss of ~75–100 cc was replaced on-table. The child was provided elective ventilatory support for about 12 h to prevent cardio-respiratory compromise from stresses of supramajor surgery in view of his tender age and limited blood volume reserve. The postoperative course was uneventful. IV antibiotics were given for 10 days. Breastfeeds were started on postoperative day 3. Drain was removed on postoperative day 6.

Histopathology of the tumor diagnosed hepatoblastoma of fetal type [Figure 3]. The resection margin was free from the tumor. As per SIOPEL I Protocol (European International Society of Pediatric Oncology Study Group), six cycles of cisplatin and doxorubicin were given at intervals of 3 weeks.[4] During thefirst two cycles, he had vomiting and developed alopecia, however, tolerated the chemotherapy very well. At present, at a follow-up of 2 years, the child is totally asymptomatic, has attained normal age-appropriate milestones. He is tumor-free as diagnosed by radiological investigations and follow-up AFP levels. His LFTs remain normal. He is on the regular follow-up to detect any tumor recurrence in the long run.
Figure 3: Histopathology slide showing tumor composed of round to polyhedral cells with large nuclei. Furthermore, seen is an occasional sinusoid (HP, view ×40)

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  Discussion Top


Liver resections in infants are often associated with significant morbidity and mortality. The reasons being - need for major liver resections in unprepared situations such as perinatal tumor rupture, massive blood loss from rupture of major blood vessels, especially the hepatic veins and/or the IVC (Inferior Vena Cava) causing sudden hemodynamic compromise, and complications arising because of massive blood transfusion or remotely from residual liver cell failure. Very few reports in world literature have described complete excision and successful salvage of infants as young as three months. Kusano et al. have reported a series of 7 children with hepatoblastoma. The youngest child in their series was 1-month-old (median age 30 months). However, before undergoing hepatectomy, all children received up to four cycles of neoadjuvant chemotherapy.[3] Lai et al. have reported successful hepatectomy in a neonate presenting with perinatal hemorrhage.[5] Our child was 3-month-old and weighed 5 kg at the time of surgery. Tumor volume was ~300–400cc. His estimated total blood volume was ~375cc. Hence, we had to be extremely cautious while doing the dissection to prevent any sudden massive blood loss. Sudden blood loss of even 100–150 cc also, would have put the child into irreversible hemodynamic compromise, leading to an on-table cardiac arrest. Our aim since the beginning was to avoid this unfortunate situation. Hence, we started off well-prepared, strictly following the technical steps/intricacies (detailed in case history section) to carry out the liver resection and providing elective postoperative ventilatory support.

From this experience, we conclude that hepatectomy can be successfully performed even in small infants, in settings with limited resources for performing major liver resections. Though sophisticated equipments like CUSA or the stapler device are desirable for parenchymal resections in small infants, one should not hesitate to perform major liver resections just because of nonavailability of these gazettes in an average pediatric surgical setup. This would prevent delays in treatment by curtailing the traveling time and waiting time at the well-established cancer centers. Optimal preoperative preparation, proper planning of the entire surgical exercise, gentle handling of tissues mainly the vessels during surgery, and elective ventilatory support are the keys to management.

Acknowledgments

We thank our anesthesia colleagues Dr. Anand Pande and Dr. Yogesh Gavali, for smooth intraoperative management of the case. We gratefully acknowledge Dr. Shona Nag, Consultant Oncologist for providing the chemotherapy regime and reassuring us from time to time during postoperative management (chemotherapy) of this child. We also thank Dr. Kalpana Kulkarni, Associate Professor - Pathology for providing the histopathology image for this report.

Financial support and sponsorship

Nil

Conflicts of interest

There are no conflict of interest.

 
  References Top

1.
Isaacs H Jr. Fetal and neonatal hepatic tumors. J Pediatr Surg 2007;42:1797-803.  Back to cited text no. 1
    
2.
Browne M, Sher D, Grant D, Deluca E, Alonso E, Whitington PF, et al. Survival after liver transplantation for hepatoblastoma: A 2-center experience. J Pediatr Surg 2008;43:1973-81.  Back to cited text no. 2
    
3.
Kusano T, Aoki H, Kinjo T, Miyazato H, Shimoji H, Isa T, et al. Successful resection for advanced hepatoblastoma, combined with perioperative chemotherapy. J Hepatobiliary Pancreat Surg 2000;7:410-6.  Back to cited text no. 3
    
4.
Russell HU, Pappo AS, Nuchtern JG, Kornguth DG, Wang LI. Solid tumors of childhood. In: De Vita VT, Lawrence TS, Rosenberg SA, editors. DeVita, Hellman, Rosenberg's Cancer: Principles and Practice of Oncology. 8th ed. Philadelphia: Wolters Kluwer, Lippincott, Williams and Wilkins; 2008. p. 2071-4.  Back to cited text no. 4
    
5.
Lai M, Burjonrappa S. Perinatal hemorrhage complicating neonatal hepatoblastoma: Case report. J Pediatr Surg 2012;47:e29-32.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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