1. Poornima Vanguri Franklin Lew
Pediatric surgery
Poornima Vanguri
Franklin
Lew

2. Cases (~2 weeks)
PGY-1
PGY-2
PGY-3
PGY-4
PGY-5
Total NA

3. Case Presentation - LA
Resident: Poornima Vanguri, MD Faculty: Patty Lange, MD – Pediatric Surgery Amit Sharma, MD – Transplant Surgery Brian Strife, MD – Interventional Radiology

4. Complication Date: 3/22/15 Procedure: Right hepatectomy
Faculty/Residents:
Lange/Sharma/Espino/Gupta
Complication: Common hepatic duct stricture requiring Roux en Y reconstruction
Assessment: Error in technique, Preventable Error, Error with significant deleterious effect on patient outcome
imaging

5. Case 16 month old Ethiopian male PMH: PSH:
premature delivery (28 wks), Extreme immaturity, grams
Hepatoblastoma of liver
Intracranial nontraumatic hemorrhage of fetus and newborn
Porencephalic cyst
CKD
PSH:
10/03/2014: Shunt construction
11/17/2014: Biopsy of liver, Insertion of Port-a-cath
Path - Hepatoblastoma, mixed epithelial type, with predominately fetal pattern
Porencephalic cyst - Neurological disorder of central nervous system characterized with cysts or cavities within cerebral hemisphere

11. MRI Abdomen 11/7/14:
Centered within the posterior right hepatic lobe, there is an approximately 3.4x2.5x3.9 cm lobulated T2 hyperintense mass
The medial margin of the mass is approximately 5-6 mm from the retrohepatic inferior vena cava The mass directly abuts a posterior branch of the right portal There is anomalous venous drainage of at least a portion of segment 6 via a separate hepatic vein, with a part of this vein being adjacent to the anterior inferomedial aspect of the The right hepatic vein extends adjacent to the anterosuperior aspect of the mass

12. Started chemo 11/21/14
Received 4 cycles of chemo but CT scan demonstrated enlarged mass and therefore resection was recommended
as per the intermediate risk arm of AHEP0731 WITHOUT doxorubicin due to his significant comorbidities

17. MRI Abdomen 3/5/15
There has been interval increase in size of known posterior superior segment right hepatic lobe hepatoblastoma. The mass now maximally measures about 6.0 cm craniocaudal by 4.2 cm mediolateral by 4.7 cm anteroposterior, previously measuring up to about 4.3 x 3.0 x 3.3 cm
The superior margin of the mass now nearly extends to the hepatic dome. The mass is now closer in proximity to right-sided intrahepatic vascular structures. The medial margin of the mass is approximately cm from the intrahepatic inferior vena cava, and also approximately cm from the bifurcation of the right and left portal veins

18. Hepatectomy 1. Total right lobectomy.
PROCEDURE PERFORMED 3/22/15:
1. Total right lobectomy.
2. Ultrasound guidance, intraoperative.
7.95 kg

19. Post-op Course
Monitored in PICU, pain control, advancing diet, clinically looked well
Gradually increasing bilirubin
Coags normal, transaminases grossly normal by POD2, blood glucose normal

20. Work-up
US 3/27/2015: some dilated intrahepatic bile ducts, patent and appropriate vasculature
MRCP 3/28/2015: Abnormal appearance of the intrahepatic biliary ducts within the left hepatic lobe, as described above, with two prominent fluid-filled structures measuring approximately 1.2x0.5 and 1.1x0.7 cm (possible bilomas) along the anticipated course of the intrahepatic biliary ducts. The proximal common bile duct is not visualized.
Called GI for possible ERCP who ultimately determined they did not have a small enough pediatric scope

21. PTC: Cholangiogram via left peripheral bile duct access showed complete central biliary reconstruction with no passage of contrast of bile into the bowel

23. OR 4/1/15 PROCEDURE PERFORMED: 1. Exploratory laparotomy.
2. Intraoperative cholangiogram.
3. Roux-en-Y hepaticojejunostomy with end-to-side jejunojejunostomy and end-to-side hepaticojejunostomy.
4. Placement of a biliary stent.
FINDINGS
Biliary stricture noted due to previous staple line from right hepatectomy. Patent intrahepatic bile ducts on cholangiogram. Adequate size of the remnant liver with no palpable abnormalities noted.

25. Analysis of Complication
CLAVIEN DINDO CLASSIFICATION
Grade III:
Requiring surgical, endoscopic or radiological intervention
Grade III-b:
intervention under general anesthesia
Grade IV:
Life-threatening complication (including CNS complications)‡ requiring IC/ICU-management
Grade IV-a: single organ dysfunction

26. Considerations that led to complication
Pre-op
Intra-op
Enlargement of mass despite chemo necessitating procedure
Proximity of mass to intrahepatic IVC and portal vein
Use of stapler close to a critical area and trying to
keep resection margin lateral to middle hepatic vein
Complication

27. Prevention of error in future
Consider using a clamp to control right hilar structures and oversewing stumps rather than using a stapler

28. Hepatoblastoma Tumor of liver precursor cells
Presents with abdominal mass usually within first 3 years of life, 50% are usually in right lobe
Associated with Beckwith-wiedemann Syndrome, FAP, Wilm’s tumor, extreme prematurity
Treatment:
Resection/Neoadjuvant chemotherapy prior to resection –
Depending on stage, can have survival 100% if completely removed
Transplantation – Up to 80% survival
Ashcraft’s Pediatric Surgery

29. Staging BASED ON PRE-OPERATIVE ASSESSMENT (PRETEXT)
Developed to compare chemotherapy efficacy and stage tumor prior to surgery
Help to time resection
Goal: to determine pre-operatively goal of achieving radical resection
For stage 2, after chemotherapy – goal is primary resection with >1cm margin
Liver divided into 4 vertical sections
Following Chemotherapy:
Primary resection for POSTTEXT 1, 2 with >1cm margin on V,P after 2 cycles
Primary resection for POSTTEXT 3 and no major venous invasion. (Margin <1cm ok)
Liver transplant referral after 2 cycles for PRETEXT 3 with venous invasion, multifocal PRETEXT3, and PRETEXT 4. Transplant within 4 wks of completion 4th cycle.

30. Predicting survival

31. Compared PRETEXT staging to Adult TNM staging (based on path) and Children’s Cancer Study Group/Pediatric Oncology Group Staging (based on completeness of resection)
Determined that Predictive value of survival
of PRETEXT is comparable to TNM staging
Reproducible form of staging

32. TNM STAGING
PRETEXT
CCSG/POG

33. Final pathology TUMOR:
Histologic Type: Hepatoblastoma, epithelial type, fetal pattern (mitotically active)
Tumor Size:
Greatest dimension cm
Additional Dimension cm
Additional Dimension cm
Resection Margin:
Uninvolved by invasive tumor
STAGE:
Staging (Children's Oncology Group): Stage I: Complete resection, margins grossly and microscopically negative for tumor

34. Complications after hepatectomy

36. Common complications Post-operative Fever and Infections
Venous Catheter
Infection
Pleural effusion
Ascites
Subphrenic infection
Incisional
UTI
Pulmonary
Post-operative Hemorrhage
Intraperitoneal
Coagulation Disorders
GI Tract Bleeding
Biliary Tract Hemorrhage
Treatment: Source control, drainage if symptomatic 
Treatment: Hemostatic agents in OR, correct coagulopathy, transfuse if needed, supportive measures 

37. Common complications Bile Leakage/Injury Liver failure
Truncation of distal bile duct in residual liver
Leakage at bile duct-intestinal anastomosis or incomplete suture around T-tubes
Injury of the bile duct from inappropriate surgical technique
Liver failure
Associated with active hepatitis, cirrhosis, limited residual liver tissue, massive intraoperative hemorrhage, mode and duration of hepatic portal vein occlusion, kind of anesthesia used and perioperative medication used
Treatment: Imaging, PTC, ERCP, re-operation 
Treatment: Monitor INR, hyperbilirubinemia, acidosis, blood glucose, ascites, mental status – management depends on severity - supportive vs. invasive 

38. European Journal of Pediatric Surgery, 2000

40. Management of bile duct injuries after hepatectomy - conclusions
Determine possible sources of biliary injury
Intraoperative injury, inadequate ligation or ischemia of a biliary stump, anatomical variations, inadequate exposure
Management
Due to risk of re-operative mortality, minimally invasive techniques should be considered
ERCP (limited availability in pediatric population)
PTC
In cases of complete obstruction, only option is operative reconstruction to prevent deleterious consequences of injury (liver failure)
Surgical Pitfalls: Prevention and Management