J Korean Med Sci.  2014 Mar;29(3):441-444. 10.3346/jkms.2014.29.3.441.

Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case

• Choi Y
• Yi NJ
• Ko JS
• Ko JM
• Jin US
• Kim HS
• Lee KH
• Cho TJ
• Suh SW
• Yoo T
• Lee KW
• Suh KS

• KMID: 1734935
• DOI: http://doi.org/10.3346/jkms.2014.29.3.441

Abstract

Osteogenesis imperfecta (OI) is a group of genetic disorders characterized by bone fragility and connective tissue manifestations. We report a successful liver transplantation (LT) in an 8-month-old boy with OI and cholestatic biliary cirrhosis. After 4 cycles of intravenous pamidronate, LT was performed under intravenous anesthesia using a left lateral section from his mother without mechanical retractors. The operation time was 420 min and estimated blood loss was 520 mL requiring one unit of RBC transfusion. He was discharged without surgical complications. Therefore, LT should be considered for patients with end stage liver disease and OI under organic multidisciplinary cooperation.

Keyword

Osteogenesis Imperfecta; Liver Transplantation; Cholestatic Liver Disease; Malignant Hyperthermia

MeSH Terms

Bone Density
Bone Density Conservation Agents/therapeutic use
Cholestasis, Intrahepatic/*diagnosis
Diphosphonates/therapeutic use
Fractures, Bone/drug therapy/etiology/radiography
Humans
Infant
*Liver Transplantation
Living Donors
Male
Osteogenesis Imperfecta/complications/*surgery
Bone Density Conservation Agents
Diphosphonates

Figure

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  Fig. 1 Multiple bone fractures. (A) Right femur fracture and callus formation and bowing of the bilateral tibia 1 month after birth. (B) Left humerus fracture at 2 months of age. (C) Callus formation in the left proximal femur at 3 months of age.

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  Fig. 2 Liver transplantation procedures. (A) Naïve liver: enlarged liver with cirrhosis. (B) Anhepatic phase: inferior vena cava (IVC) was totally clamped. Yellow arrows show vascular clamp that was applied to the suprahepatic IVC and the intrahepatic IVC and a blue arrow indicates the IVC. An arrow head points to the bulldog clamp occluding the portal vein. (C) Engrafted left lateral section after reperfusion. Bile duct was reconstructed with Roux-en-Y hepaticojejunostomy. The yellow arrow indicates jejunum Roux limb.

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  Fig. 3 Right humerus shaft was fractured at postoperative 2nd day. Arrows indicate fracture line.

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