Infantile Budd Chiari and Response to Balloon Dilatation
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SCIENTIFIC LETTER Infantile Budd Chiari and Response to Balloon Dilatation Ira Shah & Amit Dey & Sushmita Bhatnagar & Gireesh Warawdekar Received: 26 August 2013 /Accepted: 5 May 2014 # Dr. K C Chaudhuri Foundation 2014 To the Editor: Angioplasty in infants with Budd-Chiari syn- drome (BCS) is difficult due to small vessels and has poor prognosis [1]. Two infants presented with acute onset ascites and hepatomegaly without splenomegaly. Both were con- firmed to have BCS on Magnetic resonance (MR) or comput- ed tomography (CT) angiography, were started on low mo- lecular weight (LMW) heparin and had successful outcome with angioplasty. Investigations are depicted in Table 1. Pa- tient 1 underwent venoplasty of middle hepatic vein (MHV) as right hepatic vein (RHV) and left hepatic vein (LHV) could not be cannulated. Currently patient 1 is 2 ½ y of age. Patient 2 underwent right and left hepatic venoplasty as MHV could not be cannulated. He was shifted to warfarin but INR remained below 1.5 despite dose escalation. Six months later, he again had narrowing in proximal portion of RHV. He underwent a repeat angiography that showed 90 % re-stenosis of RHV and 95 % re-stenosis of LHV with multiple intrahepatic collat- erals. Again left and right hepatic venoplasty was done and he was put on low molecular weight heparin and is doing well till last follow-up at 3 y of age. BCS is a rare, life-threatening disease caused by obstruction of hepatic venous outflow usually without significant liver pa- renchyma dysfunction. Doppler ultrasound by an experienced examiner, is the most effective and reliable diagnostic modal- ity. MR or CT imaging confirms the diagnosis, being most useful in the absence of an experienced doppler ultrasound examiner [2]. A step-wise approach using anticoagulation, angioplasty/thrombolysis, transjugular intrahepatic portosystemic shunting (TIPSS), and orthotopic liver trans- plantation (OLT) provides good long-term survival in adults [3]. In a study of 16 children with BCS with a median age of 22 mo, one of the hepatic vein patency could be established by TIPSS or angioplasty in 11 children [4]. In another study, out of 13 children with BCS, 6 underwent angioplasty and 3 underwent TIPSS [5]. However, intervention in infancy is difficult owing to the small blood vessel size and issue of re- stenosis. A strong suspicion of BCS in infants presenting with acute onset ascites should be considered. Early interven- tion with anticoagulation and angioplasty may lead to good prognosis. I. Shah : A. Dey : S. Bhatnagar Pediatric Liver Clinic, B. J. Wadia Hospital for Children, Mumbai, India G. Warawdekar Interventional Radiology, Holy Spirit Hospital, Mumbai, India I. Shah (*) 1/B Saguna, 271/B St Francis Road, Vile Parle (W), Mumbai 400056, India e-mail: [email protected] Indian J Pediatr DOI 10.1007/s12098-014-1488-2
Transcript of Infantile Budd Chiari and Response to Balloon Dilatation
SCIENTIFIC LETTER
Infantile Budd Chiari and Response to Balloon Dilatation
Ira Shah & Amit Dey & Sushmita Bhatnagar &
Gireesh Warawdekar
Received: 26 August 2013 /Accepted: 5 May 2014# Dr. K C Chaudhuri Foundation 2014
To the Editor: Angioplasty in infants with Budd-Chiari syn-drome (BCS) is difficult due to small vessels and has poorprognosis [1]. Two infants presented with acute onset ascitesand hepatomegaly without splenomegaly. Both were con-firmed to have BCS on Magnetic resonance (MR) or comput-ed tomography (CT) angiography, were started on low mo-lecular weight (LMW) heparin and had successful outcomewith angioplasty. Investigations are depicted in Table 1. Pa-tient 1 underwent venoplasty of middle hepatic vein (MHV)as right hepatic vein (RHV) and left hepatic vein (LHV) couldnot be cannulated. Currently patient 1 is 2 ½ y of age. Patient 2underwent right and left hepatic venoplasty as MHV could notbe cannulated. He was shifted to warfarin but INR remainedbelow 1.5 despite dose escalation. Six months later, he againhad narrowing in proximal portion of RHV. He underwent arepeat angiography that showed 90 % re-stenosis of RHV and95 % re-stenosis of LHV with multiple intrahepatic collat-erals. Again left and right hepatic venoplasty was done and hewas put on low molecular weight heparin and is doing well tilllast follow-up at 3 y of age.
BCS is a rare, life-threatening disease caused by obstruction ofhepatic venous outflow usually without significant liver pa-renchyma dysfunction. Doppler ultrasound by an experiencedexaminer, is the most effective and reliable diagnostic modal-ity. MR or CT imaging confirms the diagnosis, being mostuseful in the absence of an experienced doppler ultrasoundexaminer [2]. A step-wise approach using anticoagulation,angioplasty/thrombolysis, transjugular intrahepaticportosystemic shunting (TIPSS), and orthotopic liver trans-plantation (OLT) provides good long-term survival in adults[3]. In a study of 16 children with BCS with a median age of22mo, one of the hepatic vein patency could be established byTIPSS or angioplasty in 11 children [4]. In another study, outof 13 children with BCS, 6 underwent angioplasty and 3underwent TIPSS [5]. However, intervention in infancy isdifficult owing to the small blood vessel size and issue of re-stenosis. A strong suspicion of BCS in infants presenting withacute onset ascites should be considered. Early interven-tion with anticoagulation and angioplasty may lead togood prognosis.
I. Shah :A. Dey : S. BhatnagarPediatric Liver Clinic, B. J. Wadia Hospital for Children,Mumbai, India
G. WarawdekarInterventional Radiology, Holy Spirit Hospital, Mumbai, India
I. Shah (*)1/B Saguna, 271/B St Francis Road, Vile Parle (W),Mumbai 400056, Indiae-mail: [email protected]
Indian J PediatrDOI 10.1007/s12098-014-1488-2
Conflict of Interest None.
Source of Funding None.
References
1. Mehta P, Shah I, Bhatnagar S. Budd-Chiari disease in infancy: threecases. Paediatr Int Child Health. 2012;32:89–92.
2. Plessier A, Rautou PE, Valla DC. Management of hepatic vasculardiseases. J Hepatol. 2012;56:S25–38.
3. Seijo S, Plessier A, Hoekstra J, Dell’era A, Mandair D, Rifai K, et al.Good long-term outcome of Budd-Chiari syndrome with a step-wisemanagement. Hepatology. 2013;57:1962–8.
4. Nagral A, Hasija R, Marar S, Nabi F. Budd-Chiari syndrome inchildren: experience with therapeutic radiological intervention. JPediatr Gastroenterol Nutr. 2010;50:74–8.
5. Alam S, Khanna R, Mukund A. Clinical and prothrombotic profile ofhepatic vein outflow tract obstruction. Indian J Pediatr. 2013. doi:10.1007/s12098-013-1131-7.
Table 1 Investigations in both the patients
Patient 1 Patient 2
Pre treatment at 12 mo ofage
Post treatment at17 mo of age
Pre treatment at8 mo of age
Post treatment at17 mo of age
Gender Female Male
Perinatal events Prematurity, LBW with prolonged NICU stay Uneventful
Weight (kg) 6 6.4 7.4 10.4
Bilirubin (mg/dl) 1 0.7 0.6 0.57
SGOT (IU/L) 80 60 40 56
SGPT (IU/L) 54 45 91 42
Total Proteins (g/dl) 6.2 7 5.3 6.6
Albumin (g/dl) 3.9 4 3.9 4
INR 1.2 – 1.3 –
Functional Protein C (%) (Normal = 70–150 %) 80 29
Functional Proteins S (%) (Normal = 70–130 %) 100 97
Anti thrombin III activity (%) (Normal=80–130 %) 100 98
Factor V Leiden mutation Not detected Not detected
Ultrasound abdomen and Doppler Ascites and no thrombus. Hepatic veins and IVCseen
Thrombus in MHV with patent RHVandLHV, along with narrowed RHVandIVC with moderate ascites.
CT/MR angiography Non visualization of hepatic veins with diffuse longsegment narrowing of IVC in its distal intrahepaticcourse for 22 mm
Thrombosed MHV with focal ostialnarrowing of RHVand attenuatedhepatic IVC
Angiography 90 % and 95 % stenosis of ostium and proximalsegment of MHV with normal IVC.
70 % stenosis of RHVostium and 90 %stenosis of LHVostium. MHV couldnot be cannulated. IVC—normal
INR International normalized ratio; IVC Inferior vena cava; LBW Low birth weight; LHV Left hepatic vein;MHVMiddle hepatic vein; NICU Neonatalintensive care unit; SGOT Serum glutamic oxaloacetic transaminase; SGPT Serum glutamic-pyruvic transaminase
Indian J Pediatr
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