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Case Reports Indian Pediatrics 1999;36: 1043-1045 |
Non Surgical Treatment of Complete Inferior Vena Cava Obstruction in a Child with Budd-Chiari Syndrome |
| Bishav Mohan, Shyam S. Kothari, Rajnish
and Juneja From the Department of Cardiology, All India
Institute of Medical Sciences, New Delhi 110 029, India. |
| Budd-Chiari syndrome (BCS) is character-ized by
structural and functional abnormalities of the liver caused by obstruction to the hepatic
venous blood(1). The etiology of BCS is diverse, but many patients with BCS from Asia have
been reported to have inferior vena cava (ivc) obstruction due to web or a membrane(2-4).
Hepatic venous obstruction commonly coexists and influences the clinical features.
Surgical treatment of such patients is difficult and is associated with high morbidity and
mortality(5,6). Balloon dilatation of IVC obstruction in adults with BCS has been well
reported(7,8). Rarely, complete obstruction of IVC has been treated by needle puncture of
IVC followed by balloon dilatation(7-9). To the best of our knowledge, such treatment for
a child with BCS has not been previously reported. We using an 8 mm and then a 18 mm
Mansfield balloon dilatation catheter. The size of the balloon used was based upon the
size of the distal IVC. Repeat pressure recordings were made in the right atrium, IVC
above and below the block. At the end of the procedure the right atrial and IVC pressure
were 8 and 13 mm Hg, respectively. The angiogram demonstrated patency of the IVC (Fig 3).
The patient experienced massive diuresis and was dischar-ged on anticoagulation therapy.
After 6 months of follow up patient had a patent IVC and marked improvement in symptoms. Discussion BCS is rare in children(2). It commonly results from vascular obstruction, from a web or a membrane in IVC(2-4). The genesis of such membrane is not clear but may be congenital. Hyperviscosity is a central feature of pathogenesis of BCS and may be responsible for the complete occlusion or partial obstruction report a 9 year old boy with BCS and complete IVC obstruction. Case Report A 9-year-old boy presented with insidious onset abdominal distention and pedal edema for the last 3 years. There was no history of jaundice, fever, drug ingestion or trauma. On examination, large non-tender hepatomegaly, splenomegaly and ascites were present. His biochemical investigations were: hemoglobin 11.4 g/dl, SGOT 56 IU, SGPT 28 IU, serum bilirubin 0.8 mg/dl, serum alkaline phosphatase 249 IU, total serum protein 7.2 g/dl and serum albumin 5.5 g/dl. An abdominal Doppler ultrasound showed complete obstruction of Inferior Vena Cava (IVC) and occlusion of left hepatic vein. On endoscopy, esophageal varices were present. BCS was diagnosed. Angiogram done through femoral route showed dilated IVC with complete blockade of intra hepatic portion of IVC and left hepatic vein (Fig.1). The IVC pressure below obstruction was 26 mm Hg. Right atrial mean pressure was 6 mm Hg.A catheter was placed from right internal jugular vein just above the total obstruction. Another angiogram in two views was done (Fig. 2). An attempt was made to perforate the obstruction with stiff end of a guidewire, but this was not possible. A stiff needle (Brockenbrough's needle) was introduced through a sheath and positioned in upper part of the distal IVC, and its position was checked by angiogram done already in two views. Using frozen frames on video replay as a guide, the obstructing segment was perforated by the Brockenbrough's needle and a sheath was advanced over it into the right atrium. An exchange guidewire was introduced and positioned in the superior vena cava.
Fig. 2. IVC angiogram (AP view) showing a catheter above the obstruction, delineating the anatomy (see text).
Fig. 3. Angiogram (AP view) after dilatation showing patent IVC with disappearance of collaterals.
After this, sequential dilatation was done from tissue tags or webs. In children, surgical management of BCS is difficult and rarely performed(10). The treatment of BCS with interventional procedure has been reported recently(3,4,11). The balloon dilatation of IVC is a safe and an effective procedure. However, a high rate of restenosis is noticed within six months, which requires multiple interventions(12). Simultane-ous dilatation of hepatic venous obstruction is required for relief. In our patient, the major right lobe hepatic vein was patent, therefore complete occlusion of left hepatic vein was not dealt with. In complete obstruction, interventionists have used septal puncture needle, biopsy needle and stiff end of guidewire(3,4,1). In our patient, complete occlusion of IVC was punctured by Brockenbrough's needle only after high quality angiogram in two views and a guide catheter from internal jugular vein was positioned just above the obstruction, clearly delineating the anatomy. Experience in adults with similar technique is cited in the literature(11). The clinical improvement was rewarding and a long term follow up is awaited. In conclusion, non surgical treatment of complete IVC obstruction is BCS in feasible and effective. References 1. Ludwig J, Hashimoto E, McGill DB, Van JA. Classification of hepatic venous outflow obstruction. Ambiguous terminology of the BCS. Mayo Clin Proc 1990; 65: 51-55. 2. Jang B, Dilawari, Bambery P, Chawla Y, Kaur U, Singh H, et al. Hepatic outflow obstruction (BCS) experience with 177 patients and a review of the literature. Medicine 1994; 73: 21-36. 3. Hirooka M, Kimure C. Membraneous obstruc-tion of the inferior vena cava. Surgical correlation and etiological study. Arch Surg 1970; 100: 656-663. 4. Nakamura T, Nakamura S, Aikawa T, Suzuki O, Onodera A, Karaji N, et al. Obstruction of inferior vena cava in the hepatic portion and the hepatic veins. Angiology 1968; 17: 479-498. 5. Kohli V, Pande GK, Dev V, Reddy KS, Kaul U, Nundy S. Management of hepatic vein outflow obstruction. 1993; 342: 718-722. 6. Millikan WJ Jr, Handersan JM, Potts, Warren WD. Approach to the spectrum of Budd Chiari Syndrome in which patient require portal decompression. Am J Surg 1985; 149: 167-176. 7. Griffith JF, Mahmoud EA, Scooper E, Ellias RJ W, Olliff SP. Radiological intervention in Budd Chiari Syndrome. Technique and outcome in 18 patients. Clin Radiol 1996; 51: 775-784. 8. Yang XL, Chen CR, Cheng TO. Non operative treatment of membraneous obstruction of the IVC by percutaneous transluminal balloon angioplasty. Am Heart Journal 1992; 124: 405-412. 9. Loya YS, Sharma S, Amrapurkar DN, Desai HG. Complete membranous obstruction of inferior vena cava: Case treated by balloon dilatation. Catheter Cardiovasc Diag 1989; 17: 164-167. 10. Odell JA, Rhodo H., Millar AJW, Hoffman H. Surgical repair in children with Budd Chiari Syndrome. J Thor Cariovasc Surg 1995; 110: 917-920. 11. Dev V, Kaul U, Sahni P, Sharma S. Balloon angioplasty for complete obstruction of IVC: Needle puncture followed by balloon dilatation. Catheter Cardiovas Diag 1992; 25: 320-322. 12. Verma S, Kaul U, Sharma S, Dev V, Wasir HS. Angioplasty for hepatic vein outflow obstruc-tion. Predictor of restenosis. Indian Heart J 1993; 45: 336. |
