LONG-TERM OUTCOME OF BILATERAL AUTOTRANSPLANTATION IN ACHILD WITH TAKAYASU’S ARTERITIS

JYOTI UPADHYAY, RICARDO RESTREPO, DIANE HEBERT, PETER CHAITAND GORDON A. MCLORIE

From the Divisions of Urology and Nephrology, and Departments of Pediatrics and Diagnostic Imaging, Hospital for Sick Children,University of Toronto, Toronto, Ontario, Canada

KEY WORDS: transplantation, autologous; hypertension, renovascular; Takayasu’s arteritis

In Takayasu’s arteritis, an inflammatory disease of thelarge arteries, involvement of the abdominal aorta and renalarteries results in stenotic, occlusive and aneurysmal le-sions.1 Predisposing factors are not well defined and severehypertension is the most common presenting sign. Previousstudies have demonstrated successful kidney autotransplan-tation in these patients for management of severe hyperten-sion.1–3 Bilateral autotransplantation may be required insome patients. We report a case of Takayasu’s arteritistreated with bilateral autotransplantation, and describelong-term followup and outcome of blood pressure, somaticgrowth and renal function.

CASE REPORT

A 4-year-old girl presented initially with severe hyperten-sion discovered during routine evaluation before ear surgery.An angiogram revealed stenosis of both renal arteries, andnarrowing of the abdominal aorta and superior mesentericartery (fig. 1). Takayasu’s arteritis was diagnosed, and treat-ment included multidrug antihypertensive therapy andshort-term steroid therapy.

At age 6 years, after failed renal angioplasty for poorlycontrolled hypertension, the patient underwent placement ofan intra-aortic stent and bilateral renal autotransplantation.The kidneys were autotransplanted to the ipsilateral com-mon iliac arteries and the left common iliac vein or vena cava.

Severe coagulopathy and intra-abdominal bleeding compli-cated the postoperative course. The patient received freshfrozen plasma, cryoprecipitate, and transfusions of blood andplatelets. No source of bleeding was identified at laparotomy.

At 10-year followup the patient has undergone no addi-tional surgery. Somatic growth has been normal (height 160cm., weight 53 kg. [65th percentile for age]). Pubertal devel-opment has progressed normally (Tanner stage 5). The pa-tient remains on long-term warfarin therapy. Blood pressureranges from 120/70 to 130/80 mm. Hg on a regimen of 10 mg.amlodipine daily.

A recent echocardiogram showed normal function withoutventricular hypertrophy. Serum creatinine was 40 �mol./l.(0.45 mg./dl., normal 0 to 106). Ultrasound demonstratedsymmetric adult sized kidneys (12.1 cm. and 12.4 cm.) withno hydronephrosis or stones. A dynamic nuclear renal studyrevealed normal symmetrical perfusion, no parenchymal de-fects and a glomerular filtration rate of 162 ml. per minuteper 1.73 m.2. Arteriogram confirmed an intact mid aorticstent, aortic caliber of 1.1 cm., patent iliac and femoral ves-sels, intact nonstenotic renal arteries and homogeneousnephrograms without avascular areas (fig. 2).

Accepted for publication June 7, 2002.

FIG. 1. Preoperative angiogram shows irregular narrowing of midabdominal aorta and concentric narrowing of proximal segment ofboth renal arteries.

FIG. 2. Post-autotransplant angiogram demonstrates minimalnarrowing of mid abdominal aorta. Reimplanted renal arteries,which are normal in caliber, are seen arising from common iliacarteries.

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DISCUSSION

Takayasu’s arteritis remains a challenge for clinicians. Ina prospective study of the natural history the estimated5-year survival was 83%, with a 10-year survival of 58% inthose with severe disease and a mortality rate as high as22%.1 Stenotic aortic/renal arterial lesions are the primaryetiological factors in severe hypertension. Surgical treatmentincludes arterial reconstruction, intra-arterial stents and re-nal autotransplantation. Hahn et al reported high failurerates with polytetrafluoroethylene grafting and intra-arterial stents, and advocated renal autotransplantation.1Studies have demonstrated improved outcomes in more re-cent cases, indicating the importance of surgical experience.2

Previously we reported the combined use of renal auto-transplantation and steroids as a feasible and successfulmodality for management of renovascular hypertension inpatients with Takayasu’s arteritis.3 Bilateral autotransplan-tation may be required in a small number of cases, adding tothe challenge of treatment, with intensive monitoring andmedical management required during the initial postopera-tive course.

The unique features of this case are the resumption ofnormal growth and pubertal development. It is generally

accepted that somatic growth may be altered in children withbilateral renal disease. Therefore, bilateral renal involve-ment in Takayasu’s arteritis with renal impairment can re-sult in a similar developmental delay. Another salient fea-ture of our case is that with long-term followup renal growthwas maintained bilaterally.

In summary, although technically challenging, the long-term outcome of bilateral autotransplantation in Takayasu’sarteritis can be favorable, with excellent control of hyperten-sion, normal somatic growth, pubertal development and pres-ervation of renal function in select patients.

REFERENCES

1. Hahn, D., Thomson, P. D., Kala, U., Beale, P. G. and Levin, S. E.:A review of Takayasu’s arteritis in children in Gauteng, SouthAfrica. Pediatr Nephrol, 12: 668, 1998

2. Martinez, A., Novick, A. C., Cunningham, R. and Goormastic,M.: Improved results of vascular reconstruction in pediatricand young adult patients with renovascular hypertension.J Urol, 144: 717, 1990

3. Merguerian, P. A., McLorie, G. A., Balfe, J. W., Khoury, A. E. andChurchill, B. M.: Renal autotransplantation in children: asuccessful treatment for renovascular hypertension. J Urol,144: 1443, 1990

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