Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration: A surgically treated case

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1 CASE REPORTS Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration: A surgically treated case Matthew J. Dougherty, MD, Keith D. Calligaro, MD, and Dominic A. DeLaurentis, MD, Philadelphia, Pa. Absence of the inferior vena cava (IVC) is an uncommon congenital abnormality. Symptoms of lower extremity venous insufficiency resulting from this anatomic abnormality have been reported only once in the English literature, and no experience with surgical treatment of this condition has been published. We report the case of an otherwise healthy 41-year-old man with an 18-month history of severe venous insufficiency involving the right leg manifested by extensive ulceration that did not respond to aggressive conservative treatment. Duplex findings were not suggestive of venous obstruction or reflux, but venography documented no filling of the common iliac vein or inferior vena cava, and outflow was via collaterals to the azygous and hemiazygous systems. Computed tomography demonstrated complete absence of the inferior vena cava with azygous continuation. A prosthetic bypass from the external iliac to the intrathoracic azygous vein was performed with complete symptomatic relief after a 30-month follow-up period. Venous bypass surgery may play a role in treatment of this rare cause of venous insufficiency. (J VASC SURG 1996;23:141-6.) CASE REPORT A previously healthy 41-year-old white male landscape worker presented with right leg edema, leg heaviness with walking, and recurrent pretibial ulceration over a 1-year period. No history of deep venous thrombosis was reported. He had been treated with compressive dressings followed by skin grafting with recurrence of ulceration in spite of vigorous compliance. He reported leg heaviness with walking but no pain. Physical examination revealed a mildly obese man with severe right leg edema, stasis dermatitis, and ulceration 6 cm in diameter at the previously grafted site. The left leg was normal, arterial pulses From the Section of Vascular Surgery, Pennsylvania Hospital, Thomas Jefferson University, Philadelphia, PA. Supported by a grant from the John F. Connelly Foundation, Philadelphia, Pa. Reprint requests: Matthew J. Dougherty, MD, 700 Spruce St., Suite 101, Philadelphia, PA Copyright by The Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter /96/$ /4/66808 Fig. 1. Ascending venogram shows apparent occlusion at common iliac vein with dilated internal iliac collaterals. 141

2 142 Dougherty, Calligaro, and DeLaurentis January 1996 Fig. 2. Computed tomography shows dilated azygous and hemiazygous veins (arrows). Note absence of intrahepatic cava. were normal, and abdominal examination was unremarkable. Venous duplex study results of both lower extremities were normal with no evidence of thrombus or reflux, nor was proximal obstructive physiologic condition noted. The proximal iliac veins and inferior vena cava (IVC) were not visualized. Ascending venography demonstrated apparent occlusion of the right common iliac vein (Fig. 1). Computed tomography ruled out a pelvic mass but showed complete absence of the inferior cava from the iliac level to the atrium with azygous continuation and collateralization via large epidural veins (Fig. 2). Venography from the left groin was performed and demonstrated excellent collateralization to the azygous system (Fig. 3). Supine femoral venous pressure on the asymptomatic left side was elevated at 17 mm Hg compared with 18 mm Hg on the right. Ambulatory venous pressure in the right foot dropped only 5 mm Hg with calf exercise. The patient was treated with fitted compression stockings, elevation, and wet-to-dry dressings for 12 weeks. The ulceration failed to heal, and he could not return to work. He then underwent venous bypass with a 13 mm ringed polytetrafluoroethylene graft from the right common iliac vein to the azygous vein in the chest via a right retroperitoneal approach and a separate seventh interspace thoracotomy. The graft was tunneled anterior to the right kidney and behind the diaphragm (Fig. 4). A 3 mm arteriovenous fistula was constructed with a venous tributary and ar- terial branch at the common femoral level on the right. The patient was treated with subcutaneous heparin and warfarin (Coumadin) and was discharged on coumadin. The leg edema subsided dramatically, and he was discharged on the eighth postoperative day. Magnetic resonance angiography demonstrated patency of the graft before discharge. At 1 month after the operation the leg edema had completely resolved and the ulceration had healed, although thrombosis had occurred in the adjunctive arteriovenous fistula. The patient remains without symptoms with a patent graft by duplex surveillance 30 months after surgery. DISCUSSION As generally defined, "absent IVC with azygous continuation" consists of an absent or atretic intrahepatic portion of the IVC with drainage via azygous and hemiazygous collaterals into the superior vena cava. The embryologic derivation of the inferior vena cava is complex, involving development and regression of segments of the paired postcardinal, subcardinal, and supracardinal veins and the hepatic veins at approximately the sixth week after conception 14 (Fig. 5). The intrahepatic IVC derives from hepatic sinusoids. Whereas the infrarenal IVC and the azygous/hemiazygous systems derive from supracardinal veins, the suprarenal, extrahepatic cava forms from the subcardinal vein. (Both subcardinal and supracardinal remnants contribute to the renal col-

3 Volume 23, Number 1 Dougherty, Calligaro, and DeLaurentis 143 Fig. 3. Left femoral venography shows left common iliac vein (A) draining to azygous and hemiazygous tributaries (B), which are massively dilated at level of diaphragm (C). lar.) The iliac veins, like the superior vena cava, derive from the posterior cardinal vein. Failure of formation of these anastomoses with persistence rather than regression of the supracardinal segments presumably leads to the clinical finding of a dilated azygous/hemiazygous "continuation" with congenital absence of the IVC. Absence of the intrahepatic inferior vena cava with azygous continuation occurs in 0.6% of patients investigated for congenital heart disease 46 and frequently coincides with situs inversus, asplenia, or polysplenism. It has been brought to attention mainly because of the associated anomalies and has been chiefly of interest as a diagnostic quandary or as it relates to surgical or heart catheterization routes. 4'6'7 More recently, absent IVC has also been recognized as a solitary, incidental finding in patients without symptoms, m,8-11 In addition to our patient,

4 144 Dougherty, Calligaro, and DeLaurentis January 1996 A -~ i. ~ External Iliac v v Vena cava f j~ -lepatic v v. t" I i t 'AzygousV.: \ ~-~ Hemi~]g~s V. \. I... J / i J I i Atretic Segment 13 m! PTFE graft B External C Iliac v v. Fig. 4. A, Normal venous anatomy; B, patient's anatomy; C, ilio-azygous graft.

5 Volume 23, Number 1 Dougherty, Calligaro, and DeLaurentis 145 Fig. 5. Embryologic derivation of inferior vena cava from 6 to 8 weeks of gestation to maturity. at least three reports have been published of absence of the infrarenal IVC along with the suprarenal segment. 1,11'a2 It is interesting that none of these patients has had the usual associated congenital anomalies in addition to this finding. It has been suggested that this finding could represent an acquired defect caused by perinatal renal vein thrombosis,13'14 although most researchers believe embryologic dysgenesis is the causey ~ Hemoptysis has been observed with this anomaly because of venous hypertension in the azygous and bronchial veno~s systems, 15,~6 but in most reports no symptoms have been directly attributed to absent IVC. Although obstruction of the IVC or iliac veins is well known to produce symptoms of venous insufficiency, only one other report of venous insufficiency related to this anomaly has been published. Presumably most patients have sufficient venous emptying through the azygous system and remain without symptoms. What led our patient to have systems at the age of 40 years is perplexing, because we could identify no thrombotic event. He did give a history of significant weight gain over recent years, which may have contributed. Undoubtedly the stasis ulceration was initiated by unnoticed local trauma, and elevated venous pressure impaired healing. It is important that color flow duplex findings in our patient were not suggestive of obstructive or reflux physiologic condition, which reinforces the still important role of ascending venography when clinical evidence suggests venous insufficiency. Absence of the IVC may be diagnosed by chest radiography, which will often show a paraspinous shadow adjoining the right hemidiaphragm. 12 The increased recognition of this anomaly corresponds to the prevalent use of computed tomography, which is the single best study to diagnose this anomaly. No reports of surgical correction of this defect have been previously reported, and although prosthetic venous bypass for other causes of iliocaval obstruction has been advocated, the need for a long graft into the thoracic cavity is highly unusual. Although this patient's contralateral egtrem'ity was asymptomatic, the elevated resting venous pressure on that side and the lack of a significant pressure gradient between the two limbs made a cross-femoral or pelvic bypass unappealing. At surgery, although the azygous vein was very large, we found it to be very

6 146 Dougherty, Calligaro, and DeLaurentis lanua~ 1996 thin-walled and fragile. Another option would have been direct anastomosis to the superior vena cava or the atrium. ~7 As advocated for other prosthetic venous bypasses, an arteriovenous fistula and oral anticoagulation were added. In spite of concerns about patency for a long prosthetic venous graft, the mid-term result has been good. In summary, recalcitrant venous ulceration should be investigated fully, including contrast venography when duplex findings are unrevealing. Congenital absence of the IVC can present with venous hypertension and symptoms of lower extremity venous insufficiency, and this anomaly can be successfully treated with prosthetic bypass if conservative measures fail. We thank Kevin Doerr, RVT, for his assistance with illustrations, and Nancy Park for assistance with manuscript preparation. REFERENCES 1. Debing E, Tielemans Y, Jolie E, Van den Brande P. Congenital absence of inferior vena cava. Eur J Vasc Surg 1993;7: Haswel! DM, Berrigan TJ. Anomalous inferior vena cava with accessory hemiazygous continuation. Radiol 1976; 119: Brem AS, O'Shea PA, Wesselhoeft CA. Absence of the inferior vena cava associated with an atrophic right kidney and hypertension. J Urol 1981;126: Heller RM, Dorst JP, James AE, Rowe RD. A usefhl sign in the recognition of azygous continuation of the inferior vena cava. Diag Radiol 1971;101: Anderson RC, Adams P, Burke B. Anomalous inferior vena cava with azygous continuation (infrahepatic interruption of the inferior vena cava). Report of 15 new cases. J Pediatr 1961;59: Folger GM. Plain film identification of failure of inferior vena caval-right atrial continuity. Cath Cardiovasc Diag 1977;3: Effler DB, Greer AE, Sifers EC. Anomaly of the vena cava inferior: report of fatality after ligation. JAMA 1951;146: Schultz CL, Morrison S, Bryan PJ. Azygous continuation of the inferior vena cava: demonstration by NMR imaging. J Comput Assist Tomogr 1984;8: Beedie RJ, Yeo W, Morcos SK. Congenital absence of the intrahepatic segment of the inferior vena cava with azygous continuation presenting as a mediastinal mass. Postgrad Med J 1989;65: Ginaldi S, Chuang VP, Wallace S. Absent hepatic segment of the inferior vena cava with azygous continuation. J Comput Assist Tomogr 1985;134: Knudtzon J, Gudmundson TE, Svane S. Congenital absence of the entire inferior vena cava. Acta Chir Scand 1986;152: Milner LB, Marchan R. Complete absence of the inferior vena cava presenting as a paraspinous mass. Thorax 1980;35: Mosli HA, Papoff WJ, Moors DE. Unusual clinical presentation of a patient with absent inferior vena cava. J Urol 1985;134: d'archambeau O, Verguts L, Myle J. Congenital absence of the inferior vena cava. Br J Radiol 1990;73: Ashour MH, Jain SK, Kattan KM, EI-Bakry AK, Khoshim M, Mesahel FM. Massive haemoptysis caused by congenital absence of a segment of inferior vena cava. Thorax 1993;48: Matsuoka T, Kimura F, Sugiyama K, Nagata N, Takatani O. Anomalous inferior vena cava with azygous continuation, dysgenesis of lung, and clinically suspected absence of left pericardium. Chest 1990;97: DeLaurentis DA, Tyson RR. Surgicalreconstructionofmajor veins. Circulation 1964;29(suppl): Submitted Feb. 7, 1995; accepted June 3, 1995.