Relief of membranous obstruction of the inferior vena cava in a 5-year-old child

Relief of membranous obstruction of the inferior vena cava in a 5-year-old child

Volume 92 Number 6 December 1986 6 7 8 9 10 II Brief communications I I0I obstruction. J THORAC CARDlOVASC SURG 71:920-927, 1976 Misbach GA, ...

567KB Sizes 0 Downloads 0 Views

Volume 92 Number 6 December 1986

6

7

8

9

10

II

Brief communications

I I0I

obstruction. J THORAC CARDlOVASC SURG 71:920-927, 1976 Misbach GA, Turley K, Ullyot DJ, Ebert PA: Left ventricular outflow enlargement by Konno procedure. J THORAC CARDIOVASC SURG 84:696-703, 1982 Guyton RA, Davis SC, Michalik RE, Williams WH, Hatcher CR Jr: Right heart assist by intermittent abdominal compression after surgery for congenital heart disease. Circulation 72:Suppl 2:97-100, 1985 Bjork VO, Eklof 0, Wallgren C, Zetterqvist P: Successful surgical treatment of an aortico-left ventricular tunnel in a four-month-old infant. J THORAC CARDIOVASC SURG 78:35-38, 1979 Nichols GM, Lees MH, Henken DP, Sunderland CO, Starr A: Aortico-left ventricular tunnel. Recognition and repair in infancy. Chest 70:74-76, 1976 Mair DD, Fulton RE, McGoon DC: Successful surgical repair of aortico-left ventricular tunnel in an infant. Mayo Clin Proc 50:691-696, 1975 Giardina ACV, Levin AR, Engle MA: Aortico-left ventricular tunnel with natal cardiac failure. South Med J 70:1351-1354, 1977

Relief of membranous obstruction of the inferior vena cava in a 5-year-old child Antonio Amodeo, M.D., Roberto Di Donato, M.D., Antonio Dessanti, M.D., Guido Caccia, M.D., Dario Zaltron, M.D., Daniele Alberti, M.D., Francesco Callea, M.D., and Carlo Marcelletti, M.D., Rome, Italy From the Dipartimento Medico-Chirurgico di Cardiologia Pediatrica, Ospedale Bambino Gesu, Rome, and Divisione Chirurgia Pediatrica, Ospedale dei Bambini, and Primo Servizio di Anatomia Patologica, Ospedale Civile, Brescia, Italy.

Membranous obstruction of the inferior vena cava is a rare congenital anomaly that may present clinical features of Budd-Chiari syndrome caused by chronic obstruction of the hepatic drainage. We report membranous obstruction of the inferior vena cava in a 5-year-old boy. Surgical repair was prompted by signs and symptoms of hepatic venous obstruction. To our knowledge, this is the youngest patient successfully operated on for this anomaly.

Membranous obstruction of the inferior vena cava (MOVe) is a rare congenital anomaly almost exclusive Address for reprints: Carlo Marcelletti, M.D., Dipartimento MedicoChirurgico di Cardiologia Pediatrica, Ospedale Pediatrico Bambino Gesu, Piazza S. Onofrio 4, 00165 Roma, Itlay

Fig. 1. Longitudinal scan shows hyperechogenic transverse septum obstructing the inferior vena cava just before its outlet into the right atrium.

to Japan and South Africa. Isolated reports of the lesion have appeared from other parts of the world, hut surgical repair has not been undertaken in children younger than 9 years of age."? Case report. A 5-year-old boy was admitted to our department in April, 1985, with a diagnosis of MOVe. He had had lack of appetite, vague malaise, and abdominal distress for several months and at admission had hepatomegaly and a huge venous collateral circulation on the abdominal wall. All routine blood and liver function tests yielded normal results. The spleen was not enlarged. The echographic study demonstrated that the inferior vena cava and the common branch of the middle and left hepatic veins were obstructed by a membrane (Fig. I). Esophagogastric fiberoscopy revealed epicardial varices Grade II-III and features of hemorrhagic gastritis. The liver biopsy tissue showed centrilobular congestion and bridging fibrosis between centrilobular adjacent areas, causing a pattern of "reversed lobulation". Cavography demonstrated the presence of a diaphragm just above the entrance of the right hepatic vein completely obstructing the inferior vena cava (Fig. 2). Dilated varicose ascending lumbar veins reached the superior vena cava through the azygos and the hemiazygos systems. Adrenal veins and inferior phrenic veins participated in the collateral circulation (Fig. 2). Radiography of the right atrium confirmed the membranous obstruction of the inferior cavoatrial junction (Fig. 3).

1 10 2

Brief communications

The Journal of Thoracic and Cardiovascular Surgery

Fig. 3. Radiograph of right atrium shows complete obstruction of the inferior vena cava few millimeters from its atrial opening.

Fig. 2. Cavogram shows complete transverse obstruction of the inferior vena cava. Middle and left hepatic veins are not visible. Right hepatic vein is visualized by retrograde injection of contrast medium. Note presence of collateral circulation involving lumbar, adrenal, diaphragmatic, and azygos veins.

Surgical technique. Through a median sternotomy the ascending aorta and the superior vena cava were cannulated, and a moderately hypothermic (25 0 C) cardiopulmonary bypass was instituted. During cardioplegic arrest, an oblique right atriotomy toward the inferior vena cava was performed, and a small vent was inserted in the left atrium through a small defect created in the atrial septum. The eustachian valve was resected for better exposure. The inferior vena cava was completely atretic at the cavoatrial junction because of the presence of a fibrous web 3 mm thick. A small dimple was visible on the web surface, through which a stab incision was made. Blood from the inferior vena cava suddenly flooded the operative field, and 4 minutes of circulatory arrest were required for complete removal of the web. Completion of the operation was routine. The postoperative course was uneventful, and the patient left the hospital in good condition. An echographic examination performed 1 month after dismissal demonstrated disappearence of the web but persistence of a 3 mm ridge on the posterior wall (Fig. 4).

Discussion. MOVC often has clinical features of Budd-Chiari syndrome (ascites, esophageal varices) as a consequence of chronic obstruction of the hepatic venous drainage. Our patient had only hepatomegaly and episodes of abdominal pain, without other findings suggestive of vena caval involvement, such as protracted ankle edema, nor did he have ascites, frequently found in the MOVC literature. According to Sugiura's classification, our patient had MOVC type la. This classification is based on the involvement of the hepatic veins: type Ia, MOVC with a patent hepatic vein; type Ib, MOVC with an occluded hepatic vein; type II, an absent segment of inferior vena cava with an occluded hepatic vein; and type III, MOVC and stenosis of the inferior vena cava with a patent hepatic vein. In our patient the right hepatic vein was patent and drained to the inferior vena cava (Fig. 2), whereas the common trunk of the left and middle hepatic veins was occluded by fibrous material, as frequently described in type la MOVC. Histologic examination showed the web to be composed of connective tissue with elastic fibers covered by endothelium. These findings, together with a well-developedcollateral circulation, indicate that the lesion had been present for a long time and suggest congenital origin of the defect, which some authors have related to closure of the ductus venosus. In conclusion, early surgical relief of MOVC may be curative, as in our patient, because it prevents the consequences of long-term obstruction of the hepatic venous drainage.

Volume 92 Number 6 December 1986

Brief communications 1 1 0 3

Fig. 4. Postoperative longitudinal scan shows transverse septum in the inferior vena cava has disappeared. A 3 mm ridge persists on the posterior wall. Left and middle hepatic veins communicate with the inferior vena cava through disobstructed common trunk.

We thank Mrs. Sara Swartz for editing the manuscript.

2

3

4

5

6

7

8

9

REFERENCES Ono J, Sakoda K, Kawada T: Membranous obstruction of the inferior vena cava. Ann Surg 197:454-458, 1983 Simson IW: Membranous obstruction of the inferior vena cava and hepatocellular carcinoma in South Africa. Gastroenterology 82: 171-178, 1982 McDermott WV, Stone MD, Bothe A Jr, Trey C: BuddChiari syndrome. Historical and clinical review with an analysis of surgical corrective procedures. Am J Surg 147:463-467, 1984 Hirooka M, Kimura C: Membranous obstruction of the hepatic portion of the inferior vena cava. Arch Surg 100:656-663, 1970 Yamamoto S, Yokoyama Y, Takeshige K, Iwatsuki S: Budd-Chiari syndrome with obstruction of the inferior vena cava. Gastroenterology 54: 1070-1084, 1968 Rector WG Jr, Xu YH, Goldstein L, Peters RL, Reynolds TB: Membranous obstruction of the inferior vena cava in the United States. Medicine (Baltimore) 64: 134-43, 1985 Cabrera J, Bruguera M, Navarro F, Caralps JM, Pare C, Rodes J: Budd-Chiari syndrome due to a membranous obstruction of the inferior vena cava in a child. J Pediatr 96:435-437,1980 Michot F, Tubiana JM, Chermet J, Levy VG, Huguet C: Budd-Chiari syndrome due to membranous obstruction of the suprahepatic segment of the inferior vena cava. Is the membranotomy the best surgical procedure? Ann Chir 34:299-304, 1980 Jortner R, Shaklai M, Pinkhas J, Garty I, Levy M, de Vries A: Advisability of surgical treatment for chronic membranous obstruction of the hepatic portion of the inferior vena cava. Pahlavi Med J 7: 106-118, 1976

Direct internal mammary-vertebral artery anastomosis: An IS-year follow-up Norman H. Baker, M.D., H. Gene Ewy, M.D., Patrick J. Moore, M.D., John W. Thomas, M.D., Peter M. Sanfelippo, M.D., George J. Brahos, M.D., and Robert F. McVicker, M.D., Columbus, Ohio From the Ohio Heart and Thoracic Surgery Center, Inc., Columbus, Ohio.

Use of the internal mammary artery for vertebral artery bypass is described.

Atherosclerotic lesions of the vertebral artery, combined with other lesions of the aortic arch, are common findings in patients with cerebral vascular insufficiency. Unilateral disease of the vertebral artery is not usually a clinical problem. Direct operations on the vertebral artery are infrequent. Endarterectomy, patch grafts, vein bypass, and reimplantation have all been reported.!" It is uncommon for the vertebral artery to arise from the aortic arch. An unusual and interesting case of such an anomaly combined with severe occlusive disease is reported. Case report. A 39-year-old white woman was admitted in June 1968 with episodes of dizziness, numbness in the right Address for reprints: Norman H. Baker, M.D., Ohio Heart and Thoracic Surgery Center, Inc., 931 Chatham Lane, Columbus, Ohio 43221.