OBSTRUCTION OF THE INFERIOR VENA CAVA
Stephen Kaufman, M.D.
Dcpartnient of Pediatrics, Unicersity of California Medical Center, San Francisco
FORTY-THREE cases of obstruction of the tion is usually evident in the groin, abdomen
inferior vena cava in childhood have and flank. Albuminuria is rare, edema of been found in a search of the English lit- the abdominal wall is unusual, and edema erature,l-l5 more than one-half of them col- of the legs is usually absent unless there is lected by Pleasants in 1 9 1 1 ' ~ ~ (Table I). Of concomitant obstruction of the iliac vein. the total cases, 13 survived the disease; 9 Acute obstruction of the middle third of of these were congenital in
rigi in.'^^"*'^^''the inferior vena cava is invariably fatal. Only three had demonstration of the ob- If it develops insidiously, marked edema struction by venography;13-l5 of these, two ensues, and thereafter superficial collateral were congenital and did not reveal any circulation gradually becomes evident. deep collateral circulation. The most com- Albuminuria and other disturbances of mon factors in the etiology were infection, renal function are frequently but not in- congenital obliteration of the inferior vena variably observed.
cava with anomalous drainage, and throm- Sudden blockage of the upper third is bus or invasion of the vessel by a malignant also fatal. In obstruction which develops tumor (Table 11). Sequelae varied with the gradually, however, there is abdominal particular etiology of the obstruction, the pain, rapidly progressing and tender hepa- acuteness of onset, the location, and the tomegaly, vomiting and often diarrhea. rapidity of its progression (Table 111). Ascites and jaundice are common and liver
CLINICAL MANIFESTATIONS function is often impaired. If the hepatic vein is also obstructed, symptoms of portal The most common symptoms of this con- hypertension supervene.
dition are found to be inconstant pain gen- ~h~ following case is
reportedbecause erally referred to the h m b a r area, edema it is typical of the syndrome of obsh-uction of the legs when
theveins are of the lower third of the inferior vena cava obstructed,
presence ofand the iliac vein, It is the fourth reported collateral c i r ~ u l a t i o n . ~ ~ ~ ~ A r t e s , jaundice, case of acquired obshuction of the inferior hemorrhoids and s ~ l e n o m e g a * ~ are uncOm- vena cava without progressive symptoms mon, and dilated superficial veins do not terminated
bydeath, and the deep col- appear other veins are
ob-lateral circulation outlined by means of ~ t r u c t e d . l - ~ * * Marked albuminuria or oligu- venography is clearly demonstrated, ria which was not present previously may
suggest the progression of the process to CASE REPORT
thevein. *lmost R.G., a 7-year-old white male, was brought anuria may supervene before adequate col- to the Herbert C. Moffitt Hospital on Febru- lateral circulatory adjustment takes place.*l ary 19, 1959, because of increasing prominence When the lower third of the inferior of the superficial abdominal veins (Figs. 1A vena cava is obstructed, collateral circula- and B). He was first seen by the referring
(Accepted September 28, 1959; submitted July 6.)
ADDRESS: c/o Dr. E. B. Shaw, Department of Pediatrics, University of California Medical Center, San Francisco 22, California.
I).+T.~ O X VY
c.4~~-'3OF OBSTRVCTION OF TIIE ~ N F E R I O R VENA CAVA OBTAIN!:D FHO\I T H E I,IT!:HITI.RE
-- -- - - - - - - -
&f.Age Sez Method of
No. (Y) Obstruction Probable Etiology Duration Outc7me*
15 IS 2 da 11 13 7 K 15 8 13 1 I E 6 12 i e 4 6
e0 l e 10 14 10 34 81 15
A1 51 5 1 4 43 7 10 1T'f Newborn Newborn Thrombosis Thrombosis I Thrombosis Fibrosis Thrombosis Thrombosis Thrombosis Fibrosis Thrombosis Thrombosis Xew growth Pressure Pressure (?) Pressure Thrombosis Invasion Invasion Pressure Thrombosis Thrombosis Thrombosis Thrombosis Fibrosis Thrombosis Thrombosis Thrombosis Thrombosis Thrombosis Congenital Thrombosis Thrombosis Congenital Congenital Congenital Congenital Congenital Congenital Congenital Congenital Congenital Thrombosis Congenital Congenital
Typhoid fever :?) Scarlet fever
Cachexia, tuberculosis Diarrhea, tuberculosis of pelvis Tuberculosis of pelvis Anemia, chloroma (?)
Tuberculosis of hip; postoperative Endothelioma, wall of inferior vena cava Adhesions, syphilis (?)
Echinococcus cyst Carcinoma right kidney Adenocarcinoma right kidney Myxosarcoma, left kidney Mixed tumor, right kidney Sarcoma, left adrenal Pelvic carcinoma Pelvic carcinoma Tumor, malignant Congenital (?) Chiari syndrome (?) Srarlet fever a Thrombophle1)itis migrans Diarrhea Congenital Gastroenteritis Diarrhea
Anomalous inferior vena cava Anomalous inferior vena cava Anomalous inferior vena cava Anomalous inferior vena cava Anomalous inferior vena cava Anomalous inferior vena cavlt Anomalous inferior vena cava Anomalous inferior vena cava Eustachian valve (?)
Thrombophlebitis & cellulitis of leg Maldevelopment rt. superior and pos-
terior cava vein
h1aldevelopment rt, superior and pos- terior cava vein
5 yr I,
1 day I)
few days I) 3 m-k I )
4 wk 1) 3 days D several wk D
3 wk D
few days D
17 mo 1,
8 mo D
few wk D
6 mo D
15 days D
8 days D
5 ak I)
9 mo I)
10 yr I, '2'2 mo I) 5 mo I )
1 rno 1)
18 mo I,
few days I)
6 wk I)
17 mo D
5 Yr D
3 mo I,
1 Yr I,
4 Yr I,
10 mo L
5 yr L
* D=Died, L=Living.
physician on July 12, 1958 with the complaint History
( ' ~ l ' h l C S Ok. ~ ) R S T I I I ' ( T I ~ N Ok. T I I K ~ ~ ~ ~ 1 1 1 0 1 1 \'EN.\ ('.\vA I N ('IIII.DIIEN A N D :\DIILTS*
, I l~ron~l)osis
i \ r i s i ~ ~ p \vitl~irr the i ~ ~ f c r i o r vewl c:tva or its br;cncl~cs 111fec.tions diseases
lstcnsion from localizctl foci of clisc;~se 1Jnkno\\-rr origin
Se\v gro\vtlrs from the wall of the infcrior vcna citva Invasion of the lunrer~ l ~ y a new gro\vtl~
Kinking of inferior vena cava Congenital
Anomalous (absence of) inferior I~CII:I cava10.14J5-17 Eustaclcian valve'a-'g
Involvc~cccnt of inferior vcna cnv:t :~ntl superior vcna rava
Thrombophlebitis migrans" Thron~botic t h r o m l ~ o c ~ t o p e n i f l
Adapted and extended from Pleasants?
-- - ~
Nu~ul)er of cases 43
Sex JIale Fcncalc Unkno\vn
I h d 31 Living 10-
Tl~rombus 3 (secondary to infection)
Pressure 1 (secondary to congenital syphilis)
Infection 15 Congenital IS
intermittent, and lasted 4 to 6 hours at a time. The bowel sounds disappeared, and guarding and rebound tenderness in all quadrants be- came marked. Urinalysis, complete blood count, guiaic test for blood in the stool, and roentgenograms of the chest and pelvis were normal. Roentgenograms of the abdomen were interpreted as showing early mechanical bowel obstruction.
A diagnosis of acute peritonitis was made and an exploratory laparotomy was performed through a right paramedian incision. A gen- eralized beefy red injection of the parietal tissues with retroperitoneal inflammation and multiple serofibrinous adhesions were present between all the intra-abdominal structures; the mesenteric nodes were slightly enlarged but not acutely inflamed. Inspection of the right iliopsoas muscle, the right lateral gutter, and the entire large and small bowel revealed no pathology. The iliac veins appeared normal and no large collateral veins were noted. A small amount of free serous fluid was seen, cultured, and found to be sterile. Histologic examination of the appendix was reported to reveal the presence of Enterobius vermicularis in an otherwise normal appendix. The imme- diate postoperative course was uncomplicated. During convalescence, edema of both thighs, swelling of the abdomen and difficulty in ex- tending the right thigh were noted. These signs and symptoms gradually subsided with- out treatment during the succeeding 4 months. In November, 1958, the first episode of mod- erately severe, bilateral flank pain occurred, associated with a recurrence of edema of the thighs, and lasted 3 days. In December, and again in February, 1959, flank pain on the left occurred. These attacks subsided spontaneous- ly and were not associated with symptoms referable to the urinary tract.
Superficial abdominal venous distension was first noted in January, 1959. This progressed rapidly and involved the thighs and lower thorax by late February, a t which time he was admitted to the Herbert C. Moffitt Hos- pital.
On physical examination a t the time of ad- mission pulse was 88, temperature 37" C, respirations 18/min, blood pressure 100/60 mm H g in the right arm, supine, 122/84 mm H g in the left thigh, prone. Height and weight
were at the 90th percentile and general health was excellent. The remainder of the physical examination was unremarkable except for the liver which was palpated 1 cm below the right costal margin and was nontender; the lower extremities which were disproportionately large, although no pitting edema could be demonstrated; and the pattern of large, dis- tended superficial veins that transversed the anterolateral aspect of the abdomen from the upper thigh to the lower thorax, with a flow of blood cephalad. There was no evidence of venous distension of the neck, varicoceles, hemorrhoids or a caput medusae.
Hemoglobin was 12 gm/100 ml, leukocyte count, 10,000/mm3 with a normal differential count. Urinalysis and Addis count were nor- mal; creatinine clearance was 102%, corrected. Nonprotein nitrogen in the blood was 33 mg/100 ml; total protein in the serum was 7.4 gm/100 ml with albumin 4.9, globulin 2.5 gm/100 ml. A bromsulphalein dye test re- vealed 1% retention in 45 minutes. Thymol turbidity was 3 units; cephalin flocculation was 2 + in 48 hours. Prothrombin time was 68% of normal. A culture of urine during mid- stream voiding had less than 100 colonies of Streptococcus viridans per milliliter. An esoph- agram showed no evidence of varices and intravenous pyelography showed prompt fill- ing, good contrast, and normal renal architec- ture. Roentgenograms of the chest revealed no abnormalities of the heart and lungs, al- though there was moderate dilatation of the azygous vein above the level of the superior vena cava.
OBSTRUCTION O F VENA CAVA
Frc:. 2. (..\, lcft) \'enogram. (B, right) Ilrawing of the collateral circulation. The extemal but not the c;~ninion iliac vein is visil~lr bilaterally. Three primary collateral circulatory chilnnels are identifiable:
1) the supc.rficia1 epignstric vein which is joined to the lateral thoracic vein by several small channels
;ind then drains into the superior vena cava via the axillary venous channels, and also connects with the intercostal vein which, i n turn, drains the azygos and hemiazygos veins; 2) the inferior epigastric
vein which rmpties into thc interaal niiininlary vein and connects the femoral vein with the superior
vcnu cilva, also with a connection between the superior and inferior epigastric veins; 3) the sacral venous plexus which extends from the external iliac to the ascending lumbar vein which, in turn,
coni~nunicatcs with the vertr11r;ll venous plexus and empties into the azygos and hemiazygos veins.
Examination in Slay, 1959, revealed that the thickening of the thighs was less marked, and
there was a slight decrease in the collateral
venous distension. The spleen was palpable
one centimeter belo\v the left costal margin; the liver was no longer palpable. Urinalysis \tl;is normal and the child was asymptomatic. N o additional procedures seemed warra~~ted at t11;it time, and the patient will be followed at regular intervals.
The present case is that of a boy with a thrombosis of the inferior vena cava of
unknown etiology. Because of the acute
onset of symptomatology at 6% years of age, and subsequent development of super- ficial and deep collateral circulation, it is unlikely that this represents an anomalous inferior vena cava of congenital origin. I n the absence of constitutional signs and
10months of observation,
in association with the operative findings, malignancy and blood dyscrasias are also unlikely.
As edema and swelling of the lower ex- tremities were present for several months
postoperatively, as superficial collateral cir- culation had not appeared early, and renal impairment is not evident, it is concluded that the original obstruction occurred be- low the renal veins and above the common iliac vein. The subsequent development of
superficial collateral circulation 6 months
postoperatively can b e explained
suming that the interference with venous
flow was the result of the thrombosis ex- tending caudally to involve the common
T h e venograms demonstrated the lower level of the obstruction and the coHateral circulation which developed but did not indicate the extent of t h e thrombosis or the patency of the renal and hepatic veins. Liddle and Waddel113 demonstrated by venography the utilization of the vertebral system, the intercostal veins, and the azy- gous vein in the collateral circulation in a 7-year-old boy, and interpreted the lack of contrast media in the inferior vena cava below the diaphragm as evidence that the renal veins must b e obstructed. T h e exten- sion of a thrombus in the inferior vena cava t o obstruct the renal vein cannot b e diag- nosed b y injecting dye below the level of obstruction, because t h e absence of con- trast media in the middle third of the in- ferior vena cava may b e d u e to the large volume of blood entering from the renal vein rather than d u e t o obstruction.
In t h e absence of signs and symptoms of renal or hepatic dysfunction in the present case, it is assumed that the venous drainage of these organs is intact. Because of the paucity of diagnosed cases of obstruction of the inferior vena cava
inchildhood, it is difficult to assess the prognosis of this pa- tient. Superficial t h r o m b o p h l e b i t i ~ ~ ~ and renal or hepatic dysfunction due to exten- sion of the thrombus are possible serious complications. I n the absence of these, surgical intervention is not indicated, as it may hasten a terminal t h r o m b o s i ~ . ~ ~
1. Pleasants, J. H.: Obstructions of the in- ferior vena cava. Bull. Johns Hopkins Hosp., 203292, 1909.
2. Idem: Obstructions of the inferior vena cava with a report of eighteen cases. Johns Hopkins Hosp. Rep., 16:363,1911. 3. Dodd, K., Johansmann, R. J., and Rapo- port, S.: Thrombosis of the vena cava and hepatic veins (Chiari's syndrome) in a patient with nephrosis. Report of a case and observation on serum phos- phatase and prothrombin time of plas- ma. Am. J. Dis. Child., 76:316, 1948. 4. Bunn, W. H.: Report of a case of throm-
bosis of the inferior vena cava and ex- tensive skin necrosis following scarlet
fever; recovery. Ohio M. J., 29:485, 1933.
5. Derow, H. A., Schlesinger, M. J., and Savitz, H. A.: Chronic progressive occlu- sion of the inferior vena cava and the renal and portal veins, with the clinical picture of the nephrotic syndrome. Re- port of a case, with review of litera- ture. Arch. Int. Med., 63:626, 1939. 6. Birnberg, V. J., and Hansen, A. E.:
Thrombophlebitis migrans. J. Pediat., 21:775, 1942.
7. Angelman, H., Hall, E. G., and Spencer, R.: The syndrome of obstruction of the inferior vena cava in childhood. Brit. M. J., 2:752, 1950.
8. Feriozi, D., Rice, E. C., Burdick, W. F., and Troendle, F. J.: Thrombosis of the inferior vena cava and renal veins with hemorrhagic renal infarction in infancy. J. Pediat., 38:235, 1951.
9. Stevens, R . C., and Tomsykoski, A. J.: Thrombosis of the inferior vena cava and renal veins with hemorrhagic renal infarction. J. Urol., 72: 120, 1954. 10. Downing, D. F.: Absence of the inferior
vena cava. PEDIATRICS, 12:675, 1953. 11. Anderson, R. C . , Heilig, W., Novick, R.,
and Jarvis, C.: Anomalous inferior vena cava with azygous drainage: so-called absence of the inferior vena cava. Am. Heart J., 49:318, 1955.
12. Kibel, M. A., and Marsden, H. B.: In- ferior vena caval and hepatic vein thrombosis; the Chiari syndrome in childhood. Arch. Dis. Childhood. 31: 225, 1956.
13. Liddle, W. D., Tr., and Waddell, W. W., Jr.: ~hrombosk bf the inferior vena cava in childhood; report of a case. Virginia M. Month., 84:354, 1957.
14. Edwards, W. M., and Bennett, C. E.: Anomaly of the inferior vena cava; re- port of case diagnosed in neonatal pe- riod. J. Pediat., 51:453, 1957.
15. Swaiman, K. F., Austrian, S., and Raile, R. B.: Congenital atresia of the inferior vena cava, common iliac veins, and left innominate vein; a case with extensive development of superficial venous col- lateral circulation. A.M.A. J. Dis. Child., 96:81, 1958.
16. Allen, E. V., Barker, N. W., and Hines, E. A., Jr.: Peripheral Vascular Diseases, 2nd Ed. Philadelphia, Saunders, 1955, pp. 482-484.
858O B S T R U C T I O N
vena cava. Brit. Heart J., 18:320, 1956. 19. Rossall, R. E., and Caldwell, R. A.: Ob- struction of the inferior vena cava by a persistent eustachian valve in a young adult. J. Clin. Path., 10:40, 1957. 20. Rosenberg, N., and Zullo, R. J.: Massive
venous thrombosis associated with inco- agulability of the blood. Arch. Surg., 76:981, 1958.
21. Weber, F. P., Rast, H., and Wendt, L.: Thrombosis of the inferior vena cava
and prognosis of the condition. M. Press, 196:262, 1938.
22. Houston, A. N., Roy, W. A., and Faust, R. A.: Thrombophlebitis of superficial abdominal veins; an unusual complica- tion of inferior vena cava ligation. J.A.M.A., 166:2158, 1958.
23. Bronte-Stewart, B., and Goetz, R. H.: Budd-Chiari's syndrome: high inferior vena cava obstruction demonstrated by venography. Angiology, 3: 167, 1952.
PROBLEMS: THE NEURO-PSYCHOLOGI- CAL
Ed.D.Springfield, Illinois, Charles C Thomas, Publisher,
Dr. Delacato, a Philadelphia educator and specialist in remedial reading with an estab- lished reputation has written a challenging book which postulates a neurologic basis as the etiology of reading and speech problems. Un- fortunately, the rigors of scientific method are not employed in validating his serious views on this subject.
In evaluating 45 retarded male readers, Dr. Delacato found such factors as emotional mal- adjustment, social immaturity, rigid parents, lax parents, severe emotional conflicts at home, poor attitude toward school, lack of motiva- tion, to be uncommon characteristics of this group. The following, however, were titled uni- versal, "in that each existed in forty or more of the group of forty-five"
. ..: Early child- hood thumb-sucking of the thumb of the domi- nant hand, posturalization during sleep, some birth complications, some lack of unilateral- ity.
. . .
While this study might provide a base line for further exploration, the author proceeds with an involved program for correction and elimination of reading difficulties. H e does not explain why reading specialists have success with the majority of their clients without fol-
know from experience that almost any ap- proach works with some, including the un- canny effectiveness of placebo in most reported experiments with drugs.
I t is very difficult for us to accept such sug- gestions as children should not be allowed to such the thumb of the dominant hand; during the ages of 5 and 6 tonal activities should be kept at a minimum; when dressing, young children should put the sock, shoes, mittens, etc., on the dominant hand or foot first, that the child's fluid intake should be restricted gently, as should be his intake of salts and sugars which tend to retain fluids in the body. In addition we find it hard to go along with the notion that neurologically damaged poor readers are relatively well adjusted. In our ex- perience almost all of the hundreds of brain- damaged children evaluated, which included many poor readers, were also emotionally mal- adjusted, although it was clear that in many cases the organicity was an etiologic factor irk the maladjustment.
While I am not prepared to recommend Dr. Delacato's book a t this time, I am impressed with the possibility that aspects of his ap- proach may be correct for a limited number of cases which exhibit the characteristics he finds in his poor-reader group. But a good deal more research, as Dr. Delacato himself sug- gests, is needed before his views could com- mand a wider acceptance.
OBSTRUCTION OF THE INFERIOR VENA CAVA IN CHILDHOOD: Report of a
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