Late sequelae of mesocaval shunts in children

Late Sequelae of Mesocaval Shunts in Children Matthew

J. Lambert,

Edward S. Tank, MD,*

Jeremiah

G. Turcotte,

III, MD, Ann Arbor, Michigan

Ann Arbor, Michigan MD, Ann Arbor, Michigan

In 1953 Marion [I] and Clatworthy, Wall, and Watman [2] independently described construction of a mesocaval anastomosis for portal decompression in children. Prior to their contributions, operative treatment of children with portal hypertension and variceal hemorrhage was often ineffective. Thrombosis of the portal vein, the most frequent cause of portal hypertension in children, precluded the construction of a portacaval shunt. The diminutive size of the splanchnic vessels in a small child led to technical problems and a high incidence of thrombosis of the shunt. Therefore, until the description of the mesocaval shunt, temporizing measures were often recommended for delaying operation until the child’s vasculature had enlarged sufficiently to permit a successful shunt. Clatworthy and DeLorimier [3] reviewed ninetythree cases of portal hypertension in children. They recorded a splenorenal shunt failure rate of 82 per cent in children under ten years of age. Portacaval shunts had a failure rate of 30 per cent in children of this age group and “makeshift” shunts were never successful. King and Shumacker [4] also noted the poor results of splenorenal shunts in children and recommended that this operation not be performed in children under eleven years of age.

From the Department of Surgery, Section of General Surgery, University of Michigan Medical Center, Ann Arbor, Michigan. This work was supported by USPHS Grant No. HE 04260-14. Reprint requests should be addressed to Dr Lambert, Department of Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48104. Presented at the Fourteenth Annual Meeting of the Society for Surgery of the Alimentary Tract, New York, New York, May 22 and 23, 1973. *Present address: University of Oregon Medical Center, 3181 Southwest Sam Jackson Park Road, Portland, Oregon 97201.

Volume 127, January

1974

In contrast, the early reports of results with the mesocaval shunt noted a negligible morbidity and mortality and a low incidence of recurrent hemorrhage [2,5,6]. Enthusiasm for this operation has endured and this operation is usually recommended as the procedure of choice in children with thrombosed portal veins and extrahepatic portal hypertension. However, there is a minimum of documentation in the literature concerning the long-term effects of a mesocaval shunt. We previously reported our initial results with portal decompression in children and now present a long-term follow-up study of fourteen children in whom a mesocaval shunt was constructed two to ten years previously ]7l. We were specifically interested in the long-term sequelae of dividing the vena cava and leaving a hypertrophied spleen in place. Liver function studies, hematologic surveys, history, and physical examinations performed in the late postoperative years after portal decompression #are summarized in this report. Material

and Methods

In January 1959 a special registry was established at the University of Michigan Medical Center to record follow-up data on all patients undergoing operative treatment for variceal hemorrhage secondary to portal hypertension. To date, 252 decompressive procedures have been performed in 248 patients. (Table I.) Thirtyone of these shunts were constructed in patients sixteen years of age or less. (Table II.) Fourteen children have had standard mesocaval shunts constructed. The first mesocaval shunt in our center was performed on July 3, 1962. All patients had esophageal varices documented by barium swallow and all had experienced at least one gastrointestinal hemorrhage. All surviving patients have been followed up through at least September 1971. The charts of all patients

19

Lambert,

TABLE

Tank,

I

and Turcotte

Type of Shunt

in 288 Patients

Type of Shunt

Number

End to side Side to side Mesocaval Splenorenal Portorenal Warren Mesorenal Double barrel portacaval Total * Four patients

underwent

122 87 19 15 6 1 1 1 252*

two shunts

each.

were reviewed. Six patients were seen in the Outpatient Department in March 1973. Each had a complete history, physical examination, and laboratory test evaluation which included liver function studies, a complete blood count, and platelet count. Selected patients had upper gastrointestinal barium x-ray studies. The families of three other patients were contacted by phone and current follow-up information was obtained. Two children last seen in late 1971 could not be contacted at this time. Results Preoperative Status. The preoperative status of each of the fourteen patients is recorded in Table III. Eight patients were male and six were female. The average age was eight years with a range of two and a half to sixteen years. The cause of portal hypertension was thrombosis of the portal vein in eleven of the children. Miscellaneous causes were congenital stenosis of the portal vein in one child and congenital hepatic fibrosis in another child. The youngest patient in the series had portal hypertension secondary to familial biliary stasis. This familial disease was also present in an identical twin and in an older sibling. The average preoperative weight of the children was 27 kg with a range of 8 to 65 kg. Seven patients had undergone at least one operation directly related to their disease. (Table IV.) None of these operations was successful in preventing recurrent hemorrhage. TABLE

II

Type of Shunt in Twenty-Nine Sixteen Years of Age or Less Type of Shunt

Number

Mesocaval Side to side End to side Splenorenal* Portorenal Total

14 7 5 4 1 31*

* Two patients

20

Patients

had prior mesocaval

shunts

which failed.

The number of episodes of hemorrhage prior to construction of a mesocaval shunt ranged from one to ten with an average of three per patient. Ascites was noted in three patients prior to con-

struction of a mesocaval shunt. Most patients had evidence of secondary hypersplenism prior to portal decompression. Splenomegaly was present in ten of eleven patients. Nine patients had thrombocytopenia with platelet counts less than 200,000 per mm3. Six of the ten patients had leukopenia with white blood cell counts less than 5,000 per mm3. With the exception of the patient with familial biliary stasis, liver function studies were not grossly deranged. The most frequently abnormal laboratory value was the serum glutamic oxalacetic transaminase. Alkaline phosphatase was elevated, but this is difficult to interpret in a growing child. Early Postshunt Morbidity and Mortality. The only owrative death occurred in the patient with familial biliary stasis and poor hepatic reserve. This child seemed to be doing well when she died suddenly on the tenth postoperative day. Autopsy was performed, but the cause of death was not obvious. It was difficult to determine with certainty if the shunt was patent, but there was no evidence of gastrointestinal hemorrhage. Transitory edema of the lower extremities occurred in two patients and mild superficial phlebitis in one patient in the immediate postoperative period. Recurrent Hemorrhage. Two patients had recurrent hemorrhage within three months of construction of the mesocaval shunt. Both these shunts proved to be occluded on mesenteric angiography. Both patients were managed conservatively for approximately three years. During this time one patient experienced four and the other five gastrointestinal hemorrhages. Both subsequently underwent successful construction of a splenorenal shunt and have not had rebleeding after five months and six years of follow-up study. Hypersplenism. Palpable spleens of moderate size have persisted in two children, but peripheral platelet, red, and white blood cell counts were within normal limits in these two patients. Three patients with depressed platelet counts preoperatively were relieved of evidence of secondary hypersplenism after construction of the mesocaval shunt. Mild to moderate thrombocytopenia persisted in four other patients despite a successful mesocaval shunt. The mesocaval shunt in another patient with persistent thrombocytopenia was

The American Journal of Surgery

Mesocavai

TABLE

Preoperative Status of Fourteen Patients with Portal Hypertension Variceal Hemorrhage Subsequently Having Mesocaval Shunts

III

Age Or) Case

and Sex

1

7!F

2

4,M

3

4,M

4

15,M

5

5,M

6

11.M

7

8,F

8

14,M

9

Disease

Number of Variceal Weigh1 Bleeding Episodes AscitesEdema (kg)

$hrJrltsinChild:en

and Secondary

Plaielet Countor White Estimate Bdirubin Blood Cell (Normal: (Normal: 200,1)00- O-1.0 count Splenomegaly X 10" 400,000) mg/lOO ml)

SGOT

i-25I u)

Alkahne Phosphatase (Normal 4-12 King-Armstrongunits)

Portal vein thrombosis Portal vein stenosis Portal vein thrombosis Portal vein thrombosis Portal vein thrombosis

26

3

0

0

Previous splenectomy

7.3

Normal

0.8

90

7.2

18

4

0

0

++

3.5

160,000

0.2

37

17.0

16

2

-t

0

+++

8.1

83,750

0.2

42

10

0

0

Previous splenectomy

5.8

18

1

0

0

++++

4.2

Portal vein thrombosis Congenital hepatic fibrosis Portal vein thrombosis

39

2

0

0

++

27

1

0

0

35

3

0

0

16,M

Portal vein thrombosis

65

3

0

0

10

8,F

29

4

0

0

11

7,F

Portal vein thrombosis Portal vein thrombosis

20

2

0

12

7,F

Portal vein thrombosis

21

1

0

17

2

6

13

14

Portalvein thrombosis 2llz.F Familial biliary stasis

5,M

0.2

51

13.4

86,000

0.7

22

3.3

2.9

83,000

0.6

31

19.0

+++

2.3

109,000

0.1

17

14.9

0

12.8

720.000

0.1

27

52.0

6.3

Normal

0.8

27

10.9

++++

4.3

110,000

2.0

0

+

5.1

169,000

0.9

0

+++

3.3

218,000

37

10.8

+

0

++++

5.8

120,000

56

18.7

+

0

+++

8.1

134,000

demonstrated to be closed and the platelet count returned to normal levels after splenectomy and a splenorenal shunt. The second patient whose mesocaval shunt thrombosed had a normal platelet count preoperat,ively, but thrombocytopenia developed after the mesocaval operation. This thrombocytopenia disappeared after splenectomy and construction of the splenorenal shunt. The platelet count in three of the four remaining patients who had depressed peripheral counts preop-

Volume127,January1974

Normal

10.8

Previous splenectomy

6.6

11.9

35

127

eratively increased by an average of 40 per cent after the mesocaval shunt. In none of these patients has hypersplenism been of’ clinical significance. (Table V.) Liver Function. Mild abnormalities in liver function have been present in most patients even years after construction of a successful shunt. Serum glutamic oxalacetic transaminase and alkaline phosphatase levels were both within normal limits in only one of eight patients studied recent-

Lambert,

Tank,

TABLE

IV

and Turcotte

Previous Operations Performed Fourteen Patients Subsequently Me;ocaval Shunt

Case

in Seven of Treated by

Operation

1 3 4

Splenorenal shunt and splenectomy Ligation of esophagel varices Splenectomy and liver biopsy; subtotal gastrectomy and ligation of varices; portacaval shunt Open liver biopsy Splenorenal shunt Ligation of esophageal varices; splenectomy Exploratory celiotomy

6

a 9 11

Comments

ly. Serum bilirubin level was mildly elevated in three patients. There is no other sign or symptom of hepatic dis,ease in these patients and all were leading normal and active lives. Esophageal Varices. Two children had barium swallow studies six to ten years after construction of the mesocaval shunt. In these children, persistent esophageal varices were demonstrated but had decreased in size compared to those on previous examinations. Neither of these children had experienced any recurrent variceal hemorrhage. Chronic

Venous

Insufficiency

and

Phlebitis.

There have been no signs or symptoms of chronic venous insufficiency in any child during the late postoperative period. There is no history or evidence on physical examination of pedal edema, varicose veins, or thrombophlebitis. TABLE

V

Postoperative

Months Post Shunt (at last follow-up Case

Status of Eleven Patients

Number of Variceal Bleeding

examination)Episodes

Growth, Development, and Rehabilitation. Ten of the eleven patients have grown normally since operation. These children have progressed normally in school and have participated in all activities with the exception of contact sports. One child was mentally retarded and schizophrenic prior to construction of the shunt and his condition has not improved since operation.

Undergoing

Immediate results achieved with mesocaval shunts have proved to be quite satisfactory both in our experience and in that of others. The only postoperative death in our series occurred in the patient with poor hepatic reserve. Seven of nine children reported on by Zuidema and Ebert [8] underwent successful construction of a mesocaval shunt. In fifteen cases in children with portal vein thrombosis reported on by Voorhees et al [6] there were no postoperative deaths. Voorhees et al [6] also reported one postoperative death and a single incidence of recurrent hemorrhage in eight children with cirrhosis in whom mesocaval shunts were constructed. Not all mesocaval shunts remain patent. There are few reports which deal specifically with the time of shunt failure. The two cases in our series in which mesocaval shunts became thrombosed became obvious two and three months postoperatively and were associated with massive recurrent variceal hemorrhage. Presumably the two failures Successful

White Blood Pedal Ascites

Edema

1

119

0

0

0

2 3 4

117 115 96

0 0 0

0 0 0

0 0 0

6 7 a 9

106 72 56 74

0 0 0 0

0 0 0 0

0 0 0 0

10

72 53 33

0 0 0

0 0 0

0 0 0

Splenomegaly Splenectomy 0 + Spenectomy 0 0 0 Splenec-

Cell Count x 103

Construction

of a Mesocaval

Shunt

Platelet Countor Estimate

-

Bilirubin

SGOT'

Alkaline Phosphataset

6.3

...

0.4

.,.

a.2 7.7 15.0

336,000 218,000

...

0.9 1.4 1.4

218 54 137

4.8

137,000 169,000 ...

1.8 0.8 1.0

57

70

35$ ...

240

... ..*

307 234 ii8

...

..*

...

...

... ...

7.8 6.2 4.6

116,000 111,000 205,000

1.1 1.3 1.3

25 56 59

39 272 255

tomy 11 13

0 0 ++

* 13-47 International Units/ml (SMA 12/60) i 10-100 International Units/ml (SMA 12/60). $5-25 International Units. $4-12 King-Armstrong Units.

22

The American Journal of Surgery

Mesocavai

Shunts

in Children

Figure 1. Common iliac vein jnjection showing complete occlusion of the inferior vena cava above its origin. Collateral blood flow is noted filling the proximal inferior vena caya’ by way of ascending lumbar and left renal veins. Figure 2. lntraoperative portogram in a child with thrombosis of the . . . . _ z. porrar vem snowing cavern0 vein. transformation of the po

*

reported by Zuidema and Ebert [8] also occurred in the early postoperative period. We suspect that most of these shunt closures are the result of technical problems. One potential technical problem is a vena cava of seemingly insufficient length to reach the superior mesenteric vein without tension. Usually tension can be avoided if the third portion of the duodenum is fully mobilized in a cephalad direction or in some cases if a broad mesoduodenum exists between the pancreas and the third portion of the duodenum, the vena cava can be brought through an opening between the pancreas and the duodenum rather than inferior to the duodenum. Another technical problem which we encountered in our experience in adults and which led to a mesocaval shunt failure was the anastomosis of the vena cava to a branch of the superior mesenteric vein rather than to the major trunk of the vein. This pitfall can be avoided by dissecting the superior mesenteric vein until it disappears over the uncinate process and beneath the body of the pancreas. Eventually, extensive collateral circulation develops around the divided inferior vena cava, presumably accounting for the lack of chronic venous insufficiency. (Figure 1.) However, at times even in children, there will be transient edema of the lower extremities, and occasionally thrombophlebitis will occur in the postoperative period. For this reason, we recommend that all of these patients have the legs elevated to a 30 degree angle postoperatively and that elastic bandages or above the knee Jobst stockings be worn at least two to three weeks after construction of a mesoca-

Volume

127,

January

1974

val shunt. Thrombophlebitis did occur in one of our patients and in two instances t,here was significant lower extremity edema. If phlebitis does occur, the patient should be given anticoagulant therapy, initially heparin and then. Coumadin@ for two or three months and the use of elastic supports should be continued throughout that time. As has been noted with portacaval shunts in adults, construction of a mesocaval shunt does not completely relieve the signs of hypersplenism. In two cases, splenomegaly persisted and in three cases moderate thrombocytopenia was evident as late as nine years after operation. However, as with portacaval s.hunts, in no case have these depressed peripheral thrombocyte or white blood cell counts been of an:y detectable climcal significance. Also, varices continued to be demlonstrated in two children many years after construction of a shunt despite the absence of recurrent hemorrhage. Perhaps persistent splenomegaly, thrombocytopenia, or varices are evidence that pressure in the portacaval system, although diminished by the shunt, has not completely returned to normal. Portal vein thrombosis as a cause of portal hypertension can usually be differentiated from other causes by the presence of near normal results on liver function studies. .4n exception to this is congenital hepatic fibrosis in which liver function is also normal. In these cases the portal vein is patent and a portacaval shunt can be constructed. Therefore, we recommend evaluation by preoperative mesenteric arteriography or operative portogram to assess the status of the portal circulation. (Figure 2. j

Lambert,

Tank,

and Turcotte

The relatively long-term lack of complications after mesocaval shunts in children is confirmed by the few other case reports in the literature [5,7,8]. These combined experiences would seem sufficient to indicate that mesocaval shunts are associated with a low morbidity and mortality in the early postoperative period, have few long-term effects when performed in children, and effectively prevent variceal hemorrhage. For these reasons we continue to consider mesocaval shunts as the procedure of choice in children with thrombosis of the portal vein. Splenorenal shunts are reserved for the few patients in whom the mesocaval shunt fails. Planning for these operations and evaluating the patency of the shunts have been greatly facilitated in recent years with the availability of tolazoline hydrochloride (Priscolinea) mesenteric arteriography [9]. Summary

Fourteen patients have had construction of mesocaval shunts at the University of Michigan Medical Center since 1962. There has been one operative death. Two patients had recurrent hemorrhage secondary to thrombosis of the shunt. Both underwent successful splenorenal shunts. Of the eleven remaining patients, all are alive and well. No patient has exhibited signs of phlebitis or chronic venous insufficiency. Two patients con-

24

varices without evitinue to have demonstrable dence of hemorrhage. Mild abnormalities in liver function studies have been noted in the majority of our patients. The mesocaval shunt is a safe and effective means of preventing variceal hemorrhage and should be considered the decompressive procedure of choice in children with thrombosis of the portal vein.

References 1. Marion P: Les obstructions portales. Sem Hop Paris 29: 2781, 1953. 2. Clatworthy HW Jr, Wall T, Watman RN: A new type of portal-to-systemic venous shunt for portal hypertension. Arch Surg 71: 588,1955. 3. Clatworthy HW Jr, DeLorimier AA: Portal decompression procedures in children. Am J Sung 107: 447, 1964. 4. King H, Shumacker HB Jr: Venous shunt procedures for portal hypertension in children. Surgery 43: 680, 1958. 5. Foster JH, Sawyers JL, Scott HW Jr: Management of portal hypertension in children. Bull SOC Int Chir 4: 398, 1964. 6. Voorhees AB Jr, Harris RC, Britton RC, Price JB, Santulli TV: Portal hypertension in children: 98 cases. Surgery 58: 540, 1965. 7. Tank ES, Wallin VW Jr, Turcotte JG, Child CG III: Surgical management of bleeding gastroesophageal varices in children. Arch Surg 98: 451, 1969. 8. Zuidema GD, Ebert PA: Mesenteric-caval anastomosis for portal decompression. Johns Hopkins Med J 120: 201, 1967. 9. Reuter SR, Redman HC: Cirrhosis and portal hypertension. Gastrointestinal Angiography. Philadelphia, Saunders, 1972, p 219.

The American Journal of Surgery