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Splenoportography in the pediatric age group
Splenoportography in the Pediatric Age Group By Jordan J. Weitzman and Philip Stanley 9 Eleven splenoportograms were performed in 10 patients between the ages of 2.5 and 17 yr. A definitive diagnosis was made in every instance. There were no complications despite the fact that 6 patients had platelet counts less than l O 0 , 0 0 0 / m m s. Fear of potential complications is unwarranted, and splenoportography should be an essential part of the workup of a child suspected of having portal hypertension. Splenoportography, when combined with m e a s u r e m e n t of splenic pulp pressure, provides precise information regarding the presence of a n d / o r change in portal hypertension and its underlying cause. With this information, therapy for the pediatric patient with portal hypertension can be individualized and managed in a logical fashion.
matic, (4) periumbilical, (5) lumbar, and (6) perirectal. The most important of these collateral systems are the gastroesophageal and splenorenal, the former being undesirable because it leads to the development of gastric and esophageal varices, and the latter being the most desirable because it seems to result in the most effective spontaneous portal systemic shunts. A more detailed description of the portal venous system and its collateral channels is reported elsewhere. 1,2
INDEX WORDS: Splenoportography; portal hypertension; splenomegaly.
The 10 patients ranged in age from 2.5 to 17 yr. One patient had two splenoportograms 7 yr apart. The indication for the splenoportogram was either splenomegaly of unknown origin (4 patients) or upper gastrointestinal bleeding associated with splenomegaly (6 patients). In the patients with splenomegaly alone, splenoportography was performed after an extensive workup (hematologic studies, liver function tests, upper gastrointestinal series, and liver-spleen scan) was nondiagnostic. All of the patients had hypersplenism with platelet counts less than 100,000/mm 3 (48,00096,000/mm 3) in 6 patients and counts between 100,000/mm3 and 120,000/mm 3 in the remaining 4 patients. Nine of the patients had barium swallows to look for esophageal varices. Prior to the splenoportogram, a platelet count, prothrombin time (PT), partial thromboplastin time (PTT), and Ivy bleeding time were performed in all patients. The PT was 60% or more in every instance, and the PTT and Ivy bleeding time were within normal limits. All of the splenoportograms were performed under general anesthesia. With the patient apneic, an Argyle Medicut Teflon intravenous catheter* is percutaneously inserted into the enlarged spleen in the midaxillary line just below the left costal margin. The catheter is directed upward at a 3045-degree angle, puncturing the splenic capsule. It is then advanced into the splenic pulp for 2-3 cm. The stylet is withdrawn, and a slow drip of blood confirms the position of
T H O U G H splenoportography almost alA Lways provides conclusive diagnostic information, this procedure has not received widespread acceptance in the evaluation of children suspected of having portal hypertension. This may be due to unjustified concern about the potential complications. The purpose of this paper is to report on 11 splenoportograms performed in 10 patients and to justify our belief that percutaneous splenoportography is the most valuable single study available to evaluate the portal venous system in a child suspected of having portal hypertension. DEFINITIONS
MATERIALS AND TECHNIQUE
Obstruction o f n o r m a l flow through the portal vein by i n t r a h e p a t i c or e x t r a h e p a t i c disease leads to the d e v e l o p m e n t o f collateral c i r c u l a t i o n t h a t will either b y p a s s t h e block a n d r e e n t e r the portal s y s t e m d i s t a l l y ( h e p a t o p e t a l flow) or divert t h e blood a w a y f r o m the liver into the caval v e n o u s s y s t e m ( h e p a t o f u g a l flow). T h e collection of h e p a t o p e t a l collaterals t h a t develop as a result of portal vein t h r o m b o s i s or o b s t r u c tion is called c a v e r n o m a t o u s t r a n s f o r m a t i o n of t h e portal v e i n . B e c a u s e of insufficiency of the hepatopetal collaterals and/or high portal v e n o u s pressure, h e p a t o f u g a l collaterals develop, s h u n t i n g blood a w a y f r o m the liver a n d into the caval systems. T h e collateral c h a n n e l s t h a t c o n n e c t t h e portal a n d caval systems are: (1) g a s t r o e s o p h a g e a l , (2) s p l e n o r e n a l , (3) d i a p h r a g -
*Manufactured by Scherwood Medical Industries, St. Louis, Mo. 63103. From the Departments of Surgery and Radiology, Children's Hospital of Los Angeles, and the University of Southern California School of Medicine, Los Angeles, Calif. Presented before the 9th Annual Meeting of the American Pediatric Surgical Association, Hot Springs, Virginia, May 3~5, 1978. Address reprint requests to Jordan J. Weitzman, M.D., 6200 Wilshire Boulevard, Los Angeles, Calif. 90048. 9 1978 by Grune & Stratton, Inc. 0022-3468/78/1307-0025501.00/0
Vol. 13, No. 6D (December),1978
707
Journal of Pediatric Surgery,
708
WEITZMAN AND STANLEY
the Teflon sheath within the splenic pulp. An extension tube with a three-way stopcock on the end is connected to the Teflon cannula. By means of a venous pressure manometer the splenic pulp pressure is measured. It is very important that once the capsule of the spleen is penetrated the patient be kept apneic whenever the cannula is manipulated. If this is not done, respiratory movements may cause the cannula to lacerate the spleen or more likely become dislodged. If the spleen is not grossly enlarged, the Medicut is inserted in the eighth or ninth intercostal space in the midaxillary line. If there is any doubt about the position of the cannula, a small test injection of contrast medium can be made and observed with an image intensifier. Forty milliliters of a low-viscosity contrast medium (e.g., Vascoray, meglumine, iothalamate, and sodium iothalamate) are then injected rapidly as serial films of the upper abdomen and lower thorax are exposed at the rate of two per second for 3 sec and then one per second for 20 sec. The cannula is left in place while the roentgenograms are being developed. This takes less than 5 min, and if the films are unsatisfactory, the study should be repeated. However, this has never been necessary in our experience. The cannula is then removed, and the patient is placed on the left side for 6 hr. RESULTS
There were no complications. Following the procedure there was no detectable drop in hemoglobin or hematocrit. In 2 cases there were small extravasations of contrast medium. This caused no problem. Except for 1 patient who had a splenorenal shunt 2 days later, all of the patients were discharged on the day following the splenoportogram. A definitive diagnosis was made in every instance. Seven patients had extrahepatic portal hypertension due to portal vein obstruction, and 3 patients had intrahepatic portal hypertension. Splenoportography confirmed the presence of esophageal varices when they were demonstrated on esophagram in 4 patients, but varices were demonstrated in 4 other patients who had normal barium swallows. ILLUSTRATIVE
Fig. 1. Splenoportogram in case 1. Portal vein thrombosis in a young child. The hepatopetal collaterals draining into the liver are poorly developed. There is preferential flow through a large coronary vein (arrow) into large esophageal varices. There is some extrasplenic extravasation of the contrast medium.
hospitalized for 1 mo after birth. During that hospitalization she had had an umbilical vein catheter in place for 2 wk. Since age 8 mo splenomegaly had been noted. At age 6 yr she was hospitalized elsewhere for evaluation. Finally, because of hypersplenism, it was recommended that "a splenectomy be considered," but the patient was referred for evaluation. Arrangements for a splenoportogram were made, but before
CASES
Case 1 An otherwise healthy 3-yr-old white male presented with splenomegaly of unknown cause. The splenomegaly had been noted since the age of 2.5 yr. After an extensive workup was nondiagnostic, a splenoportogram was performed that showed evidence of portal vein thrombosis, with poor filling of hepatopetal collaterals. Excellent collateral circulation through a markedly dilated coronary vein and large esophageal varices was demonstrated (Fig. 1). The splenic pulp pressure was 75 cm saline. The patient is now 5 yr old and as yet has not had any gastreintestinal bleeding.
Case 2 A 6.5-yr-old otherwise healthy white female presented with splenomegaly of unknown cause. The patient was
Fig. 2. Splenoportogram in case 2. Portal vein thrombosis in an older child. Compared with Fig. 1, there is much better development of the hepatopetal collaterals (cavernomatous transformation of the portal vein). Numerous gastric and esophageal varices are seen.
SPLENOPORTOG RAPHY
it was performed the patient was hospitalized for her first episode of upper gastrointestinal bleeding. An upper gastrointestinal series suggested esophageal varices. The gastrointestinal bleeding stopped in 1 day, and 3 days later a splenoportogram was performed. This study showed evidence of portal vein obstruction, with good visualization of numerous hepatopetal collaterals and gastric and esophageal varices (Fig. 2). The splenic pulp pressure was 32 cm saline.
Comments P o r t a l v e i n t h r o m b o s i s is t h e m o s t c o m m o n 2 , 1 2 2 c a u s e r t a l h y p e r t e n s i o n in t h e p e d i a t r i c a g e g r o u p . ~ I n m o s t i n s t a n c e s t h e r e is n o k n o w n e t i o l o g y , b u t in c a s e 2 t h e p l a c e m e n t o f a c a t h e t e r in t h e u m b i l i c a l v e i n c o u l d h a v e b e e n the cause. In case 1 the poor development of the hepatopetal collaterals may be due to the p a t i e n t ' s y o u n g age. A s t h i s c h i l d g r o w s , it is h o p e d t h a t t h e c o l l a t e r a l c i r c u l a t i o n to t h e liver will i n c r e a s e , r e s u l t i n g in a d e c r e a s e in t h e p o r t a l pressure.
Case 3 A white male had been noted to have splenomegaly and pancytopenia when he was 11 yr old. At another institution he had undergone extensive hematologic workups that had been nondiagnostic. At age 13 yr the patient had an episode of massive upper gastrointestinal bleeding. Fiberoptic gastroscopy demonstrated a "possible gastric ulcer." The patient was then referred for evaluation. Splenomegaly was the only
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abnormal physical finding. Liver function tests and upper gastrointestinal series were all normal. A splenoportogram revealed a normal portal vein with extensive esophageal varices (Fig. 3). The splenic pulp pressure was 30 cm saline. Because the portal hypertension was thought to be due to intrinsic liver disease, laparoscopy was performed under the same anesthetic. A needle biopsy of a cirrhotic liver was performed under direct vision. Three days later a splenectomy and splenorenal shunt were performed. At this operation the splenic puncture site was noted to be sealed, and there was no free blood in the abdominal cavity. Four years after his operation, the patient has not rebled.
Comments The findings of this splenoportogram were u n e x p e c t e d . I n v i e w o f his g o o d g e n e r a l h e a l t h a n d n o r m a l liver f u n c t i o n tests, it w a s a s s u m e d that this patient had extrahepatic portal hypert e n s i o n . I n view o f t h e p r e s e n c e o f a d v a n c e d liver disease and potential life-threatening gastrointestinal hemorrhage, a portal systemic shunt was i n d i c a t e d . I n t h i s p a t i e n t it w a s e l e c t e d to perform a splenectomy and splenorenal shunt u t i l i z i n g t h e l a r g e s p l e n i c v e i n t h a t w a s well demonstrated on the splenoportogram.
Case 4 An otherwise healthy 11-yr-old black female presented with splenomegaly of unknown origin. Past history was unremarkable. An extensive workup was nondiagnostic. A splenoportogram revealed evidence of portal vein thrombosis, with cavernomatous tranformation of the portal vein, a dilated coronary vein, and esophageal varices. The splenic pulp pressure was 37 cm saline. The patient was lost to follow-up until 7 yr later, when she was again seen because of recurrent abdominal pain. She never had upper gastrointestinal bleeding, but she did have recurrent bleeding from hemorrhoids. The spleen was no longer palpable, but hypersplenism was documented. A repeat splenoportogram again demonstrated cavernomatous transformation of the portal vein. There was a natural portal systemic shunt through the left adrenal vein to the left renal vein to the inferior vena cava (Fig. 4). There was no filling of the coronary vein, and there were no esophageal varices. The splenic pulp pressure was 24 cm saline.
Case 5
Fig. 3. Splenoportogram in case 3. Portal hypertension due to cirrhosis of the liver. The portal vein is normal. There is preferential flow of the contrast medium into huge gastric and esophageal varices.
A 16-yr-old black male presented with upper gastrointestinal bleeding presumed to be from esophageal varices. Prior to the bleeding episode the patient had ingested two 6-packs of beer and had vomited several times. At 2 yr of age the patient had been noted to have splenomegaly. From age 5 to 10 yr he had had recurrent upper gastrointestinal bleeding. At 8 yr of age a splenoportogram had demonstrated evidence of portal vein thrombosis and esophageal varices. From age 10 to 16 yr the patient had not had any gastrointestinal bleeding. Physical examination was unremarkable. The spleen was not palpable, but there was laboratory evidence of
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A N D STANLEY
Fig. 4. Splenoportogram in case 4. Portal vein thrombosis in an older patient with development o f a spontaneous portal systemic shunt. The splenic vein drains into the inferior vena cava (arrow) via a large splenorenal collateral. There is also flow into a cavernomatous transformation of the portal vein.
hypersplenism. A barium swallow was suggestive of esophageal varices, which were also seen at esophagoscopy. A splenoportogram revealed the splenic vein draining into the inferior vena cava via the left renal vein (Fig. 5). The portal vein and esophageal varices were not visualized. The splenic pulp pressure was 25 cm saline.
Comments
These last 2 cases graphically demonstrate the development of natural portal systemic shunts
via splenorenal collaterals. In both patients the splenic pulp pressure was almost normal. In case 5 the bleeding episode probably was of variceal origin brought on.bysevere vomiting and retchrag. In view of near normal splenic pulp pressure and the development of the large portal systemic shunt, surgical intervention was not indicated. .....The hazards of drinking alcoholic beverages that might precipitate vomiting were pointed out to the patient, Subsequently the patient has not rebled for 2 yr. DISCUSSION
Fig. 5. Splenoportogram in case 5. Portal vein thrombosis with development of a large portal systemic shunt in an older patient. The splenic vein drains into the inferior vena cava (arrow) via a large splenorenal collateral and the left renal vein. The hepatopetal collaterals may not be visualized because of preferential flow of the contrast medium into the inferior vena cava.
The clinical history and studies such as liver function tests, liver-spleen scan, and upper gastrointestinal series provide useful information in the differential diagnosis of childhood portal hypertension. A definitive anatomic diagnosis, however, can be provided only by radiographic visualization of the portal venous system. Sple' noportography and selective celiac and superior mesenteric arteriography are the procedures of choice. Of the two techniques, splenoportography is the most useful, since the contrast medium is injected directly into the portal venous system, providing much better visualization. The ability to perform intrasplenic pulp manometry is an important additional advantage of splenoportography. These measurements have been shown to correlate well with intraoperative measurements of portal pressure. Splenic pulp pressures below 20 cm are considered normal,
SPLENO PORTOG RAPHY
71 1
and those above 25 cm are definitely abnormal. ~ Splenoportography almost always provides precise information regarding the presence of portal hypertension, its underlying cause, and the pattern of collateral circulation. In view of this and the fact that splenoportography is employed routinely in some centers, a'4 we find it difficult to understand why it has not received the widespread acceptance it deserves. Reluctance to employ splenoportography is probably due to fear of its complications and/or the belief that the information provided is mainly "academic." Splenic laceration and hemorrhage are the only significant potential complications. It is our experience that splenic laceration will not occur if the patient is kept apneic when the spleen is punctured and also whenever the needle is manipulated. In the pediatric age group, apnea can be assured only if the study is performed under general anesthesia. The use of a flexible Teflon catheter also reduces the risk of splenic laceration. In virtually every patient in whom splenoportography is indicated there is thrombocytopenia and some prolongation of the prothrombin time. A minimum platelet count of 100,000/mm 3 and a normal prothrombin time, as recommended by some, 4'5 are not always prerequisite for performing splenoportography. We concur with Clatworthy and others, t'6'7 who believe that a platelet count of 50,000/mm 3 and a prothrombin time of 50 are usually sufficient. Although we have never found it necessary, platelets a n d / o r fresh frozen plasma could be administered during splenoportography to correct coagulation deficiencies. Immediately after splenoportography, having the patient lie on the left side for several hours is essential. In this position, the heavy spleen tamponades the puncture site.
The information obtained from an initial splenoportogram, when combined with measurement of splenic pulp pressure, is invaluable in the management of patients with portal hyperten, sion. A child with splenomegaly with or without gastrointestinal hemorrhage due to extrahepatic portal hypertension has a good long-term prognosis. We concur with Fonkalsrud and his associates 8 that a significant number of these patients can be managed conservatively despite repeated bleeding episodes. Serial splenoportography and splenic pulp pressure measurements should be very helpful in identifying progress in these patients. The development of the hepatopetal and hepatofugal collateral circulations is a dynamic process, and as many patients grow older, there is ever increasing portal systemic collateral circulation. A demonstration of this development on splenoportography would support a decision for continued conservative management and save the patient an unnecessary extensive surgical procedure. If the frequency and severity of bleeding episodes necessitate surgical intervention, it is essential that splenoportography be performed preoperatively in order to outline the anatomy for the appropriate surgical procedure. When a shunt procedure is contemplated in a patient with intrahepatic portal hypertension, splenoportography is also helpful preoperatively. The presence of a portal vein should not be taken for granted. Portal vein thrombosis may be associated with liver disease; if this is the case, a portacaval shunt will not be possible. When indicated, splenoportography should not be postponed. This delay could result in a clinical situation of serious gastrointestinal bleeding without a definitive diagnosis and valuable prognostic information.
REFERENCES
1. Clatworthy HW Jr: Extrahepatic portal hypertension, in Dunphy JE (ed): Major Problems in Clinical Surgery, Philadelphia, Saunders, 1974, pp 243-266 2. RoschJ, Dotter CT: Extrahepaticportal obstructionin childhood and its angiographic diagnosis. Am J Roentgenol 112:143-149,1971 3. PinkertonJA, HolcombGW, Foster JH: Portal hypertension in childhood. Ann Surg 175:870-886, 1972 4. SherlockS: Diseaseof the Liverand BiliarySystem (ed 5). London, Blackwell, 1975, pp 166-171 5. Rosch J: Splenoportography, in Gyepes MT (ed):
Angiography in Infants and Children. New York, Grune & Stratton, 1974, pp 233-255 6. Foster JH, Conkle DM, Crane JM et al: Splenoportography. Ann Surg 179:773-781, 1974 7. LosowskyMS, Walker BE: Liverbiopsyand splenoportography in patients with thrombocytopenia.Gastroenterology 54:241-245, 1968 8. Fonkalsrud EW, Myers NA, Robinson MJ: Management of extrahepatic portal hypertensionin children. Ann Surg 180:487-493,1974
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Discussion T. Boles (Columbus): With respect to technique, that described by Dr. Weitzman is quite good. General anesthesia is essential for the small child. However, the study can be done under local anesthesia in most school-age children. Bleeding from needle puncture of the spleen, particularly with congestive splenomegaly, must be exceedingly rare; we have not seen this. Hypersplenism with thrombocytopenia has not caused bleeding secondary to splenoportography in our experience, nor has leukopenia been a cause of infection. Demonstration of the portal venous system by this technique is far better than doing an arterial injection through the superior mesenteric artery. Occasionally, demonstration of the portal venous system by splenoportography is inade-
quate. This is particularly true if the splenic vein itself is involved. To demonstrate the entire portal venous system, it is necessary to perform venous portography via a mesenteric vein at a diagnostic laparotomy. Information from these venographic studies is essential to the planning of an appropriate shunt in children with extrahepatic portal hypertension. J. Weitzman (closure): We are using general anesthesia right now because we do not want to take a chance of getting a splenic laceration. At the present time we are trying to convince a large medical community that splenoportography is a safe procedure. We have a small series and hope that with the passage of time we will be able to convince our pediatricians. They will endoscope a child two or three times to look at the varices, but they often will not let us do a splenoportogram.
matic, (4) periumbilical, (5) lumbar, and (6) perirectal. The most important of these collateral systems are the gastroesophageal and splenorenal, the former being undesirable because it leads to the development of gastric and esophageal varices, and the latter being the most desirable because it seems to result in the most effective spontaneous portal systemic shunts. A more detailed description of the portal venous system and its collateral channels is reported elsewhere. 1,2
INDEX WORDS: Splenoportography; portal hypertension; splenomegaly.
The 10 patients ranged in age from 2.5 to 17 yr. One patient had two splenoportograms 7 yr apart. The indication for the splenoportogram was either splenomegaly of unknown origin (4 patients) or upper gastrointestinal bleeding associated with splenomegaly (6 patients). In the patients with splenomegaly alone, splenoportography was performed after an extensive workup (hematologic studies, liver function tests, upper gastrointestinal series, and liver-spleen scan) was nondiagnostic. All of the patients had hypersplenism with platelet counts less than 100,000/mm 3 (48,00096,000/mm 3) in 6 patients and counts between 100,000/mm3 and 120,000/mm 3 in the remaining 4 patients. Nine of the patients had barium swallows to look for esophageal varices. Prior to the splenoportogram, a platelet count, prothrombin time (PT), partial thromboplastin time (PTT), and Ivy bleeding time were performed in all patients. The PT was 60% or more in every instance, and the PTT and Ivy bleeding time were within normal limits. All of the splenoportograms were performed under general anesthesia. With the patient apneic, an Argyle Medicut Teflon intravenous catheter* is percutaneously inserted into the enlarged spleen in the midaxillary line just below the left costal margin. The catheter is directed upward at a 3045-degree angle, puncturing the splenic capsule. It is then advanced into the splenic pulp for 2-3 cm. The stylet is withdrawn, and a slow drip of blood confirms the position of
T H O U G H splenoportography almost alA Lways provides conclusive diagnostic information, this procedure has not received widespread acceptance in the evaluation of children suspected of having portal hypertension. This may be due to unjustified concern about the potential complications. The purpose of this paper is to report on 11 splenoportograms performed in 10 patients and to justify our belief that percutaneous splenoportography is the most valuable single study available to evaluate the portal venous system in a child suspected of having portal hypertension. DEFINITIONS
MATERIALS AND TECHNIQUE
Obstruction o f n o r m a l flow through the portal vein by i n t r a h e p a t i c or e x t r a h e p a t i c disease leads to the d e v e l o p m e n t o f collateral c i r c u l a t i o n t h a t will either b y p a s s t h e block a n d r e e n t e r the portal s y s t e m d i s t a l l y ( h e p a t o p e t a l flow) or divert t h e blood a w a y f r o m the liver into the caval v e n o u s s y s t e m ( h e p a t o f u g a l flow). T h e collection of h e p a t o p e t a l collaterals t h a t develop as a result of portal vein t h r o m b o s i s or o b s t r u c tion is called c a v e r n o m a t o u s t r a n s f o r m a t i o n of t h e portal v e i n . B e c a u s e of insufficiency of the hepatopetal collaterals and/or high portal v e n o u s pressure, h e p a t o f u g a l collaterals develop, s h u n t i n g blood a w a y f r o m the liver a n d into the caval systems. T h e collateral c h a n n e l s t h a t c o n n e c t t h e portal a n d caval systems are: (1) g a s t r o e s o p h a g e a l , (2) s p l e n o r e n a l , (3) d i a p h r a g -
*Manufactured by Scherwood Medical Industries, St. Louis, Mo. 63103. From the Departments of Surgery and Radiology, Children's Hospital of Los Angeles, and the University of Southern California School of Medicine, Los Angeles, Calif. Presented before the 9th Annual Meeting of the American Pediatric Surgical Association, Hot Springs, Virginia, May 3~5, 1978. Address reprint requests to Jordan J. Weitzman, M.D., 6200 Wilshire Boulevard, Los Angeles, Calif. 90048. 9 1978 by Grune & Stratton, Inc. 0022-3468/78/1307-0025501.00/0
Vol. 13, No. 6D (December),1978
707
Journal of Pediatric Surgery,
708
WEITZMAN AND STANLEY
the Teflon sheath within the splenic pulp. An extension tube with a three-way stopcock on the end is connected to the Teflon cannula. By means of a venous pressure manometer the splenic pulp pressure is measured. It is very important that once the capsule of the spleen is penetrated the patient be kept apneic whenever the cannula is manipulated. If this is not done, respiratory movements may cause the cannula to lacerate the spleen or more likely become dislodged. If the spleen is not grossly enlarged, the Medicut is inserted in the eighth or ninth intercostal space in the midaxillary line. If there is any doubt about the position of the cannula, a small test injection of contrast medium can be made and observed with an image intensifier. Forty milliliters of a low-viscosity contrast medium (e.g., Vascoray, meglumine, iothalamate, and sodium iothalamate) are then injected rapidly as serial films of the upper abdomen and lower thorax are exposed at the rate of two per second for 3 sec and then one per second for 20 sec. The cannula is left in place while the roentgenograms are being developed. This takes less than 5 min, and if the films are unsatisfactory, the study should be repeated. However, this has never been necessary in our experience. The cannula is then removed, and the patient is placed on the left side for 6 hr. RESULTS
There were no complications. Following the procedure there was no detectable drop in hemoglobin or hematocrit. In 2 cases there were small extravasations of contrast medium. This caused no problem. Except for 1 patient who had a splenorenal shunt 2 days later, all of the patients were discharged on the day following the splenoportogram. A definitive diagnosis was made in every instance. Seven patients had extrahepatic portal hypertension due to portal vein obstruction, and 3 patients had intrahepatic portal hypertension. Splenoportography confirmed the presence of esophageal varices when they were demonstrated on esophagram in 4 patients, but varices were demonstrated in 4 other patients who had normal barium swallows. ILLUSTRATIVE
Fig. 1. Splenoportogram in case 1. Portal vein thrombosis in a young child. The hepatopetal collaterals draining into the liver are poorly developed. There is preferential flow through a large coronary vein (arrow) into large esophageal varices. There is some extrasplenic extravasation of the contrast medium.
hospitalized for 1 mo after birth. During that hospitalization she had had an umbilical vein catheter in place for 2 wk. Since age 8 mo splenomegaly had been noted. At age 6 yr she was hospitalized elsewhere for evaluation. Finally, because of hypersplenism, it was recommended that "a splenectomy be considered," but the patient was referred for evaluation. Arrangements for a splenoportogram were made, but before
CASES
Case 1 An otherwise healthy 3-yr-old white male presented with splenomegaly of unknown cause. The splenomegaly had been noted since the age of 2.5 yr. After an extensive workup was nondiagnostic, a splenoportogram was performed that showed evidence of portal vein thrombosis, with poor filling of hepatopetal collaterals. Excellent collateral circulation through a markedly dilated coronary vein and large esophageal varices was demonstrated (Fig. 1). The splenic pulp pressure was 75 cm saline. The patient is now 5 yr old and as yet has not had any gastreintestinal bleeding.
Case 2 A 6.5-yr-old otherwise healthy white female presented with splenomegaly of unknown cause. The patient was
Fig. 2. Splenoportogram in case 2. Portal vein thrombosis in an older child. Compared with Fig. 1, there is much better development of the hepatopetal collaterals (cavernomatous transformation of the portal vein). Numerous gastric and esophageal varices are seen.
SPLENOPORTOG RAPHY
it was performed the patient was hospitalized for her first episode of upper gastrointestinal bleeding. An upper gastrointestinal series suggested esophageal varices. The gastrointestinal bleeding stopped in 1 day, and 3 days later a splenoportogram was performed. This study showed evidence of portal vein obstruction, with good visualization of numerous hepatopetal collaterals and gastric and esophageal varices (Fig. 2). The splenic pulp pressure was 32 cm saline.
Comments P o r t a l v e i n t h r o m b o s i s is t h e m o s t c o m m o n 2 , 1 2 2 c a u s e r t a l h y p e r t e n s i o n in t h e p e d i a t r i c a g e g r o u p . ~ I n m o s t i n s t a n c e s t h e r e is n o k n o w n e t i o l o g y , b u t in c a s e 2 t h e p l a c e m e n t o f a c a t h e t e r in t h e u m b i l i c a l v e i n c o u l d h a v e b e e n the cause. In case 1 the poor development of the hepatopetal collaterals may be due to the p a t i e n t ' s y o u n g age. A s t h i s c h i l d g r o w s , it is h o p e d t h a t t h e c o l l a t e r a l c i r c u l a t i o n to t h e liver will i n c r e a s e , r e s u l t i n g in a d e c r e a s e in t h e p o r t a l pressure.
Case 3 A white male had been noted to have splenomegaly and pancytopenia when he was 11 yr old. At another institution he had undergone extensive hematologic workups that had been nondiagnostic. At age 13 yr the patient had an episode of massive upper gastrointestinal bleeding. Fiberoptic gastroscopy demonstrated a "possible gastric ulcer." The patient was then referred for evaluation. Splenomegaly was the only
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abnormal physical finding. Liver function tests and upper gastrointestinal series were all normal. A splenoportogram revealed a normal portal vein with extensive esophageal varices (Fig. 3). The splenic pulp pressure was 30 cm saline. Because the portal hypertension was thought to be due to intrinsic liver disease, laparoscopy was performed under the same anesthetic. A needle biopsy of a cirrhotic liver was performed under direct vision. Three days later a splenectomy and splenorenal shunt were performed. At this operation the splenic puncture site was noted to be sealed, and there was no free blood in the abdominal cavity. Four years after his operation, the patient has not rebled.
Comments The findings of this splenoportogram were u n e x p e c t e d . I n v i e w o f his g o o d g e n e r a l h e a l t h a n d n o r m a l liver f u n c t i o n tests, it w a s a s s u m e d that this patient had extrahepatic portal hypert e n s i o n . I n view o f t h e p r e s e n c e o f a d v a n c e d liver disease and potential life-threatening gastrointestinal hemorrhage, a portal systemic shunt was i n d i c a t e d . I n t h i s p a t i e n t it w a s e l e c t e d to perform a splenectomy and splenorenal shunt u t i l i z i n g t h e l a r g e s p l e n i c v e i n t h a t w a s well demonstrated on the splenoportogram.
Case 4 An otherwise healthy 11-yr-old black female presented with splenomegaly of unknown origin. Past history was unremarkable. An extensive workup was nondiagnostic. A splenoportogram revealed evidence of portal vein thrombosis, with cavernomatous tranformation of the portal vein, a dilated coronary vein, and esophageal varices. The splenic pulp pressure was 37 cm saline. The patient was lost to follow-up until 7 yr later, when she was again seen because of recurrent abdominal pain. She never had upper gastrointestinal bleeding, but she did have recurrent bleeding from hemorrhoids. The spleen was no longer palpable, but hypersplenism was documented. A repeat splenoportogram again demonstrated cavernomatous transformation of the portal vein. There was a natural portal systemic shunt through the left adrenal vein to the left renal vein to the inferior vena cava (Fig. 4). There was no filling of the coronary vein, and there were no esophageal varices. The splenic pulp pressure was 24 cm saline.
Case 5
Fig. 3. Splenoportogram in case 3. Portal hypertension due to cirrhosis of the liver. The portal vein is normal. There is preferential flow of the contrast medium into huge gastric and esophageal varices.
A 16-yr-old black male presented with upper gastrointestinal bleeding presumed to be from esophageal varices. Prior to the bleeding episode the patient had ingested two 6-packs of beer and had vomited several times. At 2 yr of age the patient had been noted to have splenomegaly. From age 5 to 10 yr he had had recurrent upper gastrointestinal bleeding. At 8 yr of age a splenoportogram had demonstrated evidence of portal vein thrombosis and esophageal varices. From age 10 to 16 yr the patient had not had any gastrointestinal bleeding. Physical examination was unremarkable. The spleen was not palpable, but there was laboratory evidence of
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Fig. 4. Splenoportogram in case 4. Portal vein thrombosis in an older patient with development o f a spontaneous portal systemic shunt. The splenic vein drains into the inferior vena cava (arrow) via a large splenorenal collateral. There is also flow into a cavernomatous transformation of the portal vein.
hypersplenism. A barium swallow was suggestive of esophageal varices, which were also seen at esophagoscopy. A splenoportogram revealed the splenic vein draining into the inferior vena cava via the left renal vein (Fig. 5). The portal vein and esophageal varices were not visualized. The splenic pulp pressure was 25 cm saline.
Comments
These last 2 cases graphically demonstrate the development of natural portal systemic shunts
via splenorenal collaterals. In both patients the splenic pulp pressure was almost normal. In case 5 the bleeding episode probably was of variceal origin brought on.bysevere vomiting and retchrag. In view of near normal splenic pulp pressure and the development of the large portal systemic shunt, surgical intervention was not indicated. .....The hazards of drinking alcoholic beverages that might precipitate vomiting were pointed out to the patient, Subsequently the patient has not rebled for 2 yr. DISCUSSION
Fig. 5. Splenoportogram in case 5. Portal vein thrombosis with development of a large portal systemic shunt in an older patient. The splenic vein drains into the inferior vena cava (arrow) via a large splenorenal collateral and the left renal vein. The hepatopetal collaterals may not be visualized because of preferential flow of the contrast medium into the inferior vena cava.
The clinical history and studies such as liver function tests, liver-spleen scan, and upper gastrointestinal series provide useful information in the differential diagnosis of childhood portal hypertension. A definitive anatomic diagnosis, however, can be provided only by radiographic visualization of the portal venous system. Sple' noportography and selective celiac and superior mesenteric arteriography are the procedures of choice. Of the two techniques, splenoportography is the most useful, since the contrast medium is injected directly into the portal venous system, providing much better visualization. The ability to perform intrasplenic pulp manometry is an important additional advantage of splenoportography. These measurements have been shown to correlate well with intraoperative measurements of portal pressure. Splenic pulp pressures below 20 cm are considered normal,
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and those above 25 cm are definitely abnormal. ~ Splenoportography almost always provides precise information regarding the presence of portal hypertension, its underlying cause, and the pattern of collateral circulation. In view of this and the fact that splenoportography is employed routinely in some centers, a'4 we find it difficult to understand why it has not received the widespread acceptance it deserves. Reluctance to employ splenoportography is probably due to fear of its complications and/or the belief that the information provided is mainly "academic." Splenic laceration and hemorrhage are the only significant potential complications. It is our experience that splenic laceration will not occur if the patient is kept apneic when the spleen is punctured and also whenever the needle is manipulated. In the pediatric age group, apnea can be assured only if the study is performed under general anesthesia. The use of a flexible Teflon catheter also reduces the risk of splenic laceration. In virtually every patient in whom splenoportography is indicated there is thrombocytopenia and some prolongation of the prothrombin time. A minimum platelet count of 100,000/mm 3 and a normal prothrombin time, as recommended by some, 4'5 are not always prerequisite for performing splenoportography. We concur with Clatworthy and others, t'6'7 who believe that a platelet count of 50,000/mm 3 and a prothrombin time of 50 are usually sufficient. Although we have never found it necessary, platelets a n d / o r fresh frozen plasma could be administered during splenoportography to correct coagulation deficiencies. Immediately after splenoportography, having the patient lie on the left side for several hours is essential. In this position, the heavy spleen tamponades the puncture site.
The information obtained from an initial splenoportogram, when combined with measurement of splenic pulp pressure, is invaluable in the management of patients with portal hyperten, sion. A child with splenomegaly with or without gastrointestinal hemorrhage due to extrahepatic portal hypertension has a good long-term prognosis. We concur with Fonkalsrud and his associates 8 that a significant number of these patients can be managed conservatively despite repeated bleeding episodes. Serial splenoportography and splenic pulp pressure measurements should be very helpful in identifying progress in these patients. The development of the hepatopetal and hepatofugal collateral circulations is a dynamic process, and as many patients grow older, there is ever increasing portal systemic collateral circulation. A demonstration of this development on splenoportography would support a decision for continued conservative management and save the patient an unnecessary extensive surgical procedure. If the frequency and severity of bleeding episodes necessitate surgical intervention, it is essential that splenoportography be performed preoperatively in order to outline the anatomy for the appropriate surgical procedure. When a shunt procedure is contemplated in a patient with intrahepatic portal hypertension, splenoportography is also helpful preoperatively. The presence of a portal vein should not be taken for granted. Portal vein thrombosis may be associated with liver disease; if this is the case, a portacaval shunt will not be possible. When indicated, splenoportography should not be postponed. This delay could result in a clinical situation of serious gastrointestinal bleeding without a definitive diagnosis and valuable prognostic information.
REFERENCES
1. Clatworthy HW Jr: Extrahepatic portal hypertension, in Dunphy JE (ed): Major Problems in Clinical Surgery, Philadelphia, Saunders, 1974, pp 243-266 2. RoschJ, Dotter CT: Extrahepaticportal obstructionin childhood and its angiographic diagnosis. Am J Roentgenol 112:143-149,1971 3. PinkertonJA, HolcombGW, Foster JH: Portal hypertension in childhood. Ann Surg 175:870-886, 1972 4. SherlockS: Diseaseof the Liverand BiliarySystem (ed 5). London, Blackwell, 1975, pp 166-171 5. Rosch J: Splenoportography, in Gyepes MT (ed):
Angiography in Infants and Children. New York, Grune & Stratton, 1974, pp 233-255 6. Foster JH, Conkle DM, Crane JM et al: Splenoportography. Ann Surg 179:773-781, 1974 7. LosowskyMS, Walker BE: Liverbiopsyand splenoportography in patients with thrombocytopenia.Gastroenterology 54:241-245, 1968 8. Fonkalsrud EW, Myers NA, Robinson MJ: Management of extrahepatic portal hypertensionin children. Ann Surg 180:487-493,1974
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Discussion T. Boles (Columbus): With respect to technique, that described by Dr. Weitzman is quite good. General anesthesia is essential for the small child. However, the study can be done under local anesthesia in most school-age children. Bleeding from needle puncture of the spleen, particularly with congestive splenomegaly, must be exceedingly rare; we have not seen this. Hypersplenism with thrombocytopenia has not caused bleeding secondary to splenoportography in our experience, nor has leukopenia been a cause of infection. Demonstration of the portal venous system by this technique is far better than doing an arterial injection through the superior mesenteric artery. Occasionally, demonstration of the portal venous system by splenoportography is inade-
quate. This is particularly true if the splenic vein itself is involved. To demonstrate the entire portal venous system, it is necessary to perform venous portography via a mesenteric vein at a diagnostic laparotomy. Information from these venographic studies is essential to the planning of an appropriate shunt in children with extrahepatic portal hypertension. J. Weitzman (closure): We are using general anesthesia right now because we do not want to take a chance of getting a splenic laceration. At the present time we are trying to convince a large medical community that splenoportography is a safe procedure. We have a small series and hope that with the passage of time we will be able to convince our pediatricians. They will endoscope a child two or three times to look at the varices, but they often will not let us do a splenoportogram.