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Spontaneous rupture of a mesenteric varix in portal hypertension
BRIEF REPORTS washed u n d e r r u n n i n g water. T h e formalin is changed and the nylon mesh and sections put back into it.
Mounting A sheet of transparent Perspex is flooded with m o u n t i n g gelatine solution (vide infi'a). T h e section is picked up with tim fingers and placed on the Perspex. Folds, bubbles, and surplus gelatine are teased out with the fingers. More m o u n t i n g solntion is poured onto the section and then it is covered with a sheet of Whatman 3 mm. chromatogral)hy paper. Carefnl layering of the paper toward the operator eliminates most of the bubbles. Tim Perspex is tilted to allow excess solution to r u n off and then t u r n e d over to allow the section to be gently manilmlatcd into tim best position u n d e r direct vision through the Perspex. For organs that tend to stick to the Perspex, especially brain, a sheet of water-permeable cellophane (grade P.T. (300) is wet'trader the tap and put over the l'erspex before it is flooded with motmting solution. A second sheet of cellophane must also be put on top of the paper in order to prevent the paper section from curling up after removal front the l'erspex.
Mounting Gelatine Solution Gelatine (80-100 Bloom) Glycerine Cellosohe 1% Thimerosal Water to
Embedding Gelatine Solution Gelatine (80-100 Bloom) Cellosolve (2-ethoxyethanol) Capryl alcohol 1% Thimerosal Water to
1000 240 30 20 5800
gin. ml. ml. nd. nil.
This is modified fi'om tim Gongh-Wentworth formula by reducing the gelatine content. In addition the solution is stored in a warm cupboard (40 ~ C.) for three to four days I)efi~re use. Fresh or fnll strength gehttine is too rubbery and does not come away fi'om tim section but remains to give a dirty background. About 1000 ml. is used for each block.
gin. ml. ml. ml. nil.
Tim glycerine in the Gough-Went~'orth formula is reduced for the Jamaican climate because it makes the sections rather stick)'. For six sections, 700 ml. is used. ACKNOXVLEDGMENTS I am most grateful to Professor G. Bras, University of the West Indies, for his continned support and enconragement, and to *h'. L. Dawson for photograplly.
References 1. Gough,J., and Wentworth,J. E.: The use of thin sections of entire organs in morbid anatomicalstudies.J. Roy. Micr. Sot., 69:231, 19-t9. 2. Gough,J.: Twenty)ears of the technicof paper mounted sections. In l.iebow,A. A., and Smith, D. E. (eds.): The Lung. Bahimorc, The Williams& WilkinsCo., 1968. 3. Whimster, W. F.: Rapid giant paper sections of hmg. Thorax, 24:739. 1968. Department of Pathology Royal Free Hospital Gray's hm Road ' London, W.C. 1
Drying T h e l'erspex is placed in the air conditioning draught (23 ~ C.). All d a m p patches disappear from the paper after 18 bouts or overnight. Drying is completed in the warm cabinet (40 ~ C.) for an hour or so and then the paper ntounted section can be peeled fiom tim l'erspex. It should be trintmed to remove an)" surl)lus gelatine at the edges, which can be stick)'. Storage in a dr)" atntosphere prevents deliquescence and the growth of molds. T h e double layer of cellophane allows the sections to be peeled more easily front the l'crsl)ex and the final product is ntuch tougher. Opaque areas develop d u r i n g drying if the sections are not cut thin enough.
75 50 40 10 1000
SPONTANEOUS RUPTURE OF A MESENTERIC VARIX IN PORTAL HYPERTENSION MELVIN H, SHER, M.D., F.A.C.S.* Abstract
In a patient with Laennec's cirrhosis and severe portal hypertension spontaneous rupture of a mesenteric varb: resulted in hemoperitoneum and death. A review of, the literature revealed one previous description of a similar situation. In portal hypertension, regardless of the cause, a collateral circulation is formed, a compensatory mechanisnt that carries the constant threat that tim veins invoh'ed may r u p t u r e u n d e r the increased intrahnninal presstue with ensuing hemorrlmge. When tiffs happens the esophageal or esophagogastric veins are usually involved and as a rule pose a relatively simple problem in diagnosis. T h e sittmtion is quite different when the source of blccding is an intraperitoneal blood *Clinical Instructor in Surgery, Boston Universit)" Medical School, Boston. Department of Surgery, Framinghanl Union ttoslfital, Framinghanl, Massachusetts.
167
H U M A N P A T H O L O G Y - - V O L U M E 1, N U M B E R 1 March, 1970
Figure 1. Mesenteric varices at postmortem.
channel and the diagnosis nmst be m a d e at the exclusion of an ahnost endless list o f possibilities. T h e choices may include as widely varying conditions as pathologic or traumatic r u p t u r e o f a viscus, eroding prinmry or metastatic tumors, mesenterie thrombosis, stab o r gunshot wounds, acute lmmorrhagic pancreatitis, perforated gastric or d u o d e n a l ulcer, r u p t u r e d aneurysm, bleeding from a degenerating hepatonm, r u p t u r e d or twisted ovarian cyst; ectopic pregnancy, r u p t u r e d uterine veins, or bleeding from a henmrrhagic corpus luteum. This imposing list o f conditions would challenge even the most experienced diagnostician. O t h e r less frequent but well d o c u m e n t e d causes should not be i g n o r e d - h e m o p e r i t o n e u m secondary to bleeding varices in the cecnm or in the vaginal vault or from r u p t u r e d peritoneal adhesions) ~ T h e unique case r e p o r t e d by Rothschild et al. a involved bleeding into the peritoneal cavity fron~ a r u p t u r e d mesenteric varix in a patient with portal hypertension. T h e ' case here recorded duplicates this occttrrence.
168
CASE REPORT A 43 )'ear old white male known to have alcoholic cirrhosis was admitted to the F r a m i n g h a m Union Hospital on August 26, 1968, with intermittent lower abdominal pain o f three days' duration as the chief complaint. He admitted to a heavy alcoholic intake over
the preceding 20 years a n d had had several hospitalizations because of epistaxis. In March 1968 he experienced his first episode o f hematemesis and was hospitalized at another institution. He presented with the clinical a n d laboratory manifestations of alcoholic cirrhosis and recovered on conservative therapy. He was discharged but was advised to u n d e r g o portacaval shunt surgery. A t adnfission examination the blood pressure was unobtainable and the apical pulse was 50 beats per minute. He was resuscitated with controlled respiration (20 per minute) via an endotracheal tube a n d received several blood transfusions. T h e positive physical findings included dilated pupils that were slightly reactive to light, bilateral gynecomastia, a distended a b d o m e n with a definite fluid wave, spider angiomas, a n d clubbed fingers with cyanotic nailbeds. T h e laboratory data revealed a normal electrocardiogram and normal chest a n d abdominal x-rays. T h e hemoglobin was 5.7 gin. p e r 100 ml. with a hematocrit o f 18 per cent. Tim leucocyte count was 48110 with a normal ditI'erential count. T h e serum'amylase was 81 nnits; serum calcium, 8.5 rag. per 100 nd.; total bilirubin, 0.6 mg. per 1 0 0 ml.; and serum glutamic oxaloacetic acid, 24 ReitmanFrankel units, l'eritoneal paracentesis yielded a blood)" fluid with a total protein of 3.9 rag. per 100 nil., a specific gravity of 1.026, a hemoglobin level of 5.7 gin. p e r 100 ml., and a Immatocrit o f ! 8 per cent. With evidence of intra-abdominal hemorrhage the patient received blood transfissions and exploration was p e r f o r m e d through a long midline abdominal incision. T h e a b d o m e n contained large amounts of free blood. T h e liver was enlarged and had a cobble-stone surface with varying size nodules. T h e spleen was two to three times its nornml size. T h e pancreas, stomach, and d u o d e n u m were normal. Tire site o f bleeding was f o u n d to be a large r u p t u r e d varix in the mesentery of tim snmall bowel. T h e mesenteric veins o f the small intestine were markedly d i l a t e d - u p to 0.5 c m . - s e r p i g i n o u s , firm, and thick walled with no a p p a r e n t thrombotic occlusion. Bleeding was controlled by suture ligation, and a portacaval shunt was carried out. T h e patient was r e t u r n e d to the intensive care unit, but later in tire day cardiac arrest occurred in which resuscitation was of no avail. Necropsy examination showed advanced hepatic cirrhosis of the portal type, splenomegaly with acute and chronic congestion, p u l m o n a r y congestion, the recent portacaval shunt, a n d a ligated mesenteric varix. T h e
BRIEF REPORTS
venous channels in the mesentery o f the small intestine were prominently tortuous and dilated and exhibited sinuosities and induration o f the walls. In some areas the veins showed a tendency to conglomeration as seen in the meninges in conjunction witlt arteriovenous malformations (Sturge-WeberDimitri disease). However, histologic sections o f the varices revealed extensive fibrosis and sclerotic invohttion of the walls (phlebosclerosis) in ttte absence o f deviations in structure consistent with malformation. Discussion Esophagogastric varices may wax and wane, a n d therefore their a p p e a r a n c e need not be related to tlte portal pressure at the time. Increased venous pressure is not tile essential or exclusive cause o f spontaneous r n p t u r e o f the esophageal or esopbagogastric varices, for ulceration, trauma, and bleeding contribute to the catastrophic even(. Since pressure in a closed system is equally distributed, it is hard to explain why blceding is prevalent in the esopttagogastric region ahnost to tim exclusion o f other sites. T h e concept that tile r u p t u r e o f the blood vessel occurs at tim site of a previous structural defect, either congenital or acquired, is sound but difficuh to prove and as suclt has been given very little consideration. As a rule, secondary clmnges, primarily collagcnous tissue proliferation and sclerosis, take place in the vessel wall as the resuh o f persistent intraluminal Itypertension, thereby obscuring any possible structural defect. When the r u p t u r e d vessel is in tim peritoneal cavity, tim ensuing lmmoperitoneunl causes peritoneal irritation, mtd as a consequence tire clinical picture is one o f splinting, tenderness, low grade fever, anentia, mild leucocytosis, and occasionally jaundice. T h e
course d e p e n d s on tim site and rate of bleeding and on whether t h e bleeding is within a confined space or i s free into tile abdominal ca~:it)'. W h e n b l e e d i n g is free, shock develops rapidly and overshadows all other manifestations. Not infrequently the onset of shock is insidious over days or weeks after tim onset o f tim illness, as tim protecting blood clot is displaced from the bleeding vessel. This event is associated with a s u d d e n slmrp, lancinating pain followed by d e e p e n i n g shock. Prior to tlte delayed rupture, tim e x p a n d i n g bematoma may cause signs a n d symptoms o f obstruction together with increasingly severe nmnifestalions of distress resuhing fi-om stretching of the peritoneal m a m b r a n e s . T o my knowledge tills case is the second r e p o r t e d o f spontaneous r u p t u r e of mesenteric varices witlt h e m o p e r i t o n e u m with hepatic cirrhosis. Tile ltistory of the otlter case is very similar to ours, both cases ending in death from acute anemia and irreversible shock. T h e possibility that tim r u p t u r e o f the ntesenteric varix was linked to a venous nmlforntation is entertained but cannot be proved from the resuhs o f the autopsy. References 1. Reimann, D. L., and Cowley, R. A.: Obscure cb.uses of intraabdominal hemorrhage. Amer. J. Surg., 71:329, 19t6. 2. Kreek, M. J., Raziano, J. v., ttardy, R. E., and Jeffrics, G. I1., Jr.: Portal hypertension with bleeding vaginal varices. Ann. Int. Mcd., 66:756, 1967. 3. l.evy, J. s., Ilardin, J. ll., Shipp, ll., and Kccling, J. ll.: Varices o[ tile CeCtlO1 as all lltltlStlal cause I)f gastr/lintestinal bleeding. (;astroenterology, 33:637, 1957. -I. Rothschild, .l.J., (;elernt, I., mid Sloan, W.: Ruptured nleSellteric vat'ix ill ch-rhosis--IlntlSllal Catlse flir Ilell/Operitoneum. New Eng. J. Med.0 278:97, !968. FranliJ'lghanl Union Ilospital 05 Evergreen Street Franlingham, Massachusetts 1117111
169
Mounting A sheet of transparent Perspex is flooded with m o u n t i n g gelatine solution (vide infi'a). T h e section is picked up with tim fingers and placed on the Perspex. Folds, bubbles, and surplus gelatine are teased out with the fingers. More m o u n t i n g solntion is poured onto the section and then it is covered with a sheet of Whatman 3 mm. chromatogral)hy paper. Carefnl layering of the paper toward the operator eliminates most of the bubbles. Tim Perspex is tilted to allow excess solution to r u n off and then t u r n e d over to allow the section to be gently manilmlatcd into tim best position u n d e r direct vision through the Perspex. For organs that tend to stick to the Perspex, especially brain, a sheet of water-permeable cellophane (grade P.T. (300) is wet'trader the tap and put over the l'erspex before it is flooded with motmting solution. A second sheet of cellophane must also be put on top of the paper in order to prevent the paper section from curling up after removal front the l'erspex.
Mounting Gelatine Solution Gelatine (80-100 Bloom) Glycerine Cellosohe 1% Thimerosal Water to
Embedding Gelatine Solution Gelatine (80-100 Bloom) Cellosolve (2-ethoxyethanol) Capryl alcohol 1% Thimerosal Water to
1000 240 30 20 5800
gin. ml. ml. nd. nil.
This is modified fi'om tim Gongh-Wentworth formula by reducing the gelatine content. In addition the solution is stored in a warm cupboard (40 ~ C.) for three to four days I)efi~re use. Fresh or fnll strength gehttine is too rubbery and does not come away fi'om tim section but remains to give a dirty background. About 1000 ml. is used for each block.
gin. ml. ml. ml. nil.
Tim glycerine in the Gough-Went~'orth formula is reduced for the Jamaican climate because it makes the sections rather stick)'. For six sections, 700 ml. is used. ACKNOXVLEDGMENTS I am most grateful to Professor G. Bras, University of the West Indies, for his continned support and enconragement, and to *h'. L. Dawson for photograplly.
References 1. Gough,J., and Wentworth,J. E.: The use of thin sections of entire organs in morbid anatomicalstudies.J. Roy. Micr. Sot., 69:231, 19-t9. 2. Gough,J.: Twenty)ears of the technicof paper mounted sections. In l.iebow,A. A., and Smith, D. E. (eds.): The Lung. Bahimorc, The Williams& WilkinsCo., 1968. 3. Whimster, W. F.: Rapid giant paper sections of hmg. Thorax, 24:739. 1968. Department of Pathology Royal Free Hospital Gray's hm Road ' London, W.C. 1
Drying T h e l'erspex is placed in the air conditioning draught (23 ~ C.). All d a m p patches disappear from the paper after 18 bouts or overnight. Drying is completed in the warm cabinet (40 ~ C.) for an hour or so and then the paper ntounted section can be peeled fiom tim l'erspex. It should be trintmed to remove an)" surl)lus gelatine at the edges, which can be stick)'. Storage in a dr)" atntosphere prevents deliquescence and the growth of molds. T h e double layer of cellophane allows the sections to be peeled more easily front the l'crsl)ex and the final product is ntuch tougher. Opaque areas develop d u r i n g drying if the sections are not cut thin enough.
75 50 40 10 1000
SPONTANEOUS RUPTURE OF A MESENTERIC VARIX IN PORTAL HYPERTENSION MELVIN H, SHER, M.D., F.A.C.S.* Abstract
In a patient with Laennec's cirrhosis and severe portal hypertension spontaneous rupture of a mesenteric varb: resulted in hemoperitoneum and death. A review of, the literature revealed one previous description of a similar situation. In portal hypertension, regardless of the cause, a collateral circulation is formed, a compensatory mechanisnt that carries the constant threat that tim veins invoh'ed may r u p t u r e u n d e r the increased intrahnninal presstue with ensuing hemorrlmge. When tiffs happens the esophageal or esophagogastric veins are usually involved and as a rule pose a relatively simple problem in diagnosis. T h e sittmtion is quite different when the source of blccding is an intraperitoneal blood *Clinical Instructor in Surgery, Boston Universit)" Medical School, Boston. Department of Surgery, Framinghanl Union ttoslfital, Framinghanl, Massachusetts.
167
H U M A N P A T H O L O G Y - - V O L U M E 1, N U M B E R 1 March, 1970
Figure 1. Mesenteric varices at postmortem.
channel and the diagnosis nmst be m a d e at the exclusion of an ahnost endless list o f possibilities. T h e choices may include as widely varying conditions as pathologic or traumatic r u p t u r e o f a viscus, eroding prinmry or metastatic tumors, mesenterie thrombosis, stab o r gunshot wounds, acute lmmorrhagic pancreatitis, perforated gastric or d u o d e n a l ulcer, r u p t u r e d aneurysm, bleeding from a degenerating hepatonm, r u p t u r e d or twisted ovarian cyst; ectopic pregnancy, r u p t u r e d uterine veins, or bleeding from a henmrrhagic corpus luteum. This imposing list o f conditions would challenge even the most experienced diagnostician. O t h e r less frequent but well d o c u m e n t e d causes should not be i g n o r e d - h e m o p e r i t o n e u m secondary to bleeding varices in the cecnm or in the vaginal vault or from r u p t u r e d peritoneal adhesions) ~ T h e unique case r e p o r t e d by Rothschild et al. a involved bleeding into the peritoneal cavity fron~ a r u p t u r e d mesenteric varix in a patient with portal hypertension. T h e ' case here recorded duplicates this occttrrence.
168
CASE REPORT A 43 )'ear old white male known to have alcoholic cirrhosis was admitted to the F r a m i n g h a m Union Hospital on August 26, 1968, with intermittent lower abdominal pain o f three days' duration as the chief complaint. He admitted to a heavy alcoholic intake over
the preceding 20 years a n d had had several hospitalizations because of epistaxis. In March 1968 he experienced his first episode o f hematemesis and was hospitalized at another institution. He presented with the clinical a n d laboratory manifestations of alcoholic cirrhosis and recovered on conservative therapy. He was discharged but was advised to u n d e r g o portacaval shunt surgery. A t adnfission examination the blood pressure was unobtainable and the apical pulse was 50 beats per minute. He was resuscitated with controlled respiration (20 per minute) via an endotracheal tube a n d received several blood transfusions. T h e positive physical findings included dilated pupils that were slightly reactive to light, bilateral gynecomastia, a distended a b d o m e n with a definite fluid wave, spider angiomas, a n d clubbed fingers with cyanotic nailbeds. T h e laboratory data revealed a normal electrocardiogram and normal chest a n d abdominal x-rays. T h e hemoglobin was 5.7 gin. p e r 100 ml. with a hematocrit o f 18 per cent. Tim leucocyte count was 48110 with a normal ditI'erential count. T h e serum'amylase was 81 nnits; serum calcium, 8.5 rag. per 100 nd.; total bilirubin, 0.6 mg. per 1 0 0 ml.; and serum glutamic oxaloacetic acid, 24 ReitmanFrankel units, l'eritoneal paracentesis yielded a blood)" fluid with a total protein of 3.9 rag. per 100 nil., a specific gravity of 1.026, a hemoglobin level of 5.7 gin. p e r 100 ml., and a Immatocrit o f ! 8 per cent. With evidence of intra-abdominal hemorrhage the patient received blood transfissions and exploration was p e r f o r m e d through a long midline abdominal incision. T h e a b d o m e n contained large amounts of free blood. T h e liver was enlarged and had a cobble-stone surface with varying size nodules. T h e spleen was two to three times its nornml size. T h e pancreas, stomach, and d u o d e n u m were normal. Tire site o f bleeding was f o u n d to be a large r u p t u r e d varix in the mesentery of tim snmall bowel. T h e mesenteric veins o f the small intestine were markedly d i l a t e d - u p to 0.5 c m . - s e r p i g i n o u s , firm, and thick walled with no a p p a r e n t thrombotic occlusion. Bleeding was controlled by suture ligation, and a portacaval shunt was carried out. T h e patient was r e t u r n e d to the intensive care unit, but later in tire day cardiac arrest occurred in which resuscitation was of no avail. Necropsy examination showed advanced hepatic cirrhosis of the portal type, splenomegaly with acute and chronic congestion, p u l m o n a r y congestion, the recent portacaval shunt, a n d a ligated mesenteric varix. T h e
BRIEF REPORTS
venous channels in the mesentery o f the small intestine were prominently tortuous and dilated and exhibited sinuosities and induration o f the walls. In some areas the veins showed a tendency to conglomeration as seen in the meninges in conjunction witlt arteriovenous malformations (Sturge-WeberDimitri disease). However, histologic sections o f the varices revealed extensive fibrosis and sclerotic invohttion of the walls (phlebosclerosis) in ttte absence o f deviations in structure consistent with malformation. Discussion Esophagogastric varices may wax and wane, a n d therefore their a p p e a r a n c e need not be related to tlte portal pressure at the time. Increased venous pressure is not tile essential or exclusive cause o f spontaneous r n p t u r e o f the esophageal or esopbagogastric varices, for ulceration, trauma, and bleeding contribute to the catastrophic even(. Since pressure in a closed system is equally distributed, it is hard to explain why blceding is prevalent in the esopttagogastric region ahnost to tim exclusion o f other sites. T h e concept that tile r u p t u r e o f the blood vessel occurs at tim site of a previous structural defect, either congenital or acquired, is sound but difficuh to prove and as suclt has been given very little consideration. As a rule, secondary clmnges, primarily collagcnous tissue proliferation and sclerosis, take place in the vessel wall as the resuh o f persistent intraluminal Itypertension, thereby obscuring any possible structural defect. When the r u p t u r e d vessel is in tim peritoneal cavity, tim ensuing lmmoperitoneunl causes peritoneal irritation, mtd as a consequence tire clinical picture is one o f splinting, tenderness, low grade fever, anentia, mild leucocytosis, and occasionally jaundice. T h e
course d e p e n d s on tim site and rate of bleeding and on whether t h e bleeding is within a confined space or i s free into tile abdominal ca~:it)'. W h e n b l e e d i n g is free, shock develops rapidly and overshadows all other manifestations. Not infrequently the onset of shock is insidious over days or weeks after tim onset o f tim illness, as tim protecting blood clot is displaced from the bleeding vessel. This event is associated with a s u d d e n slmrp, lancinating pain followed by d e e p e n i n g shock. Prior to tlte delayed rupture, tim e x p a n d i n g bematoma may cause signs a n d symptoms o f obstruction together with increasingly severe nmnifestalions of distress resuhing fi-om stretching of the peritoneal m a m b r a n e s . T o my knowledge tills case is the second r e p o r t e d o f spontaneous r u p t u r e of mesenteric varices witlt h e m o p e r i t o n e u m with hepatic cirrhosis. Tile ltistory of the otlter case is very similar to ours, both cases ending in death from acute anemia and irreversible shock. T h e possibility that tim r u p t u r e o f the ntesenteric varix was linked to a venous nmlforntation is entertained but cannot be proved from the resuhs o f the autopsy. References 1. Reimann, D. L., and Cowley, R. A.: Obscure cb.uses of intraabdominal hemorrhage. Amer. J. Surg., 71:329, 19t6. 2. Kreek, M. J., Raziano, J. v., ttardy, R. E., and Jeffrics, G. I1., Jr.: Portal hypertension with bleeding vaginal varices. Ann. Int. Mcd., 66:756, 1967. 3. l.evy, J. s., Ilardin, J. ll., Shipp, ll., and Kccling, J. ll.: Varices o[ tile CeCtlO1 as all lltltlStlal cause I)f gastr/lintestinal bleeding. (;astroenterology, 33:637, 1957. -I. Rothschild, .l.J., (;elernt, I., mid Sloan, W.: Ruptured nleSellteric vat'ix ill ch-rhosis--IlntlSllal Catlse flir Ilell/Operitoneum. New Eng. J. Med.0 278:97, !968. FranliJ'lghanl Union Ilospital 05 Evergreen Street Franlingham, Massachusetts 1117111
169