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Massive gastric hemorrhage: An unusual complication of Henoch-Schönlein purpura
Massive Gastric Hemorrhage: An Unusual Complication of H e n o c h - S c h ~ n l e i n Purpura By Thomas R. Weber, Jay L. Grosfeld, Jerry Bergstein, and Joseph Fitzgerald Indianapolis, Indiana
O T w o severely ill children with multisystem involvement of Henoch-Sch6nlein purpura developed massive upper gastrointestinal hemorrhage, requiring operative ligation of gastric stress ulcers, and vagotomy and pyloroplasty. Arteriographic embolization was also used in one of the children when she developed recurrent hemorrhage. Survival in both children emphasizes the need for aggressive management of the many complications these children may develop. INDEX WORDS: Henoch-Sch6nlein purpura; gastric hemorrhage.
ENOCH-SCHONLEIN
(HS)
PUR-
H P U R A is a disease of unknown cause, characterized by cramping abdominal pain, purpuric rash, and various combinations of renal, central nervous system, joint, and gastrointestinal symptoms. ~ While intussusception and bloody diarrhea have been commonly observed in previously reported cases, massive gastric hemorrhage is a rare complication. This report describes two children with HS purpura who developed massive upper gastrointestinal hemorrhage related to stress ulceration in conjunction with other severe multisystem manifestations of this condition. Aggressive surgical therapy is warranted in these critically ill children to achieve survival. CASE REPORTS Case 1. A 13-year-old girl was transferred to JW Riley Hospital for Children at the Indiana University Medical Center in Indianapolis because of renal failure and a purpuric rash over the buttocks, consistent with Henoch-Schonlein purpura. Two days prior to transfer, she developed cramping abdominal pain and vomiting, followed by bloody diarrhea. Her previous history was negative, as was her family history. Physical examination revealed a disoriented, ill-appearing
From the Indiana University School of Medicine, Department of Surgery, Section of Pediatric Surgery, Department of Pediatrics, J W Riley Hospital for Children, Indianapolis, Indiana. Address reprint requests to Jay L. Grosfeld, MD, Section of Pediatric Surgery, J W Riley Hospital for Children, 1100 14/. Michigan Street, Indianapolis, IN 46223. © 1983 by Grune & Stratton, Inc. 00 22- 3468/8 3/180 5~9010501.00/0
576
girl with a purpuric rash over the buttocks and lower extremities. The abdomen was distended and diffusely tender. Bloody stool was obtained on rectal exam. Although examination of the joints was initially normal, she later developed arthritis of the knees and ankles. Her initial urinalysis was normal, but later studies showed proteinuria and hematuria. Renal biopsy revealed diffuse proliferative glomerulonephritis with crescents in 50% of glomeruli. Because of the severity of the glomerulonephritis and the abdominal cramping, she was started on prednisone and lmuran (Burroughs-Welcome). On the day following institution of steroids, bright red blood was noted in her nasogastric tube. This was initially irrigated clear with iced saline, and she was begun on cimetidine and frequent antacids. The bleeding recurred 12 hours later in massive amounts, causing hypotension and tachycardia. After rapid transfusion of whole blood and stabilization, she was taken to the operating room, where upper gastrointestinal endoscopy was attempted. It was unsuccessful due to the rapid accumulation of blood in the stomach. She underwent immediate laparotomy and gastrotomy, which demonstrated two briskly hemorrhaging ulcers along the lesser curvature of the stomach, near the fundic-antral junction. These were oversewn, and a truncal vagotomy and one-layer Heineke-Mikulicz pyloroplasty was performed. A gastrostomy tube was inserted. Postoperatively she required an intracranial pressure monitor for control of cerebral edema as well as a tracheostomy and positive pressure ventilation for pulmonary edema. Cimetidine and antacids were continued to maintain gastric pH greater than 5. Seven days after the first operative procedure, recurrent massive upper gastrointestinal hemorrhage necessitated a second laparotomy, with oversewing of two additional bleeding gastric ulcers. She experienced massive upper gastrointestinal hemorrhage again four days later, which was successfully treated by arteriographic embolization of the left gastric artery by Gelfoam clot. Serum gastrin levels were normal, and a Hollander test was negative for incomplete vagotomy. She was maintained on cimetidine and antacids, and gradually resolved the other manifestations of HS purpura over a 3-month period. Follow-up at 2 years after discharge has shown a continued satisfactory postoperative course.
Case 2. An 8-year-old boy was transferred to JW Riley Hospital for Children with a l-week history of fever, sore throat, and abdominal pain, and a two-day history of purpura over the buttocks, lower extremities, and elbows. Physical examination and laboratory studies confirmed the diagnosis of HS purpura. On the day following admission, he developed bloody diarrhea and became stuperous and unresponsive. Computerized tomography of the head showed a large left parietooccipital intracerebral hemorrhage. He was treated with Decadron and phenobarbitol, but continued to deteriorate, requiring craniotomy and evacuation of the clot on the seventh hospital day.
Journal of Pediatric Surgery, Vol. 18, No. 5 (October), 1983
GASTRIC HEMORRHAGE IN H-S PURPURA
Over the next 4 days, the patient was treated for persistent increased intracranial pressure with mannitol and pentobarbital; additional complications included hypertension, pneumonia, and intermittent upper gastrointestinal hemorrhage. Prednisone and lmuran therapy were initiated for rapidly progressive glomerulonephritis. Cimetidine and antacids were also begun to maintain gastric pH above 5. On the 22nd hospital day, he developed massive upper gastrointestinal hemorrhage, unresponsive to iced saline lavage. Celiac arteriography showed brisk hemorrhage from the lesser curvature of the stomach (Fig. 1). Infusion of vasopressin (0.4 units/ kg/min) directly into the feeding artery did not show the rate of hemorrhage. He underwent emergency laparotomy, and oversewing of several gastric ulcers and vagotomy and pyloroplasty. Postoperatively, he was maintained on cimetidine and antacids and showed no further signs of hemorrhage. Over the next 2 months he gradually resolved the remaining manifestations of HS purpura, and was discharged on antihypertensives and phenobarbital. Follow-up visits have shown continued improvement in mental status. DISCUSSION
HS purpura can present with devastating multisystem involvement, and continues to result in mortality despite recent advances in pediatric intensive care and aggressive drug therapy. ~This is adequately demonstrated by the complexity of the disease in the two case reports presented here. Continued improvement in survival might be expected if an aggressive therapeutic approach is taken toward the many complications these critically ill children can develop. The present report emphasizes several important factors. Massive upper gastrointestinal hemorrhage can be a significant complication in these children. High-dose steroid therapy, increased intracranial pressure, pneumonia and sepsis, renal failure, and coagulation abnormalities all may contribute to the susceptibility of these patients to stress ulceration and gastrointestinal hemorrhage. The typical location for stress ulceration, along the lesser curvature of the stomach high in the fundus, was observed in both our cases. For these reasons, aggressive prophylactic therapy with antacids and cimetidine would seem indicated early in the course of the disease. Unfortunately, massive bleeding occurred in our two cases despite the early institution of antacids and cimetidine. Massive hemorrhage precluded the use of fiberoptic endoscopy for diagnosis in these cases, but arteriography showed the point of hemorrhage very effectively (Fig. 1). This has been true
577
for other patients with acute ulcers seen recently at this institution. 2 Arteriographic attempts at stopping the hemorrhage using an infusion of vasopressin and/or embolization techniques through the catheter have been used successfully in adults but have had limited application in children. Attempted vasopressin infusion in our second patient and a patient previously reported from this institution 2 was unsuccessful in even slowing the rate of bleeding. Our first patient did respond to Gelfoam embolization when she bled a third time. The etiology of stress ulceration is in most cases unknown, and thus the most appropriate form of therapy for this condition is controversial. 3'4 Gastric acid hypersecretion is rarely observed, and digestive symptoms are nonexistent. The exception to this observation is the patient with increased intracranial pressure, where both hypergastrinemia and hyperacidity have been demonstrated, s It should be noted that both of our cases developed increased intracranial pressure early in their disease. Ischemia of gastric mucosa seems to play an important role in the development of stress ulceration. Experimentally4 decreased gastric mucosal blood flow can be demonstrated following hemorrhage, restraint, or endotoxin induced stress. Vasculitis involving gastric mucosa, which has been seen in other series of HS purpura, 6 may also cause
Fig. 1. Celiac arteriogram during active gastric hemorrhage in case 2. Arrows indicate extravasation of contrast f r o m t w o hemorrhaging gastric ulcers along the lesser curvature.
578
WEBER ET AL
ischemia and necrosis of mucosa with subsequent hemorrhage. The fact that massive hemorrhage occurred in our two cases in spite of vigorous medical therapy preoperatively with antacids and cimetidine would suggest that mucosal necrosis was partially responsible for this complication. Histologic evidence of this possibility however, is not available in our patients. A n u m b e r of operative approaches for stress ulceration, ranging from conservative (simple ligature of the ulcer) to radical (total gastrectomy) procedures have been advocated, without unanimous agreemeent. 3 In our two patients, gastrotomy with oversewing of the ulcers by deep suture ligation, using nonabsorbable suture material, combined with vagotomy and pyloroplasty was effective in stopping the hemorrhage. V a g o t o m y seemed indicated in both individuals as increased intracranial pressure was observed. In addition, a previous series concerning acute stress ulcer in children has shown the effectiveness of this operative approach. 2 Recurrent hemorrhage, however, did occur in our case 1 and was m a n a g e d by oversewing two additional ulcers in the first instance. A second recurrence of bleed-
ing was finally successfully controlled by Gelfoam clot embolization, avoiding the need for resection. Despite a large n u m b e r o f cases of H S purpura reported in the literature, there are few examples of upper gastrointestinal hemorrhage as a complication. Gilbert and DaSalva 6 reported a case with autopsy findings of widespread gastric necrosis with h e m o r r h a g e . C o h e n et al. 7 described a duodenal h e m a t o m a with mild gastrointestinal h e m o r r h a g e in a child, treated conservatively with success. O t h e r reports o f intraabdominal manifestations of H S purpura do not mention upper gastrointestinal h e m o r r h a g e as a complication. 8-1° It is apparent that aggressive surgical therapy is warranted in these critically ill children with multisystem involvement of their disease if survival is to be achieved. V a g o t o m y and pyloroplasty with oversewing of the bleeding points has proven to be well tolerated and relatively effective in abating these massively bleeding gastric lesions. In instances of rebleeding, embolization of the bleeding vessel m a y prove useful and avoid the necessity for extensive gastric resection.
REFERENCES
1. Allan DM, Diamond LK, Howell DA: Anaphylactoid purpura in children (Schonlein-Henoch syndrome): Review with follow-up of the renal complications. Am J Dis Child 99:833-854~ 1960 2. Grosfeld JL, Shipley F, Fitzgerald JF, et al: Acute peptic ulcer in infancy and childhood. Am Surg 44:13-19, 1978 3. Drapanas T, Woolvertan WC, Reeder JW, et al: Experiences with surgical management of acute gastric mucosal hemorrhage. Ann Surg 173:628-640, 1971 4. Moody FG, Cheung LY: Stress ulcers: Their pathogenesis, diagnosis, and treatment. Surg Clin North Am 56:14691478, 1976 5. Bowen JC, Fleming WH, Thompson JC: Increased gastrin release following penetrating central nervous system injury. Surgery 75:720--724, 1974
6. Gilbert FF, DaSalva AQ: Henoeh-Schonlein purpura: Report of a case with gastroinestinal hemorrhage and necrosis. Clin Pediatr 5:181-186, 1966 7. Cohen N, Grauer M, Berant M: Duodenal intramural hematoma in anaphylactoid purpura. J Pediatr 80:856, 1976 (letter) 8. Toledo-Pereyra LH, Van Renden T, Cich JA, Vonehir EG: Management of intraabdominal Henoch-Schonlein purpura. The role of surgery. Minn Med 59:376-379, 1976 9. Klein GL, Stafford S: Unusual gastrointestinal manifestations of Henoch-Schonlein purpura. Am J Dis Child 129:1238-1239, 1975 (letter) 10. Goldbloom RB, Drummond KN: Anaphylactoid purpura with massive gastrointestinal hemorrhage and glomerulonephritis. Am J Dis Child 116:97-102, 1968
O T w o severely ill children with multisystem involvement of Henoch-Sch6nlein purpura developed massive upper gastrointestinal hemorrhage, requiring operative ligation of gastric stress ulcers, and vagotomy and pyloroplasty. Arteriographic embolization was also used in one of the children when she developed recurrent hemorrhage. Survival in both children emphasizes the need for aggressive management of the many complications these children may develop. INDEX WORDS: Henoch-Sch6nlein purpura; gastric hemorrhage.
ENOCH-SCHONLEIN
(HS)
PUR-
H P U R A is a disease of unknown cause, characterized by cramping abdominal pain, purpuric rash, and various combinations of renal, central nervous system, joint, and gastrointestinal symptoms. ~ While intussusception and bloody diarrhea have been commonly observed in previously reported cases, massive gastric hemorrhage is a rare complication. This report describes two children with HS purpura who developed massive upper gastrointestinal hemorrhage related to stress ulceration in conjunction with other severe multisystem manifestations of this condition. Aggressive surgical therapy is warranted in these critically ill children to achieve survival. CASE REPORTS Case 1. A 13-year-old girl was transferred to JW Riley Hospital for Children at the Indiana University Medical Center in Indianapolis because of renal failure and a purpuric rash over the buttocks, consistent with Henoch-Schonlein purpura. Two days prior to transfer, she developed cramping abdominal pain and vomiting, followed by bloody diarrhea. Her previous history was negative, as was her family history. Physical examination revealed a disoriented, ill-appearing
From the Indiana University School of Medicine, Department of Surgery, Section of Pediatric Surgery, Department of Pediatrics, J W Riley Hospital for Children, Indianapolis, Indiana. Address reprint requests to Jay L. Grosfeld, MD, Section of Pediatric Surgery, J W Riley Hospital for Children, 1100 14/. Michigan Street, Indianapolis, IN 46223. © 1983 by Grune & Stratton, Inc. 00 22- 3468/8 3/180 5~9010501.00/0
576
girl with a purpuric rash over the buttocks and lower extremities. The abdomen was distended and diffusely tender. Bloody stool was obtained on rectal exam. Although examination of the joints was initially normal, she later developed arthritis of the knees and ankles. Her initial urinalysis was normal, but later studies showed proteinuria and hematuria. Renal biopsy revealed diffuse proliferative glomerulonephritis with crescents in 50% of glomeruli. Because of the severity of the glomerulonephritis and the abdominal cramping, she was started on prednisone and lmuran (Burroughs-Welcome). On the day following institution of steroids, bright red blood was noted in her nasogastric tube. This was initially irrigated clear with iced saline, and she was begun on cimetidine and frequent antacids. The bleeding recurred 12 hours later in massive amounts, causing hypotension and tachycardia. After rapid transfusion of whole blood and stabilization, she was taken to the operating room, where upper gastrointestinal endoscopy was attempted. It was unsuccessful due to the rapid accumulation of blood in the stomach. She underwent immediate laparotomy and gastrotomy, which demonstrated two briskly hemorrhaging ulcers along the lesser curvature of the stomach, near the fundic-antral junction. These were oversewn, and a truncal vagotomy and one-layer Heineke-Mikulicz pyloroplasty was performed. A gastrostomy tube was inserted. Postoperatively she required an intracranial pressure monitor for control of cerebral edema as well as a tracheostomy and positive pressure ventilation for pulmonary edema. Cimetidine and antacids were continued to maintain gastric pH greater than 5. Seven days after the first operative procedure, recurrent massive upper gastrointestinal hemorrhage necessitated a second laparotomy, with oversewing of two additional bleeding gastric ulcers. She experienced massive upper gastrointestinal hemorrhage again four days later, which was successfully treated by arteriographic embolization of the left gastric artery by Gelfoam clot. Serum gastrin levels were normal, and a Hollander test was negative for incomplete vagotomy. She was maintained on cimetidine and antacids, and gradually resolved the other manifestations of HS purpura over a 3-month period. Follow-up at 2 years after discharge has shown a continued satisfactory postoperative course.
Case 2. An 8-year-old boy was transferred to JW Riley Hospital for Children with a l-week history of fever, sore throat, and abdominal pain, and a two-day history of purpura over the buttocks, lower extremities, and elbows. Physical examination and laboratory studies confirmed the diagnosis of HS purpura. On the day following admission, he developed bloody diarrhea and became stuperous and unresponsive. Computerized tomography of the head showed a large left parietooccipital intracerebral hemorrhage. He was treated with Decadron and phenobarbitol, but continued to deteriorate, requiring craniotomy and evacuation of the clot on the seventh hospital day.
Journal of Pediatric Surgery, Vol. 18, No. 5 (October), 1983
GASTRIC HEMORRHAGE IN H-S PURPURA
Over the next 4 days, the patient was treated for persistent increased intracranial pressure with mannitol and pentobarbital; additional complications included hypertension, pneumonia, and intermittent upper gastrointestinal hemorrhage. Prednisone and lmuran therapy were initiated for rapidly progressive glomerulonephritis. Cimetidine and antacids were also begun to maintain gastric pH above 5. On the 22nd hospital day, he developed massive upper gastrointestinal hemorrhage, unresponsive to iced saline lavage. Celiac arteriography showed brisk hemorrhage from the lesser curvature of the stomach (Fig. 1). Infusion of vasopressin (0.4 units/ kg/min) directly into the feeding artery did not show the rate of hemorrhage. He underwent emergency laparotomy, and oversewing of several gastric ulcers and vagotomy and pyloroplasty. Postoperatively, he was maintained on cimetidine and antacids and showed no further signs of hemorrhage. Over the next 2 months he gradually resolved the remaining manifestations of HS purpura, and was discharged on antihypertensives and phenobarbital. Follow-up visits have shown continued improvement in mental status. DISCUSSION
HS purpura can present with devastating multisystem involvement, and continues to result in mortality despite recent advances in pediatric intensive care and aggressive drug therapy. ~This is adequately demonstrated by the complexity of the disease in the two case reports presented here. Continued improvement in survival might be expected if an aggressive therapeutic approach is taken toward the many complications these critically ill children can develop. The present report emphasizes several important factors. Massive upper gastrointestinal hemorrhage can be a significant complication in these children. High-dose steroid therapy, increased intracranial pressure, pneumonia and sepsis, renal failure, and coagulation abnormalities all may contribute to the susceptibility of these patients to stress ulceration and gastrointestinal hemorrhage. The typical location for stress ulceration, along the lesser curvature of the stomach high in the fundus, was observed in both our cases. For these reasons, aggressive prophylactic therapy with antacids and cimetidine would seem indicated early in the course of the disease. Unfortunately, massive bleeding occurred in our two cases despite the early institution of antacids and cimetidine. Massive hemorrhage precluded the use of fiberoptic endoscopy for diagnosis in these cases, but arteriography showed the point of hemorrhage very effectively (Fig. 1). This has been true
577
for other patients with acute ulcers seen recently at this institution. 2 Arteriographic attempts at stopping the hemorrhage using an infusion of vasopressin and/or embolization techniques through the catheter have been used successfully in adults but have had limited application in children. Attempted vasopressin infusion in our second patient and a patient previously reported from this institution 2 was unsuccessful in even slowing the rate of bleeding. Our first patient did respond to Gelfoam embolization when she bled a third time. The etiology of stress ulceration is in most cases unknown, and thus the most appropriate form of therapy for this condition is controversial. 3'4 Gastric acid hypersecretion is rarely observed, and digestive symptoms are nonexistent. The exception to this observation is the patient with increased intracranial pressure, where both hypergastrinemia and hyperacidity have been demonstrated, s It should be noted that both of our cases developed increased intracranial pressure early in their disease. Ischemia of gastric mucosa seems to play an important role in the development of stress ulceration. Experimentally4 decreased gastric mucosal blood flow can be demonstrated following hemorrhage, restraint, or endotoxin induced stress. Vasculitis involving gastric mucosa, which has been seen in other series of HS purpura, 6 may also cause
Fig. 1. Celiac arteriogram during active gastric hemorrhage in case 2. Arrows indicate extravasation of contrast f r o m t w o hemorrhaging gastric ulcers along the lesser curvature.
578
WEBER ET AL
ischemia and necrosis of mucosa with subsequent hemorrhage. The fact that massive hemorrhage occurred in our two cases in spite of vigorous medical therapy preoperatively with antacids and cimetidine would suggest that mucosal necrosis was partially responsible for this complication. Histologic evidence of this possibility however, is not available in our patients. A n u m b e r of operative approaches for stress ulceration, ranging from conservative (simple ligature of the ulcer) to radical (total gastrectomy) procedures have been advocated, without unanimous agreemeent. 3 In our two patients, gastrotomy with oversewing of the ulcers by deep suture ligation, using nonabsorbable suture material, combined with vagotomy and pyloroplasty was effective in stopping the hemorrhage. V a g o t o m y seemed indicated in both individuals as increased intracranial pressure was observed. In addition, a previous series concerning acute stress ulcer in children has shown the effectiveness of this operative approach. 2 Recurrent hemorrhage, however, did occur in our case 1 and was m a n a g e d by oversewing two additional ulcers in the first instance. A second recurrence of bleed-
ing was finally successfully controlled by Gelfoam clot embolization, avoiding the need for resection. Despite a large n u m b e r o f cases of H S purpura reported in the literature, there are few examples of upper gastrointestinal hemorrhage as a complication. Gilbert and DaSalva 6 reported a case with autopsy findings of widespread gastric necrosis with h e m o r r h a g e . C o h e n et al. 7 described a duodenal h e m a t o m a with mild gastrointestinal h e m o r r h a g e in a child, treated conservatively with success. O t h e r reports o f intraabdominal manifestations of H S purpura do not mention upper gastrointestinal h e m o r r h a g e as a complication. 8-1° It is apparent that aggressive surgical therapy is warranted in these critically ill children with multisystem involvement of their disease if survival is to be achieved. V a g o t o m y and pyloroplasty with oversewing of the bleeding points has proven to be well tolerated and relatively effective in abating these massively bleeding gastric lesions. In instances of rebleeding, embolization of the bleeding vessel m a y prove useful and avoid the necessity for extensive gastric resection.
REFERENCES
1. Allan DM, Diamond LK, Howell DA: Anaphylactoid purpura in children (Schonlein-Henoch syndrome): Review with follow-up of the renal complications. Am J Dis Child 99:833-854~ 1960 2. Grosfeld JL, Shipley F, Fitzgerald JF, et al: Acute peptic ulcer in infancy and childhood. Am Surg 44:13-19, 1978 3. Drapanas T, Woolvertan WC, Reeder JW, et al: Experiences with surgical management of acute gastric mucosal hemorrhage. Ann Surg 173:628-640, 1971 4. Moody FG, Cheung LY: Stress ulcers: Their pathogenesis, diagnosis, and treatment. Surg Clin North Am 56:14691478, 1976 5. Bowen JC, Fleming WH, Thompson JC: Increased gastrin release following penetrating central nervous system injury. Surgery 75:720--724, 1974
6. Gilbert FF, DaSalva AQ: Henoeh-Schonlein purpura: Report of a case with gastroinestinal hemorrhage and necrosis. Clin Pediatr 5:181-186, 1966 7. Cohen N, Grauer M, Berant M: Duodenal intramural hematoma in anaphylactoid purpura. J Pediatr 80:856, 1976 (letter) 8. Toledo-Pereyra LH, Van Renden T, Cich JA, Vonehir EG: Management of intraabdominal Henoch-Schonlein purpura. The role of surgery. Minn Med 59:376-379, 1976 9. Klein GL, Stafford S: Unusual gastrointestinal manifestations of Henoch-Schonlein purpura. Am J Dis Child 129:1238-1239, 1975 (letter) 10. Goldbloom RB, Drummond KN: Anaphylactoid purpura with massive gastrointestinal hemorrhage and glomerulonephritis. Am J Dis Child 116:97-102, 1968