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Mesenteric vascular occlusion resulting in intestinal necrosis in children
Mesenteric Vascular Occlusion Resulting in Intestinal Necrosis in Children By Pelin Og˘uzkurt, Mehmet Emin S¸enocak, Arbay O. Ciftci, F. Cahit Tanyel, and Nebil Bu¨yu¨kpamukc¸u Ankara, Turkey
Purpose: The records of 4 patients who had necrotic bowel secondary to acute mesenteric vascular occlusion affecting various levels of mesenteric vasculature were reviewed to determine the clinical manifestations, diagnostic investigations, predisposing factors, complications, and outcome of mesenteric vascular thrombosis in children. Methods: The medical records of the patients (3 boys, 1 girl) treated between 1981 and 1996, inclusive, for bowel infarction secondary to mesenteric vascular thrombosis, were reviewed with regard to signs and symptoms, laboratory tests, radiological investigations, surgical findings, histopathologic examinations, and outcome. Results: The ages of the patients ranged between 1 and 14 years with a mean age of 8.2 years. Initial symptoms, present in all patients, were abdominal pain, abdominal distension, and tenderness. Laboratory and radiological findings including abdominal radiographs and abdominal ultrasonography were nondiagnostic. Selective superior mesenteric angiography showed complete obliteration of the superior mesenteric artery with absence of venous return in 1 case. Three patients with massive intestinal necrosis died of multiorgan failure or the complications of short bowel syndrome. Histological
T
HE ANNUAL INCIDENCE of mesenteric infarction is approximately 1 in 100,000.1 Acute mesenteric vascular occlusion is associated with a high mortality rate. The age range generally is reported to be between 20 and 80 years, with most patients having an underlying disorder.2,3 Mesenteric ischemia and bowel infarction represent a broad spectrum of disease with diverse etiologies in elderly people.4,5 A primary or idiopathic form with no apparent cause also has been reported.3,6 Although very infrequently encountered, intestinal necrosis as a result of mesenteric vascular thrombosis also may be seen in childhood. Four patients with mesenteric vascular thrombosis with acute development of abdominal symptoms are presented. The aim of this study is to identify the mode of presentation, the underlying pathologies or factors that result in vascular thrombosis, the role of surgery in the treatment, and the life-saving principles of therapy. MATERIALS AND METHODS The records of patients with intestinal necrosis caused by mesenteric vascular thrombosis diagnosed between 1981 and 1996, inclusive, were reviewed. The ages at presentation, sex, clinical findings, laboratory investigations, diagnostic and therapeutic radiological Journal of Pediatric Surgery, Vol 35, No 8 (August), 2000: pp 1161-1164
examination of the resected intestinal segments showed the typical findings of polyarteritis nodosa in 2 patients. One patient had a previous history of right femoral vein thrombosis, whereas 1 patient had no known underlying disorders predisposing vascular thrombosis.
Conclusions: Mesenteric vascular occlusion is a rare but serious disease leading to death in children. The patients present with similar clinical signs, most frequent and important are acute abdominal pain, vomiting, and distension. Mesenteric vascular occlusion is a rare cause of acute abdomen in childhood, which requires urgent diagnosis and intervention. In suspected mesenteric vascular insufficiency, angiography should be performed followed by intraarterial thrombolytic infusion therapy in selected cases. When intestinal infarction is suspected, immediate surgical resection of compromised bowel is necessary with appropriate postoperative anticoagulation or treatment of any underlying disease. J Pediatr Surg 35:1161-1164. Copyright r 2000 by W.B. Saunders Company. INDEX WORDS: Mesenteric vascular occlusion, infarction, intestine.
approaches, surgical interventions, medical therapy, pathology, and outcome were analyzed retrospectively according to the special coding system that includes all the spectrum of disorders related to pediatric surgery in general headings.
RESULTS
Three boys and 1 girl underwent emergency surgery for intestinal infarction caused by mesenteric vascular thrombosis. The children ranged in age between 1 and 14 years with a mean age of 8.2 years. One patient had a spontaneous right femoral vein thrombosis that required thrombectomy 2 months before the present admission. The other 3 patients were healthy before presentation. The most striking and constant initial symptom was abdominal pain noted in all patients. The pain usually was intense, intermittent, and generalized. It was sudden onset (case 4) or prolonged for 24 hours to several days (3 From the Department of Pediatric Surgery, Hacettepe University Medical Faculty, Ankara, Turkey. Address reprint requests to Mehmet Emin S¸enocak, Kehribar sokak No: 9/46, 06700 C ¸ ankaya, Ankara, Turkey. Copyright r 2000 by W.B. Saunders Company 0022-3468/00/3508-0003$03.00/0 doi:10.1053/jpsu.2000.8718 1161
˘ UZKURT ET AL OG
1162
patients). The accompanying symptoms were vomiting, bloody stools, and diarrhea. The clinical manifestations were abdominal distension and tenderness, tachycardia, petechial lesions, mild cardiac murmur, and fever (Table 1). Nonspecific laboratory changes were observed. In case 4, the protein C and S levels were decreased transiently (3 mg/L and 20 mg/L, respectively) and returned to normal levels in the first postoperative week, but antithrombin III level was normal. Therefore, it was regarded as a result of consumption during thrombotic process. Abdominal radiographs showed dilated, thickened bowel loops and air-fluid levels in 2 patients and free peritoneal air in 1 child. In 2 patients, abdominal ultrasonography was performed that showed dilated bowel loops accompanied by increased amount of peritoneal fluid. In case 4, selective superior mesenteric angiography showed complete obliteration of the superior mesenteric artery (SMA) beyond 3 cm with absence of venous return (Fig 1). The colon was vascularized through the retrograde flow of middle colic vessels. The catheter could not be advanced, and balloon embolectomy was considered to be impossible. Therefore, a bolus of 100,000 IU urokinase was injected intraarterially, and it was followed by an infusion of 4,000 IU/min over 60 minutes. Repeated angiography showed no passage. High-dose urokinase application failed to revascularize the SMA. All patients underwent emergency laparotomy. Massive gangrene of the small bowel was found in 3 patients, and 2 necrotic jejunal segments with 10 and 25 cm in length were noted in 1 patient. Histological examination of the resected segments showed hemorrhagic necrosis
and patchy ulcerations of the intestinal mucosa, inflammatory exudates in some portions of serosa, and hemorrhage and edema in the submucosa. Fibrin thrombi were identified in the small vessels of mesentery and in the superior mesenteric vein branches. Vascular lesions including fibrosis, obliteration, and polymorphonuclear cell infiltration of the vascular walls were consistent with polyarteritis nodosa (PAN) in 2 patients (Table 1). Case 1 had not been treated previously with anticoagulants after the diagnosis of femoral vein thrombosis in another center, and the anticoagulant agent was started after the first abdominal operation. However, after the initial bowel resection, the patient has undergone 2 consecutive operations within 48 hours because of progressive bowel necrosis. The patient in case 4 also received long-term anticoagulant therapy postoperatively. Three patients with massive intestinal necrosis died of short bowel syndrome and infection or multiorgan failure caused by systemic manifestations of PAN. The surviving patient was put on medical treatment for the systemic manifestations of PAN after the operation. DISCUSSION
Bowel infarction caused by mesenteric vascular occlusion is a lethal condition.6 The mortality rate is reported to be 60% to 80% in adult series.4 Mesenteric ischemia represents a broad spectrum of diseases with various etiologies.7 Mesenteric vascular thrombosis has been reported in association with a number of disease processes.5 These include hereditary and acquired thrombotic disorders.8 There are certain clinical indicators of
Table 1. Clinical Data of the Patients Case 1
Age (yr)/sex Symptoms and signs Length of history (d) Physical findings Previous medical history Radiological investigations Angiography
1/M Abdominal pain, vomiting, bloody stool 1 Abdominal distension and tenderness Right femoral vein thrombosis Air-fluid levels No
Case 2
Case 3
10/F Abdominal pain, cutaneous lesions, seizures 2 Abdominal distension and tenderness Not remarkable
8/M Abdominal pain, vomiting, purpuric lesions 1 Abdominal distension and rigidity Not remarkable
14/M Abdominal pain, vomiting 1 Abdominal distension and tenderness Not remarkable
Air-fluid levels, dilated bowel loops No
Free peritoneal air
2 segments of jejunum (10 and 25 cm in length) Small mesenteric artery branches Fibrinoid necrosis in the walls of medium and small arteries (PAN) Survived
Dilated bowel loops, airfluid levels Complete obliteration of SMA Whole jejunum and ileum
Sites of the lesions
Segments of jejunum and ileum
Whole ileum
Thrombosed vessels
Superior mesenteric vein branches Fibrin thrombi in the small vessels of mesentery Exitus (postoperative third day)
Small vessels of the mesentery Necrotizing vasculitis (PAN)
Pathology
Outcome
Exitus (postoperative second day)
No
Case 4
Superior mesenteric artery and vein Fresh thrombus in SMA and SMV Exitus (postoperative third month)
MESENTERIC VASCULAR OCCLUSION
Fig 1. Selective superior mesenteric angiography show complete obliteration of the SMA beyond 3 cm (arrow).
hypercoagulable state.9 Therefore, the detailed history should be obtained for a family history of thrombosis, recurrent spontaneous thrombosis, and thrombosis at an early age and in unusual sites. Resistance to anticoagulant therapy and migratory superficial thrombophlebitis also should raise the suspicion of a primary hypercoagulable state. More sophisticated and detailed laboratory findings that may indicate the presence of thromboembolism include detection of platelet-specific marker proteins, increased platelet turnover, increased platelet procoagulantactivity, reduced fibrinolytic activity, circulating fibrin monomer complexes, prothrombin fragment 1,2, abnormal levels of fibrinopeptide A, thrombin-antithrombin III and plasmin- antiplasmin complexes, and abnormal levels of fibrin degradation products.9 Acquired thrombotic disorders can be called risk factors.8 Vascular endothelial abnormalities and cardiac diseases, diabetes mellitus, vasculitis (polyarteritis nodosa), artificial surfaces (prosthetic valves, vascular grafts), trauma, and surgery are the risk factors for the development of thrombotic processes.1,3,5,8,10,11 A primary or idiopathic form of mesenteric vascular thrombosis also can occur in young people without any underlying disorder.3,5,7,12 There are a few cases of mesenteric venous thrombosis in the pediatric age group. Eight cases were reported in a review from 1900 to 1976.5 The age range of these children was 4 days to 14 years. Although 7 patients had a predisposing factors for vascular disease, 1 patient had no underlying disease. The major difficulty is the lack of definitive diagnostic and laboratory tests.13 The biochemical abnormalities usually occur late in the course of the disease, when necrosis already has developed. In suspected cases of
1163
acute mesenteric ischemia, complete blood count, blood pH and blood gas content analysis, and blood biochemistry results should be obtained as soon as possible.13 Coagulation tests including coagulation factors, protein C and S, antithrombin III, prothrombin time and partial thromboplastin time, fibrinogen level, and fibrin degradation products also should be added to the investigations. Complete physical examination, especially the vital signs and abdominal findings, are important in the initial evaluation. Nonspecific bowel gas and air-fluid levels are seen in the abdominal radiographs.2 Marked reduction of air or gasless abdomen may be noted in the early stage of infarction as well.2,11 Computed tomography (CT) scans and ultrasound scans may show thickened bowel loops which is caused by venous thrombosis. CT also may show pneumatosis intestinalis, thumbprinting of the small bowel, and mesenteric or portal venous gas, but both techniques only give clues for infarcted or ischemic bowel.2,7 Although CT could not be able to give a specific diagnosis in cases of mesenteric infarction secondary to arterial occlusion, it may be diagnostic tool for cases of mesenteric venous thrombosis.7 Angiography has been used as a diagnostic and therapeutic procedure, especially for arterial occlusion. Angiography shows spasm of arteries, diminished or absent blood flow in affected bowel wall, and absence of venous drainage.5 Treatment of superior mesenteric artery (SMA) thrombosis or thromboembolism by intraarterial infusion of urokinase or streptokinase by local high-dose or selective low-dose therapy have been reported to result in successful thrombolysis.14,15 In children, mesenteric vascular thrombosis may occur both in idiopathic form or in association with a predisposing disease. In our series, 3 patients had underlying disorders: polyarteritis nodosa in 2 and previous history of deep venous thrombosis in the other. In case 1, deep venous thrombosis preceeded the progressive thrombosis of mesenteric vessels resulting in infarction of almost the whole small intestine. In this patient there was no documentation of the coagulation profile to find out whether a coagulation disorder such as antithrombin III or protein C deficiency was present.5,12 The patient may have represented a type of hereditary thrombotic disorder (a primary hypercoagulable state) or spontaneous thrombosis in which a previous history of peripheral venous thrombosis of the extremities and migratory thrombophlebitis is not unusual, but no exact cause can be found.5,8,12 The patients in case 2 and 3 have acquired thrombotic disorder in which the risk factor was a specific type of vasculitis: polyarteritis nodosa.16 In PAN the fibrinoid necrosis in the walls of medium- and small-sized arteries in addition to obliteration and fibrosis of the vessels result in bowel infarction. PAN rarely involves the gastrointesti-
˘ UZKURT ET AL OG
1164
nal system in childhood, and only a few cases have been reported with an initial presentation of mesenteric thrombosis.17 In cases 2 and 3, PAN was documented in the histological examination of the resected segments. Both patients presented with complications related to gastrointestinal involvement and could be diagnosed after laparotomy. In case 4, there are no identifiable risk factors as well as underlying hereditary thrombotic disorders. Therefore, the patient was regarded to have idiopathic thrombotic process. We can not say that all of our cases are examples of venous thrombosis. Excluding the cases 2 and 3 of PAN, the others were considered to be mesenteric venous thrombosis resulting in arterial occlusion. In cases 2 and 3, the initial occlusive mechanism was the fibrinoid necrosis of small- and medium-sized arteries. Whatever the cause of thrombosis, the obliteration of the mesenteric veins or venules result in hypovolemia and hemoconcentration that causes reduced flow in the SMA with arterial spasm and led to arterial thrombosis. Irreversible necrosis occurs at about 14 hours.5 Mesenteric vascular occlusion is a rare but serious disease in childhood leading to death. The patients may
present with diverse clinical signs and symptoms often suggestive of intestinal obstruction rather than mesenteric infarction. It must be borne in mind in cases of acute abdomen with or without systemic manifestations because of its rapid and usually fatal outcome. Selective SMA angiography is the most reliable diagnostic procedure in suspected cases. Thrombolysis of the SMA thrombosis through intraarterial infusion of urokinase or streptokinase may result in revascularization of the small intestine. Angiographic demonstration of the arterial blood flow with venous return in the small mesenteric vessels also is the superior monitorization method for the intestinal viability. Therefore, patency should be checked 30 minutes after intraarterial infusion of thrombolytic agent with an opaque injection associated with power Doppler ultrasound scan. If repeated angiography shows occlusion, laparotomy should be performed urgently. Surgical resection of necrotic bowel and anticoagulant therapy to control progressive vascular thrombosis should be carried out without delay. Time-consuming radiological and laboratory investigations should be avoided.
REFERENCES 1. Singh RP, Lee STJ: Acute mesenteric vascular occlusion: A review of 40 cases. Int Surg 65:231-234, 1980 2. Nozaki H, Kohno A, Narimatsu A, et al: Superior mesenteric artery occlusion: An enhanced CT finding. J Comput Assist Tomogr 15:866-867, 1991 3. Nair HT, Muscroft TJ: Idiopathic mesenteric thrombosis. Br J Clin Pract 44:651-652, 1990 4. Inderbitzi R, Wagner HE, Seiler C, et al: Acute mesenteric ischemia. Eur J Surg 158:123-126, 1992 5. Grendell JH, Ockner RK: Mesenteric venous thrombosis. Gastroenterology 82:358-372, 1982 6. Rogers MD, Thompson JE, Garret WV, et al: Mesenteric vascular problems. Ann Surg 195:554-565, 1982 7. Kim JY, Ha HK, Kyun JY, et al: Intestinal infarction secondary to mesenteric venous thrombosis: CT pathologic correlation. J Comput Assist Tomogr 17:382-385, 1993 8. Lee GR, Bithell TC, Foerster J, et al: Wintrobe’s Clinical Hematology. Philadelphia, PA, Lea & Febiger, 1993, p 1521 9. Lee GR, Bithell TC, Foerster J, et al: Wintrobe’s Clinical Hematology. Philadelphia, PA, Lea & Febiger, 1993, p 1524 10. Tsai CJ, Kuo YC, Chen PC, et al: The spectrum of acute
intestinal vascular failure: A collective review of 43 cases in Taiwan. Br J Clin Pract 44:603-608, 1990 11. Umpleby HC: Thrombosis of the superior mesenteric vein. Br J Surg 74:694-696, 1987 12. Olson JF, Steuber CP, Hawkin E, et al: Functional deficiency of protein C associated with mesenteric venous thrombosis and splenic infarction. Am J Pediatr Hematol Oncol 13:168-171, 1991 13. Hunter GC, Guernsey JM: Mesenteric ischemia. Med Clin North Am 72:1091-1115, 1988 14. Badiola CM, Scoppetta DJ: Rapid revascularization of an embolic superior mesenteric artery occlusion using pulse-spray pharmacomechanical thrombolysis of urokinase. AJR 169:55-57, 1997 15. McBride KD, Gaines PA: Thrombolysis of a partially occluding superior mesenteric artery thromboembolus by infusion of streptokinase. Cardiovasc Intervent Radiol 17:164-166, 1994 16. Schaller JG: Vasculitis syndromes, in Behrman RE, Kliegman RM, Arvin AM (eds): Nelson Textbook of Pediatrics, Philadelphia, PA Saunders, 1996, pp 680-681 17. Gu¨ndog¯du HZ, Kale G, Tanyel FC: Intestinal perforation as an initial presentation of polyarteritis nodosa in an 8-year old boy. J Pediatr Surg 28:632-634, 1993
Purpose: The records of 4 patients who had necrotic bowel secondary to acute mesenteric vascular occlusion affecting various levels of mesenteric vasculature were reviewed to determine the clinical manifestations, diagnostic investigations, predisposing factors, complications, and outcome of mesenteric vascular thrombosis in children. Methods: The medical records of the patients (3 boys, 1 girl) treated between 1981 and 1996, inclusive, for bowel infarction secondary to mesenteric vascular thrombosis, were reviewed with regard to signs and symptoms, laboratory tests, radiological investigations, surgical findings, histopathologic examinations, and outcome. Results: The ages of the patients ranged between 1 and 14 years with a mean age of 8.2 years. Initial symptoms, present in all patients, were abdominal pain, abdominal distension, and tenderness. Laboratory and radiological findings including abdominal radiographs and abdominal ultrasonography were nondiagnostic. Selective superior mesenteric angiography showed complete obliteration of the superior mesenteric artery with absence of venous return in 1 case. Three patients with massive intestinal necrosis died of multiorgan failure or the complications of short bowel syndrome. Histological
T
HE ANNUAL INCIDENCE of mesenteric infarction is approximately 1 in 100,000.1 Acute mesenteric vascular occlusion is associated with a high mortality rate. The age range generally is reported to be between 20 and 80 years, with most patients having an underlying disorder.2,3 Mesenteric ischemia and bowel infarction represent a broad spectrum of disease with diverse etiologies in elderly people.4,5 A primary or idiopathic form with no apparent cause also has been reported.3,6 Although very infrequently encountered, intestinal necrosis as a result of mesenteric vascular thrombosis also may be seen in childhood. Four patients with mesenteric vascular thrombosis with acute development of abdominal symptoms are presented. The aim of this study is to identify the mode of presentation, the underlying pathologies or factors that result in vascular thrombosis, the role of surgery in the treatment, and the life-saving principles of therapy. MATERIALS AND METHODS The records of patients with intestinal necrosis caused by mesenteric vascular thrombosis diagnosed between 1981 and 1996, inclusive, were reviewed. The ages at presentation, sex, clinical findings, laboratory investigations, diagnostic and therapeutic radiological Journal of Pediatric Surgery, Vol 35, No 8 (August), 2000: pp 1161-1164
examination of the resected intestinal segments showed the typical findings of polyarteritis nodosa in 2 patients. One patient had a previous history of right femoral vein thrombosis, whereas 1 patient had no known underlying disorders predisposing vascular thrombosis.
Conclusions: Mesenteric vascular occlusion is a rare but serious disease leading to death in children. The patients present with similar clinical signs, most frequent and important are acute abdominal pain, vomiting, and distension. Mesenteric vascular occlusion is a rare cause of acute abdomen in childhood, which requires urgent diagnosis and intervention. In suspected mesenteric vascular insufficiency, angiography should be performed followed by intraarterial thrombolytic infusion therapy in selected cases. When intestinal infarction is suspected, immediate surgical resection of compromised bowel is necessary with appropriate postoperative anticoagulation or treatment of any underlying disease. J Pediatr Surg 35:1161-1164. Copyright r 2000 by W.B. Saunders Company. INDEX WORDS: Mesenteric vascular occlusion, infarction, intestine.
approaches, surgical interventions, medical therapy, pathology, and outcome were analyzed retrospectively according to the special coding system that includes all the spectrum of disorders related to pediatric surgery in general headings.
RESULTS
Three boys and 1 girl underwent emergency surgery for intestinal infarction caused by mesenteric vascular thrombosis. The children ranged in age between 1 and 14 years with a mean age of 8.2 years. One patient had a spontaneous right femoral vein thrombosis that required thrombectomy 2 months before the present admission. The other 3 patients were healthy before presentation. The most striking and constant initial symptom was abdominal pain noted in all patients. The pain usually was intense, intermittent, and generalized. It was sudden onset (case 4) or prolonged for 24 hours to several days (3 From the Department of Pediatric Surgery, Hacettepe University Medical Faculty, Ankara, Turkey. Address reprint requests to Mehmet Emin S¸enocak, Kehribar sokak No: 9/46, 06700 C ¸ ankaya, Ankara, Turkey. Copyright r 2000 by W.B. Saunders Company 0022-3468/00/3508-0003$03.00/0 doi:10.1053/jpsu.2000.8718 1161
˘ UZKURT ET AL OG
1162
patients). The accompanying symptoms were vomiting, bloody stools, and diarrhea. The clinical manifestations were abdominal distension and tenderness, tachycardia, petechial lesions, mild cardiac murmur, and fever (Table 1). Nonspecific laboratory changes were observed. In case 4, the protein C and S levels were decreased transiently (3 mg/L and 20 mg/L, respectively) and returned to normal levels in the first postoperative week, but antithrombin III level was normal. Therefore, it was regarded as a result of consumption during thrombotic process. Abdominal radiographs showed dilated, thickened bowel loops and air-fluid levels in 2 patients and free peritoneal air in 1 child. In 2 patients, abdominal ultrasonography was performed that showed dilated bowel loops accompanied by increased amount of peritoneal fluid. In case 4, selective superior mesenteric angiography showed complete obliteration of the superior mesenteric artery (SMA) beyond 3 cm with absence of venous return (Fig 1). The colon was vascularized through the retrograde flow of middle colic vessels. The catheter could not be advanced, and balloon embolectomy was considered to be impossible. Therefore, a bolus of 100,000 IU urokinase was injected intraarterially, and it was followed by an infusion of 4,000 IU/min over 60 minutes. Repeated angiography showed no passage. High-dose urokinase application failed to revascularize the SMA. All patients underwent emergency laparotomy. Massive gangrene of the small bowel was found in 3 patients, and 2 necrotic jejunal segments with 10 and 25 cm in length were noted in 1 patient. Histological examination of the resected segments showed hemorrhagic necrosis
and patchy ulcerations of the intestinal mucosa, inflammatory exudates in some portions of serosa, and hemorrhage and edema in the submucosa. Fibrin thrombi were identified in the small vessels of mesentery and in the superior mesenteric vein branches. Vascular lesions including fibrosis, obliteration, and polymorphonuclear cell infiltration of the vascular walls were consistent with polyarteritis nodosa (PAN) in 2 patients (Table 1). Case 1 had not been treated previously with anticoagulants after the diagnosis of femoral vein thrombosis in another center, and the anticoagulant agent was started after the first abdominal operation. However, after the initial bowel resection, the patient has undergone 2 consecutive operations within 48 hours because of progressive bowel necrosis. The patient in case 4 also received long-term anticoagulant therapy postoperatively. Three patients with massive intestinal necrosis died of short bowel syndrome and infection or multiorgan failure caused by systemic manifestations of PAN. The surviving patient was put on medical treatment for the systemic manifestations of PAN after the operation. DISCUSSION
Bowel infarction caused by mesenteric vascular occlusion is a lethal condition.6 The mortality rate is reported to be 60% to 80% in adult series.4 Mesenteric ischemia represents a broad spectrum of diseases with various etiologies.7 Mesenteric vascular thrombosis has been reported in association with a number of disease processes.5 These include hereditary and acquired thrombotic disorders.8 There are certain clinical indicators of
Table 1. Clinical Data of the Patients Case 1
Age (yr)/sex Symptoms and signs Length of history (d) Physical findings Previous medical history Radiological investigations Angiography
1/M Abdominal pain, vomiting, bloody stool 1 Abdominal distension and tenderness Right femoral vein thrombosis Air-fluid levels No
Case 2
Case 3
10/F Abdominal pain, cutaneous lesions, seizures 2 Abdominal distension and tenderness Not remarkable
8/M Abdominal pain, vomiting, purpuric lesions 1 Abdominal distension and rigidity Not remarkable
14/M Abdominal pain, vomiting 1 Abdominal distension and tenderness Not remarkable
Air-fluid levels, dilated bowel loops No
Free peritoneal air
2 segments of jejunum (10 and 25 cm in length) Small mesenteric artery branches Fibrinoid necrosis in the walls of medium and small arteries (PAN) Survived
Dilated bowel loops, airfluid levels Complete obliteration of SMA Whole jejunum and ileum
Sites of the lesions
Segments of jejunum and ileum
Whole ileum
Thrombosed vessels
Superior mesenteric vein branches Fibrin thrombi in the small vessels of mesentery Exitus (postoperative third day)
Small vessels of the mesentery Necrotizing vasculitis (PAN)
Pathology
Outcome
Exitus (postoperative second day)
No
Case 4
Superior mesenteric artery and vein Fresh thrombus in SMA and SMV Exitus (postoperative third month)
MESENTERIC VASCULAR OCCLUSION
Fig 1. Selective superior mesenteric angiography show complete obliteration of the SMA beyond 3 cm (arrow).
hypercoagulable state.9 Therefore, the detailed history should be obtained for a family history of thrombosis, recurrent spontaneous thrombosis, and thrombosis at an early age and in unusual sites. Resistance to anticoagulant therapy and migratory superficial thrombophlebitis also should raise the suspicion of a primary hypercoagulable state. More sophisticated and detailed laboratory findings that may indicate the presence of thromboembolism include detection of platelet-specific marker proteins, increased platelet turnover, increased platelet procoagulantactivity, reduced fibrinolytic activity, circulating fibrin monomer complexes, prothrombin fragment 1,2, abnormal levels of fibrinopeptide A, thrombin-antithrombin III and plasmin- antiplasmin complexes, and abnormal levels of fibrin degradation products.9 Acquired thrombotic disorders can be called risk factors.8 Vascular endothelial abnormalities and cardiac diseases, diabetes mellitus, vasculitis (polyarteritis nodosa), artificial surfaces (prosthetic valves, vascular grafts), trauma, and surgery are the risk factors for the development of thrombotic processes.1,3,5,8,10,11 A primary or idiopathic form of mesenteric vascular thrombosis also can occur in young people without any underlying disorder.3,5,7,12 There are a few cases of mesenteric venous thrombosis in the pediatric age group. Eight cases were reported in a review from 1900 to 1976.5 The age range of these children was 4 days to 14 years. Although 7 patients had a predisposing factors for vascular disease, 1 patient had no underlying disease. The major difficulty is the lack of definitive diagnostic and laboratory tests.13 The biochemical abnormalities usually occur late in the course of the disease, when necrosis already has developed. In suspected cases of
1163
acute mesenteric ischemia, complete blood count, blood pH and blood gas content analysis, and blood biochemistry results should be obtained as soon as possible.13 Coagulation tests including coagulation factors, protein C and S, antithrombin III, prothrombin time and partial thromboplastin time, fibrinogen level, and fibrin degradation products also should be added to the investigations. Complete physical examination, especially the vital signs and abdominal findings, are important in the initial evaluation. Nonspecific bowel gas and air-fluid levels are seen in the abdominal radiographs.2 Marked reduction of air or gasless abdomen may be noted in the early stage of infarction as well.2,11 Computed tomography (CT) scans and ultrasound scans may show thickened bowel loops which is caused by venous thrombosis. CT also may show pneumatosis intestinalis, thumbprinting of the small bowel, and mesenteric or portal venous gas, but both techniques only give clues for infarcted or ischemic bowel.2,7 Although CT could not be able to give a specific diagnosis in cases of mesenteric infarction secondary to arterial occlusion, it may be diagnostic tool for cases of mesenteric venous thrombosis.7 Angiography has been used as a diagnostic and therapeutic procedure, especially for arterial occlusion. Angiography shows spasm of arteries, diminished or absent blood flow in affected bowel wall, and absence of venous drainage.5 Treatment of superior mesenteric artery (SMA) thrombosis or thromboembolism by intraarterial infusion of urokinase or streptokinase by local high-dose or selective low-dose therapy have been reported to result in successful thrombolysis.14,15 In children, mesenteric vascular thrombosis may occur both in idiopathic form or in association with a predisposing disease. In our series, 3 patients had underlying disorders: polyarteritis nodosa in 2 and previous history of deep venous thrombosis in the other. In case 1, deep venous thrombosis preceeded the progressive thrombosis of mesenteric vessels resulting in infarction of almost the whole small intestine. In this patient there was no documentation of the coagulation profile to find out whether a coagulation disorder such as antithrombin III or protein C deficiency was present.5,12 The patient may have represented a type of hereditary thrombotic disorder (a primary hypercoagulable state) or spontaneous thrombosis in which a previous history of peripheral venous thrombosis of the extremities and migratory thrombophlebitis is not unusual, but no exact cause can be found.5,8,12 The patients in case 2 and 3 have acquired thrombotic disorder in which the risk factor was a specific type of vasculitis: polyarteritis nodosa.16 In PAN the fibrinoid necrosis in the walls of medium- and small-sized arteries in addition to obliteration and fibrosis of the vessels result in bowel infarction. PAN rarely involves the gastrointesti-
˘ UZKURT ET AL OG
1164
nal system in childhood, and only a few cases have been reported with an initial presentation of mesenteric thrombosis.17 In cases 2 and 3, PAN was documented in the histological examination of the resected segments. Both patients presented with complications related to gastrointestinal involvement and could be diagnosed after laparotomy. In case 4, there are no identifiable risk factors as well as underlying hereditary thrombotic disorders. Therefore, the patient was regarded to have idiopathic thrombotic process. We can not say that all of our cases are examples of venous thrombosis. Excluding the cases 2 and 3 of PAN, the others were considered to be mesenteric venous thrombosis resulting in arterial occlusion. In cases 2 and 3, the initial occlusive mechanism was the fibrinoid necrosis of small- and medium-sized arteries. Whatever the cause of thrombosis, the obliteration of the mesenteric veins or venules result in hypovolemia and hemoconcentration that causes reduced flow in the SMA with arterial spasm and led to arterial thrombosis. Irreversible necrosis occurs at about 14 hours.5 Mesenteric vascular occlusion is a rare but serious disease in childhood leading to death. The patients may
present with diverse clinical signs and symptoms often suggestive of intestinal obstruction rather than mesenteric infarction. It must be borne in mind in cases of acute abdomen with or without systemic manifestations because of its rapid and usually fatal outcome. Selective SMA angiography is the most reliable diagnostic procedure in suspected cases. Thrombolysis of the SMA thrombosis through intraarterial infusion of urokinase or streptokinase may result in revascularization of the small intestine. Angiographic demonstration of the arterial blood flow with venous return in the small mesenteric vessels also is the superior monitorization method for the intestinal viability. Therefore, patency should be checked 30 minutes after intraarterial infusion of thrombolytic agent with an opaque injection associated with power Doppler ultrasound scan. If repeated angiography shows occlusion, laparotomy should be performed urgently. Surgical resection of necrotic bowel and anticoagulant therapy to control progressive vascular thrombosis should be carried out without delay. Time-consuming radiological and laboratory investigations should be avoided.
REFERENCES 1. Singh RP, Lee STJ: Acute mesenteric vascular occlusion: A review of 40 cases. Int Surg 65:231-234, 1980 2. Nozaki H, Kohno A, Narimatsu A, et al: Superior mesenteric artery occlusion: An enhanced CT finding. J Comput Assist Tomogr 15:866-867, 1991 3. Nair HT, Muscroft TJ: Idiopathic mesenteric thrombosis. Br J Clin Pract 44:651-652, 1990 4. Inderbitzi R, Wagner HE, Seiler C, et al: Acute mesenteric ischemia. Eur J Surg 158:123-126, 1992 5. Grendell JH, Ockner RK: Mesenteric venous thrombosis. Gastroenterology 82:358-372, 1982 6. Rogers MD, Thompson JE, Garret WV, et al: Mesenteric vascular problems. Ann Surg 195:554-565, 1982 7. Kim JY, Ha HK, Kyun JY, et al: Intestinal infarction secondary to mesenteric venous thrombosis: CT pathologic correlation. J Comput Assist Tomogr 17:382-385, 1993 8. Lee GR, Bithell TC, Foerster J, et al: Wintrobe’s Clinical Hematology. Philadelphia, PA, Lea & Febiger, 1993, p 1521 9. Lee GR, Bithell TC, Foerster J, et al: Wintrobe’s Clinical Hematology. Philadelphia, PA, Lea & Febiger, 1993, p 1524 10. Tsai CJ, Kuo YC, Chen PC, et al: The spectrum of acute
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