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Spontaneous intramural duodenal hematoma resulting from Glanzmann's thrombasthenia
J Ped Surg Case Reports 27 (2017) 61e63
Contents lists available at ScienceDirect
Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com
Spontaneous intramural duodenal hematoma resulting from Glanzmann’s thrombasthenia Soo-Hong Kim, Yong-Hoon Cho*, Hae-Young Kim Department of Pediatric Surgery, Pusan National University Children’s Hospital, South Korea
a r t i c l e i n f o
a b s t r a c t
Article history: Received 30 August 2017 Received in revised form 15 September 2017 Accepted 17 September 2017
Glanzmann’s thrombasthenia (GT) is a very rare platelet disorder and could cause spontaneous bleeding. Without known past medical history about bleeding, diagnosing this condition may be difficult. It commonly presents with mucocutaneous bleeding, but can also cause other types of bleeding. Intramural duodenal hematoma (IDH) is an uncommon medical condition generally accompanying with certain predisposing factors. Diagnosing IDH is challenging particularly when atypical image findings were observed with unknown risk factors. This is a case report of a 9-year-old boy with spontaneous IDH followed by serious complications due to unrecognized GT. Ó 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Keywords: Intramural duodenal hematoma Glanzmann’s thrombasthenia Coagulation Bleeding Children
Unrecognized underlying diseases could affect the patient’s clinical courses. The initial clinical impression may influence not only the diagnostic approach but the management plan and complications. Intramural duodenal hematoma (IDH) is an uncommon medical condition generally accompanying with certain predisposing factors, such as trauma, anticoagulation therapy, and bleeding disorder [1e3]. Glanzmann’s thrombasthenia (GT) is an autosomal recessive or autoimmune disease manifesting as a platelet dysfunction caused by a deficiency or abnormality of the membrane glycoprotein IIbeIIIa complex and could result in a faulty aggregation of platelet and diminished clot retraction [4,5]. Diagnosing IDH is challenging particularly in a clinical situation with only atypical imaging findings and unrecognized risk factors such as in this case [6]. Here, a spontaneous IDH case is reported followed by serious complications due to undiagnosed GT with a brief literature review. 1. Case report A 9-year-old boy was referred to us for a 2-day history of recurrent vomiting, epigastric pain, and mass at the duodenum or pancreas on ultrasonography (US). Could not identify any specific
* Corresponding author. Department of Surgery, Pusan National University School of Medicine, Geumo-ro 20, Mulgeum-eup, Yangsan, Gyeongnam, 50612, South Korea. E-mail address: [email protected] (Y.-H. Cho).
medical history, medications, and trauma; however, his father suffered from Crohn’s disease for 10 years. Physical examination showed a mild tenderness at the epigastric area and right upper quadrant of the abdomen. Laboratory results showed normal range other than the increased serum amylase and lipase level, 309 (normal range, 22e80) IU/L and 312 (normal range, 5e31) IU/L, respectively. Radiologic studies, US and computed tomography (CT), revealed a 7.0 4.9 4.0 cm-sized soft tissue attenuating mass at the right side of the duodenal second portion, causing duodenal obstruction and probable pancreatitis (Figs. 1 and 2). Subsequent endoscopic examination showed a round erythematous intraluminal mass obstructing the duodenal second portion (Fig. 3). These findings suggested a duodenal submucosal tumor, including lymphoma, gastrointestinal stromal tumor, etc. Initially, an endoscopic and ultrasonography-guided biopsy was performed and revealed chronic inflammation and hemorrhage only. Preferentially, a nasojejunal tube insertion was attempted but failed. Despite of the conservative management including nil per oral, nasogastric tube insertion, and intravenous fluid for 7 days, he consistently suffered from vomiting and pain. Since the diagnosis was equivocal, surgical removal and biopsy were decided. The operative findings revealed about 7 4-cm-sized round mass along the lateral border of the duodenum extending from the second to fourth portion. After the complete removal of the mass, Roux-en-Y duodenojejunostomy was performed because the defect of the duodenal wall was too large to close. It was pathologically confirmed as a simple IDH. Symptoms and laboratory findings
2213-5766/Ó 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). https://doi.org/10.1016/j.epsc.2017.09.016
62
S.-H. Kim et al. / J Ped Surg Case Reports 27 (2017) 61e63
Fig. 1. Ultrasound image showing a heterogeneous echoic mass in the duodenum (arrows). Note the internal low echoic portion that may suggest internal necrosis or hemorrhage (asterisk).
improved shortly after the operation, but they worsened again after 4 days. The US and CT revealed a 4.6 3.2-cm-sized new hematoma, swelling of the anastomotic site, and pancreatic duct dilatation. During this stage, the patient manifested spontaneous multiple bruises in his legs. Laboratory tests including platelet aggregation test with light transmission aggregometry were performed. In two consecutive platelet aggregation tests, the amplitudes of aggregation significantly dropped at the arachidonic acid, collagen, adenosine diphosphate (ADP), and epinephrine, but relatively unchanged at the ristocetin. Other tests did not show any abnormal results. He was diagnosed with a GT. The hematoma was controlled with percutaneous external drainage after platelet transfusion. However, pancreatitis and pancreatic duct dilatation were unimproved, even after 8 weeks of treatment. Additionally, stent insertion to the pancreatic duct by an endoscopic retrograde cholangiopancreatography failed due to limited working space. Inevitably, second operation was scheduled to resolve the problem. A sphincteroplasty with stenting of the pancreatic duct was performed. One week after, the stent could be removed and he recovered well without further medical problems. 2. Discussion IDH is generally characterized with abdominal pain and signs of intestinal obstruction, such as vomiting and tenderness. Jaundice and pancreatitis could also be observed due to extrinsic compression of the hematoma [2,6,7]. The followings are some etiologic
Fig. 3. Endoscopic findings: Round erythematous mass obstructing duodenum second portion.
factors causing an IDH: trauma, anticoagulation therapy, pancreatitis, bleeding disorders, malignancy, and endoscopy; however, trauma and anticoagulation therapy are the most common cause in pediatric and adult patients, respectively [5]. Without knowing the risk factors, diagnosing IDH is sometimes challenging [6]. In case of the stable condition of, conservative treatments including bowel rest, nasogastric tube insertion, correction of coagulopathy, and intravenous fluids are usually recommended. Otherwise, surgical intervention is necessary in case of significant intraluminal hemorrhage, bowel perforation, ischemia, and unresolved abdominal pain or obstruction in spite of conservative therapy. For unresolved cases without complication, removal of the hematoma is known as the best surgical option. Percutaneous aspiration and endoscopic dilatation could also be used [2,3,8]. GT is a very rare disease with an estimated prevalence of one per a million. Without knowing the past medical history about bleeding, diagnosing GT could not be achieved. The majority of GT patients are diagnosed before 5 years-old, because of recurrent episodes of epistaxis, gingival bleeding and bruising. Occasionally, it should be considered as a diagnosis when unexpected excessive bleeding occurs after surgery or minor trauma, and a menorrhagia requiring transfusion develops in female without medical history [5]. It usually causes bleeding with predisposing conditions. However, hemorrhage spontaneously occurring in the viscera is a rare clinical presentation, such as in this report [1]. Most hematologic studies reveal normal ranges, except a prolonged bleeding time only. Light transmission aggregometry is used as the gold standard diagnostic tool for assessing platelet function, which is highly
Fig. 2. Contrast-enhanced CT axial (A) and coronal (B) images revealing the hypoattenuation mass involving second portion of the duodenum. Note the elongated shape of the mass and a small mottled air in the duodenum (arrowhead) on the coronal image.
S.-H. Kim et al. / J Ped Surg Case Reports 27 (2017) 61e63
specific for GT. Platelet function analyzer, flow cytometry, and mutation analysis are also useful diagnostic tests [5]. In this case, some factors interfered with the diagnosis of IDH and GT. First, the symptoms and signs of bleeding disorder could not be identified at the initial assessment, because the patient and parents denied its occurrence. Otherwise, we could have paid more attention to the possibility of bleeding disorder, which would lead to easier diagnosis and earlier implementation of initial appropriate management. Then, more extensive procedures could have been avoided. Second, radiologic findings were atypical. The US and CT are known as the proper imaging modalities for IDH [2]. US findings usually revealed various, nonspecific findings and described thickening and hypoechogenicity of the duodenal wall, bowel-related mass, or variable echogenic cystic lesion in the expected location [9]. CT findings also revealed circumferential bowel wall thickening, intramural hyperdensity, luminal narrowing and intestinal obstruction [2]. Meanwhile, in this case, the US and CT revealed hypovascular soft tissue attenuation and poorly enhanced density lesion with inner necrotic portion, but not a cystic mass or intramural hyperdensity. Pre-contrast enhanced CT images might be helpful to distinguish hematoma and mass; however, this may also increase the radiation hazards. Finally, a possibility of malignant duodenal lesion was highly considered. It was attributed to soft -tissue-like imaging findings and lack of other risk factors. Moreover, the possibility of GT was not assumed, so the differential diagnosis was focused on lymphoma and gastrointestinal stromal tumor, instead of IDH. However, duodenal malignancies are extremely rare in pediatric patients [10]. IDH is a more common disease than duodenal malignancies. Therefore, more common diseases should be considered first. In conclusion, when atypical duodenal mass is encountered in pediatric patients, IDH should be considered as a differential
63
diagnosis even if it is rare. Additionally, finding any possible causes, i.e. bleeding disorder such as a GT, should also be considered. Careful history taking and whole-body physical examination are primarily essential to identify the undiagnosed risk factors, which can probably cause spontaneous bleeding. Conflict of interest All the authors have no conflict of interest to declare. No financial relationship to disclose. References [1] DeRose Jr JJ, Diamond S, Bergman K. Spontaneous duodenal hematoma in a patient with Glanzmann’s thrombasthenia. J Pediatr Surg 1997;32:1341e3. [2] Eichele DD, Ross M, Tang P, Hutchins GF, Mailliard M. Spontaneous intramural duodenal hematoma in type 2B von Willebrand disease. World J Gastroenterol 2013;19:7205e8. [3] Elmoghazy W, Noaman I, Mahfouz AE, Elaffandi A, Khalaf H. Surgical management of complicated intra-mural duodenal hematoma: a case-report and review of literature. Int J Surg Case Rep 2015;17:103e5. [4] Rawal A, Sarode R, Curtis BR, Karandikar NJ, Friedman K, Rogers ZR. Acquired Glanzmann’s thrombasthenia as part of multiple-autoantibody syndrome in a pediatric heart transplant patient. J Pediatr 2004;144:672e4. [5] Solh T, Botsford A, Solh M. Glanzmann’s thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med 2015;6: 219e27. [6] Wong K, Thomas R. A curious case of spontaneous duodenal haematoma presenting a diagnostic challenge. BMJ Case Rep 2014;2014. [7] Moore SW, Erlandson ME. Intramural hematoma of the duodenum. Ann Surg 1963;157:798e807. [8] Furukawa K, Iida T, Shiba H, Akita H, Sasaki M, Yanaga K. Nontraumatic intramural hematoma of the duodenum. Clin J Gastroenterol 2010;3:22e4. [9] Ghersin E, Gaitini D, Wills O, Soudack M, Engel A. Intramural duodenal hematoma mimicking an intestinal mass on sonography. J Ultrasound Med 2002; 21:693e5. [10] Roy S, Raskin L, Raymond VM, Thibodeau SN, Mody RJ, Gruber SB. Pediatric duodenal cancer and biallelic mismatch repair gene mutations. Pediatr Blood Cancer 2009;53:116e20.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com
Spontaneous intramural duodenal hematoma resulting from Glanzmann’s thrombasthenia Soo-Hong Kim, Yong-Hoon Cho*, Hae-Young Kim Department of Pediatric Surgery, Pusan National University Children’s Hospital, South Korea
a r t i c l e i n f o
a b s t r a c t
Article history: Received 30 August 2017 Received in revised form 15 September 2017 Accepted 17 September 2017
Glanzmann’s thrombasthenia (GT) is a very rare platelet disorder and could cause spontaneous bleeding. Without known past medical history about bleeding, diagnosing this condition may be difficult. It commonly presents with mucocutaneous bleeding, but can also cause other types of bleeding. Intramural duodenal hematoma (IDH) is an uncommon medical condition generally accompanying with certain predisposing factors. Diagnosing IDH is challenging particularly when atypical image findings were observed with unknown risk factors. This is a case report of a 9-year-old boy with spontaneous IDH followed by serious complications due to unrecognized GT. Ó 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Keywords: Intramural duodenal hematoma Glanzmann’s thrombasthenia Coagulation Bleeding Children
Unrecognized underlying diseases could affect the patient’s clinical courses. The initial clinical impression may influence not only the diagnostic approach but the management plan and complications. Intramural duodenal hematoma (IDH) is an uncommon medical condition generally accompanying with certain predisposing factors, such as trauma, anticoagulation therapy, and bleeding disorder [1e3]. Glanzmann’s thrombasthenia (GT) is an autosomal recessive or autoimmune disease manifesting as a platelet dysfunction caused by a deficiency or abnormality of the membrane glycoprotein IIbeIIIa complex and could result in a faulty aggregation of platelet and diminished clot retraction [4,5]. Diagnosing IDH is challenging particularly in a clinical situation with only atypical imaging findings and unrecognized risk factors such as in this case [6]. Here, a spontaneous IDH case is reported followed by serious complications due to undiagnosed GT with a brief literature review. 1. Case report A 9-year-old boy was referred to us for a 2-day history of recurrent vomiting, epigastric pain, and mass at the duodenum or pancreas on ultrasonography (US). Could not identify any specific
* Corresponding author. Department of Surgery, Pusan National University School of Medicine, Geumo-ro 20, Mulgeum-eup, Yangsan, Gyeongnam, 50612, South Korea. E-mail address: [email protected] (Y.-H. Cho).
medical history, medications, and trauma; however, his father suffered from Crohn’s disease for 10 years. Physical examination showed a mild tenderness at the epigastric area and right upper quadrant of the abdomen. Laboratory results showed normal range other than the increased serum amylase and lipase level, 309 (normal range, 22e80) IU/L and 312 (normal range, 5e31) IU/L, respectively. Radiologic studies, US and computed tomography (CT), revealed a 7.0 4.9 4.0 cm-sized soft tissue attenuating mass at the right side of the duodenal second portion, causing duodenal obstruction and probable pancreatitis (Figs. 1 and 2). Subsequent endoscopic examination showed a round erythematous intraluminal mass obstructing the duodenal second portion (Fig. 3). These findings suggested a duodenal submucosal tumor, including lymphoma, gastrointestinal stromal tumor, etc. Initially, an endoscopic and ultrasonography-guided biopsy was performed and revealed chronic inflammation and hemorrhage only. Preferentially, a nasojejunal tube insertion was attempted but failed. Despite of the conservative management including nil per oral, nasogastric tube insertion, and intravenous fluid for 7 days, he consistently suffered from vomiting and pain. Since the diagnosis was equivocal, surgical removal and biopsy were decided. The operative findings revealed about 7 4-cm-sized round mass along the lateral border of the duodenum extending from the second to fourth portion. After the complete removal of the mass, Roux-en-Y duodenojejunostomy was performed because the defect of the duodenal wall was too large to close. It was pathologically confirmed as a simple IDH. Symptoms and laboratory findings
2213-5766/Ó 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). https://doi.org/10.1016/j.epsc.2017.09.016
62
S.-H. Kim et al. / J Ped Surg Case Reports 27 (2017) 61e63
Fig. 1. Ultrasound image showing a heterogeneous echoic mass in the duodenum (arrows). Note the internal low echoic portion that may suggest internal necrosis or hemorrhage (asterisk).
improved shortly after the operation, but they worsened again after 4 days. The US and CT revealed a 4.6 3.2-cm-sized new hematoma, swelling of the anastomotic site, and pancreatic duct dilatation. During this stage, the patient manifested spontaneous multiple bruises in his legs. Laboratory tests including platelet aggregation test with light transmission aggregometry were performed. In two consecutive platelet aggregation tests, the amplitudes of aggregation significantly dropped at the arachidonic acid, collagen, adenosine diphosphate (ADP), and epinephrine, but relatively unchanged at the ristocetin. Other tests did not show any abnormal results. He was diagnosed with a GT. The hematoma was controlled with percutaneous external drainage after platelet transfusion. However, pancreatitis and pancreatic duct dilatation were unimproved, even after 8 weeks of treatment. Additionally, stent insertion to the pancreatic duct by an endoscopic retrograde cholangiopancreatography failed due to limited working space. Inevitably, second operation was scheduled to resolve the problem. A sphincteroplasty with stenting of the pancreatic duct was performed. One week after, the stent could be removed and he recovered well without further medical problems. 2. Discussion IDH is generally characterized with abdominal pain and signs of intestinal obstruction, such as vomiting and tenderness. Jaundice and pancreatitis could also be observed due to extrinsic compression of the hematoma [2,6,7]. The followings are some etiologic
Fig. 3. Endoscopic findings: Round erythematous mass obstructing duodenum second portion.
factors causing an IDH: trauma, anticoagulation therapy, pancreatitis, bleeding disorders, malignancy, and endoscopy; however, trauma and anticoagulation therapy are the most common cause in pediatric and adult patients, respectively [5]. Without knowing the risk factors, diagnosing IDH is sometimes challenging [6]. In case of the stable condition of, conservative treatments including bowel rest, nasogastric tube insertion, correction of coagulopathy, and intravenous fluids are usually recommended. Otherwise, surgical intervention is necessary in case of significant intraluminal hemorrhage, bowel perforation, ischemia, and unresolved abdominal pain or obstruction in spite of conservative therapy. For unresolved cases without complication, removal of the hematoma is known as the best surgical option. Percutaneous aspiration and endoscopic dilatation could also be used [2,3,8]. GT is a very rare disease with an estimated prevalence of one per a million. Without knowing the past medical history about bleeding, diagnosing GT could not be achieved. The majority of GT patients are diagnosed before 5 years-old, because of recurrent episodes of epistaxis, gingival bleeding and bruising. Occasionally, it should be considered as a diagnosis when unexpected excessive bleeding occurs after surgery or minor trauma, and a menorrhagia requiring transfusion develops in female without medical history [5]. It usually causes bleeding with predisposing conditions. However, hemorrhage spontaneously occurring in the viscera is a rare clinical presentation, such as in this report [1]. Most hematologic studies reveal normal ranges, except a prolonged bleeding time only. Light transmission aggregometry is used as the gold standard diagnostic tool for assessing platelet function, which is highly
Fig. 2. Contrast-enhanced CT axial (A) and coronal (B) images revealing the hypoattenuation mass involving second portion of the duodenum. Note the elongated shape of the mass and a small mottled air in the duodenum (arrowhead) on the coronal image.
S.-H. Kim et al. / J Ped Surg Case Reports 27 (2017) 61e63
specific for GT. Platelet function analyzer, flow cytometry, and mutation analysis are also useful diagnostic tests [5]. In this case, some factors interfered with the diagnosis of IDH and GT. First, the symptoms and signs of bleeding disorder could not be identified at the initial assessment, because the patient and parents denied its occurrence. Otherwise, we could have paid more attention to the possibility of bleeding disorder, which would lead to easier diagnosis and earlier implementation of initial appropriate management. Then, more extensive procedures could have been avoided. Second, radiologic findings were atypical. The US and CT are known as the proper imaging modalities for IDH [2]. US findings usually revealed various, nonspecific findings and described thickening and hypoechogenicity of the duodenal wall, bowel-related mass, or variable echogenic cystic lesion in the expected location [9]. CT findings also revealed circumferential bowel wall thickening, intramural hyperdensity, luminal narrowing and intestinal obstruction [2]. Meanwhile, in this case, the US and CT revealed hypovascular soft tissue attenuation and poorly enhanced density lesion with inner necrotic portion, but not a cystic mass or intramural hyperdensity. Pre-contrast enhanced CT images might be helpful to distinguish hematoma and mass; however, this may also increase the radiation hazards. Finally, a possibility of malignant duodenal lesion was highly considered. It was attributed to soft -tissue-like imaging findings and lack of other risk factors. Moreover, the possibility of GT was not assumed, so the differential diagnosis was focused on lymphoma and gastrointestinal stromal tumor, instead of IDH. However, duodenal malignancies are extremely rare in pediatric patients [10]. IDH is a more common disease than duodenal malignancies. Therefore, more common diseases should be considered first. In conclusion, when atypical duodenal mass is encountered in pediatric patients, IDH should be considered as a differential
63
diagnosis even if it is rare. Additionally, finding any possible causes, i.e. bleeding disorder such as a GT, should also be considered. Careful history taking and whole-body physical examination are primarily essential to identify the undiagnosed risk factors, which can probably cause spontaneous bleeding. Conflict of interest All the authors have no conflict of interest to declare. No financial relationship to disclose. References [1] DeRose Jr JJ, Diamond S, Bergman K. Spontaneous duodenal hematoma in a patient with Glanzmann’s thrombasthenia. J Pediatr Surg 1997;32:1341e3. [2] Eichele DD, Ross M, Tang P, Hutchins GF, Mailliard M. Spontaneous intramural duodenal hematoma in type 2B von Willebrand disease. World J Gastroenterol 2013;19:7205e8. [3] Elmoghazy W, Noaman I, Mahfouz AE, Elaffandi A, Khalaf H. Surgical management of complicated intra-mural duodenal hematoma: a case-report and review of literature. Int J Surg Case Rep 2015;17:103e5. [4] Rawal A, Sarode R, Curtis BR, Karandikar NJ, Friedman K, Rogers ZR. Acquired Glanzmann’s thrombasthenia as part of multiple-autoantibody syndrome in a pediatric heart transplant patient. J Pediatr 2004;144:672e4. [5] Solh T, Botsford A, Solh M. Glanzmann’s thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med 2015;6: 219e27. [6] Wong K, Thomas R. A curious case of spontaneous duodenal haematoma presenting a diagnostic challenge. BMJ Case Rep 2014;2014. [7] Moore SW, Erlandson ME. Intramural hematoma of the duodenum. Ann Surg 1963;157:798e807. [8] Furukawa K, Iida T, Shiba H, Akita H, Sasaki M, Yanaga K. Nontraumatic intramural hematoma of the duodenum. Clin J Gastroenterol 2010;3:22e4. [9] Ghersin E, Gaitini D, Wills O, Soudack M, Engel A. Intramural duodenal hematoma mimicking an intestinal mass on sonography. J Ultrasound Med 2002; 21:693e5. [10] Roy S, Raskin L, Raymond VM, Thibodeau SN, Mody RJ, Gruber SB. Pediatric duodenal cancer and biallelic mismatch repair gene mutations. Pediatr Blood Cancer 2009;53:116e20.