Pub Date : 2024-04-01DOI: 10.1097/jova.0000000000000084
Michael Mohnasky, Jeyhan Wood, Elizabeth Nieman, Jennifer Brondon, Kamran Khan, Kyung Rae Kim
Pyogenic granulomas are common, acquired vascular lesions that most often arise spontaneously or in sites of previous trauma. However, there are reports of a few cases that describe a rare condition, congenital disseminated pyogenic granuloma (CDPG), in which an infant either is born with or shortly after birth develops multiple pyogenic granulomas. A hallmark of CDPG is negative staining for glucose transporter 1 (GLUT-1) on immunohistochemistry, which helps distinguish it from the more common multifocal infantile hemangiomas. Because few case reports have described CDPG, much is unknown about its characteristics, clinical course, and most effective treatment options. Here, we present a case of an infant with a unique presentation of CDPG with lesions that are atypically large and growing at a rapid pace. We also describe a novel approach to treating large pyogenic granulomas in CDPG via staged glue embolization and surgical excision.
{"title":"Rapidly Growing Congenital Disseminated Pyogenic Granuloma in the Scalp Treated with Staged Embolization and Excision: A Case Report","authors":"Michael Mohnasky, Jeyhan Wood, Elizabeth Nieman, Jennifer Brondon, Kamran Khan, Kyung Rae Kim","doi":"10.1097/jova.0000000000000084","DOIUrl":"https://doi.org/10.1097/jova.0000000000000084","url":null,"abstract":"Pyogenic granulomas are common, acquired vascular lesions that most often arise spontaneously or in sites of previous trauma. However, there are reports of a few cases that describe a rare condition, congenital disseminated pyogenic granuloma (CDPG), in which an infant either is born with or shortly after birth develops multiple pyogenic granulomas. A hallmark of CDPG is negative staining for glucose transporter 1 (GLUT-1) on immunohistochemistry, which helps distinguish it from the more common multifocal infantile hemangiomas. Because few case reports have described CDPG, much is unknown about its characteristics, clinical course, and most effective treatment options. Here, we present a case of an infant with a unique presentation of CDPG with lesions that are atypically large and growing at a rapid pace. We also describe a novel approach to treating large pyogenic granulomas in CDPG via staged glue embolization and surgical excision.","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"122 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140762182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-04-01DOI: 10.1097/jova.0000000000000087
Olivia A. Keane, Carolyn Taylor, Shayla Bergmann
Kaposiform hemangioendothelioma (KHE) is a rare vascular and lymphatic tumor of childhood that commonly presents on the skin and extremities. KHE of the mediastinum affecting the heart and great vessels is extremely rare and often locally aggressive. We describe our case of mediastinal KHE with Kasabach–Merritt phenomenon presenting in a 2-month-old with persistent pericardial effusion and thrombocytopenia in the absence of cutaneous findings. Treatment success was achieved with sirolimus, propranolol, and an initial prednisolone wean.
{"title":"Kaposiform Hemangioendothelioma of the Mediastinum With Kasabach-Merritt Phenomenon Presenting With Pericardial Effusion in a 2-Month-Old","authors":"Olivia A. Keane, Carolyn Taylor, Shayla Bergmann","doi":"10.1097/jova.0000000000000087","DOIUrl":"https://doi.org/10.1097/jova.0000000000000087","url":null,"abstract":"Kaposiform hemangioendothelioma (KHE) is a rare vascular and lymphatic tumor of childhood that commonly presents on the skin and extremities. KHE of the mediastinum affecting the heart and great vessels is extremely rare and often locally aggressive. We describe our case of mediastinal KHE with Kasabach–Merritt phenomenon presenting in a 2-month-old with persistent pericardial effusion and thrombocytopenia in the absence of cutaneous findings. Treatment success was achieved with sirolimus, propranolol, and an initial prednisolone wean.","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"49 26","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140788672","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-03-18DOI: 10.1097/jova.0000000000000085
Michael T. Dellinger, T. Ferry, David Williams, Tracy Milne, Louise Murgia, Michael Kelly
Complex lymphatic anomalies (CLAs) are rare diseases with variable clinical manifestations caused by the abnormal development of lymphatic vessels. The Lymphatic Malformation Institute (LMI), Lymphangiomatosis & Gorham’s Disease Alliance (LGDA), and LGD Alliance Europe sponsored an international conference on CLAs so leaders in the field could discuss recent advances in research on CLAs. The conference occurred in Dallas, Texas, between September 29th and September 30th, 2023. The event had 22 distinguished speakers and 38 attendees. Additional researchers, clinicians, and patients attended the conference virtually. In this article, we summarize the key takeaways from the meeting. The conference emphasized the crucial role of the patient voice in guiding research, and attendees heard stories from patients and parents of children with CLAs. The conference covered topics such as the genetic causes of CLAs, preclinical models, clinical advances, new technologies for CLA research, lymphatic imaging, and patient-centered research. Videos of all the presentations can be found on the LGDA website. The meeting revealed that while progress has been made, many challenges still exist surrounding the diagnosis and treatment of CLAs. Sustained research efforts are necessary to fill the gaps in knowledge and improve the care and quality of life of CLA patients.
{"title":"Meeting Report for the 2023 International Scientific Conference on Complex Lymphatic Anomalies","authors":"Michael T. Dellinger, T. Ferry, David Williams, Tracy Milne, Louise Murgia, Michael Kelly","doi":"10.1097/jova.0000000000000085","DOIUrl":"https://doi.org/10.1097/jova.0000000000000085","url":null,"abstract":"\u0000 \u0000 Complex lymphatic anomalies (CLAs) are rare diseases with variable clinical manifestations caused by the abnormal development of lymphatic vessels. The Lymphatic Malformation Institute (LMI), Lymphangiomatosis & Gorham’s Disease Alliance (LGDA), and LGD Alliance Europe sponsored an international conference on CLAs so leaders in the field could discuss recent advances in research on CLAs.\u0000 \u0000 \u0000 \u0000 The conference occurred in Dallas, Texas, between September 29th and September 30th, 2023. The event had 22 distinguished speakers and 38 attendees. Additional researchers, clinicians, and patients attended the conference virtually.\u0000 \u0000 \u0000 \u0000 In this article, we summarize the key takeaways from the meeting. The conference emphasized the crucial role of the patient voice in guiding research, and attendees heard stories from patients and parents of children with CLAs. The conference covered topics such as the genetic causes of CLAs, preclinical models, clinical advances, new technologies for CLA research, lymphatic imaging, and patient-centered research. Videos of all the presentations can be found on the LGDA website.\u0000 \u0000 \u0000 \u0000 The meeting revealed that while progress has been made, many challenges still exist surrounding the diagnosis and treatment of CLAs. Sustained research efforts are necessary to fill the gaps in knowledge and improve the care and quality of life of CLA patients.\u0000","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"157 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140233981","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-03-12DOI: 10.1097/jova.0000000000000080
Bryony Lucas, S. Malay, Irina B. Pateva
Vascular anomalies (VA) are a heterogeneous group of diseases which include vascular malformations and vascular tumors. In the pediatric population, there is a sparsity of data on the frequency of associated hematologic and nonhematologic conditions as well as overall outcomes for patients. The objective of this study was to determine the rate of certain concurrent hematologic and nonhematologic conditions in patients with VA who underwent procedures and compare those to patients who did not undergo procedures. To examine the rates of intensive care unit admissions and discharge mortality in patients who had a vascular anomaly. This is a multicenter retrospective cohort study using data from the Pediatric Health Information System. The database was queried for children 0–18 years old with a diagnosis of a VA who had inpatient encounters between January 2016 and September 2021. The most common hematologic conditions were thrombocytopenia, iron deficiency anemia, and venous thrombosis. The most common nonhematologic condition was electrolyte imbalances. Intensive care unit admissions were highest among patients with other congenital malformations of the peripheral vascular system, cerebral cavernous malformations, and sporadic arteriovenous malformations. Discharge mortality was highest in patients with lymphatic malformations and primary lymphedema. This is the largest database study of pediatric patients with VA and their concurrent medical conditions and outcomes to date. While our study could not prove associations, we hope that our results will serve as a foundation for more extensive research regarding the impact of these identified conditions and outcomes and contribute to improved management of patients with VA.
{"title":"Frequency of Hematologic and Nonhematologic Conditions in Pediatric Patients with Vascular Anomalies: A Pediatric Health Information System Database Study","authors":"Bryony Lucas, S. Malay, Irina B. Pateva","doi":"10.1097/jova.0000000000000080","DOIUrl":"https://doi.org/10.1097/jova.0000000000000080","url":null,"abstract":"\u0000 \u0000 Vascular anomalies (VA) are a heterogeneous group of diseases which include vascular malformations and vascular tumors. In the pediatric population, there is a sparsity of data on the frequency of associated hematologic and nonhematologic conditions as well as overall outcomes for patients.\u0000 \u0000 \u0000 \u0000 The objective of this study was to determine the rate of certain concurrent hematologic and nonhematologic conditions in patients with VA who underwent procedures and compare those to patients who did not undergo procedures. To examine the rates of intensive care unit admissions and discharge mortality in patients who had a vascular anomaly.\u0000 \u0000 \u0000 \u0000 This is a multicenter retrospective cohort study using data from the Pediatric Health Information System. The database was queried for children 0–18 years old with a diagnosis of a VA who had inpatient encounters between January 2016 and September 2021.\u0000 \u0000 \u0000 \u0000 The most common hematologic conditions were thrombocytopenia, iron deficiency anemia, and venous thrombosis. The most common nonhematologic condition was electrolyte imbalances. Intensive care unit admissions were highest among patients with other congenital malformations of the peripheral vascular system, cerebral cavernous malformations, and sporadic arteriovenous malformations. Discharge mortality was highest in patients with lymphatic malformations and primary lymphedema.\u0000 \u0000 \u0000 \u0000 This is the largest database study of pediatric patients with VA and their concurrent medical conditions and outcomes to date. While our study could not prove associations, we hope that our results will serve as a foundation for more extensive research regarding the impact of these identified conditions and outcomes and contribute to improved management of patients with VA.\u0000","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"80 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140250830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-27DOI: 10.1097/jova.0000000000000078
Maya Guhan, E. Lambert, Amir Pezeshkmehr, Tara L. Rosenberg
Salivary gland obstruction can present as unilateral, episodic facial swelling. Vascular malformations as a cause of parotid duct obstruction with associated unilateral cheek swelling and pain are rare. We describe the case of a 16-year-old female with a protracted 2-year history of unilateral facial swelling and pain that was misdiagnosed as recurrent parotitis on computed tomography and sialoendoscopy. Eventually, the 16-year-old female was diagnosed by magnetic resonance imaging to have a microcystic lymphatic malformation of the parotid duct as the cause of her cheek swelling and pain. Afterwards, the patient was referred to undergo interstitial bleomycin sclerotherapy. This case demonstrates a unique microcystic lymphatic malformation producing obstructive symptoms as well as the utility of magnetic resonance imaging for the diagnosis of a patient presenting with recurrent unilateral facial pain and swelling.
{"title":"Parotid Gland Microcystic Lymphatic Malformation Producing Obstructive Symptoms","authors":"Maya Guhan, E. Lambert, Amir Pezeshkmehr, Tara L. Rosenberg","doi":"10.1097/jova.0000000000000078","DOIUrl":"https://doi.org/10.1097/jova.0000000000000078","url":null,"abstract":"\u0000 \u0000 Salivary gland obstruction can present as unilateral, episodic facial swelling. Vascular malformations as a cause of parotid duct obstruction with associated unilateral cheek swelling and pain are rare.\u0000 \u0000 \u0000 \u0000 We describe the case of a 16-year-old female with a protracted 2-year history of unilateral facial swelling and pain that was misdiagnosed as recurrent parotitis on computed tomography and sialoendoscopy. Eventually, the 16-year-old female was diagnosed by magnetic resonance imaging to have a microcystic lymphatic malformation of the parotid duct as the cause of her cheek swelling and pain. Afterwards, the patient was referred to undergo interstitial bleomycin sclerotherapy.\u0000 \u0000 \u0000 \u0000 This case demonstrates a unique microcystic lymphatic malformation producing obstructive symptoms as well as the utility of magnetic resonance imaging for the diagnosis of a patient presenting with recurrent unilateral facial pain and swelling.\u0000","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140426586","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-07DOI: 10.1097/jova.0000000000000082
Lorna Holcroft, Maeve A. Mc Aleer, Alan D. Irvine
Lymphedema is a chronic condition characterized by the accumulation of lymphatic fluid in interstitial and fibro-adipose tissues. Primary lymphedema that arises from intrinsic defects in the lymphatic system is a rare form of lymphedema with a variable age of onset and heterogeneous presentation. Although a number of genetic variants have been identified and matched to corresponding phenotypes, a genetic cause for lymphedema is found in less than 30% of individuals with primary lymphedema. Recently, Michelini et al proposed NOTCH1 as a candidate gene for primary lymphedema, having identified missense variants in NOTCH1 in 7 of 235 patients with primary lymphedema for whom no causative gene had previously been identified. We report the case of a young female who developed bilateral primary lower limb lymphedema and on genetic testing was found to have a previously unreported heterozygous frameshift variant in the NOTCH1 gene predicting loss of function.
{"title":"A Novel Likely Pathogenic Variant in NOTCH1 Associated With Primary Lymphedema","authors":"Lorna Holcroft, Maeve A. Mc Aleer, Alan D. Irvine","doi":"10.1097/jova.0000000000000082","DOIUrl":"https://doi.org/10.1097/jova.0000000000000082","url":null,"abstract":"Lymphedema is a chronic condition characterized by the accumulation of lymphatic fluid in interstitial and fibro-adipose tissues. Primary lymphedema that arises from intrinsic defects in the lymphatic system is a rare form of lymphedema with a variable age of onset and heterogeneous presentation. Although a number of genetic variants have been identified and matched to corresponding phenotypes, a genetic cause for lymphedema is found in less than 30% of individuals with primary lymphedema. Recently, Michelini et al proposed NOTCH1 as a candidate gene for primary lymphedema, having identified missense variants in NOTCH1 in 7 of 235 patients with primary lymphedema for whom no causative gene had previously been identified. We report the case of a young female who developed bilateral primary lower limb lymphedema and on genetic testing was found to have a previously unreported heterozygous frameshift variant in the NOTCH1 gene predicting loss of function.","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"30 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139796965","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-07DOI: 10.1097/jova.0000000000000082
Lorna Holcroft, Maeve A. Mc Aleer, Alan D. Irvine
Lymphedema is a chronic condition characterized by the accumulation of lymphatic fluid in interstitial and fibro-adipose tissues. Primary lymphedema that arises from intrinsic defects in the lymphatic system is a rare form of lymphedema with a variable age of onset and heterogeneous presentation. Although a number of genetic variants have been identified and matched to corresponding phenotypes, a genetic cause for lymphedema is found in less than 30% of individuals with primary lymphedema. Recently, Michelini et al proposed NOTCH1 as a candidate gene for primary lymphedema, having identified missense variants in NOTCH1 in 7 of 235 patients with primary lymphedema for whom no causative gene had previously been identified. We report the case of a young female who developed bilateral primary lower limb lymphedema and on genetic testing was found to have a previously unreported heterozygous frameshift variant in the NOTCH1 gene predicting loss of function.
{"title":"A Novel Likely Pathogenic Variant in NOTCH1 Associated With Primary Lymphedema","authors":"Lorna Holcroft, Maeve A. Mc Aleer, Alan D. Irvine","doi":"10.1097/jova.0000000000000082","DOIUrl":"https://doi.org/10.1097/jova.0000000000000082","url":null,"abstract":"Lymphedema is a chronic condition characterized by the accumulation of lymphatic fluid in interstitial and fibro-adipose tissues. Primary lymphedema that arises from intrinsic defects in the lymphatic system is a rare form of lymphedema with a variable age of onset and heterogeneous presentation. Although a number of genetic variants have been identified and matched to corresponding phenotypes, a genetic cause for lymphedema is found in less than 30% of individuals with primary lymphedema. Recently, Michelini et al proposed NOTCH1 as a candidate gene for primary lymphedema, having identified missense variants in NOTCH1 in 7 of 235 patients with primary lymphedema for whom no causative gene had previously been identified. We report the case of a young female who developed bilateral primary lower limb lymphedema and on genetic testing was found to have a previously unreported heterozygous frameshift variant in the NOTCH1 gene predicting loss of function.","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"53 11-12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139856932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-06DOI: 10.1097/jova.0000000000000081
Andrew Baker, Kevin Wong, Laura Gonzales-Krellwitz, Elizabeth Kim, Graham M Strub
Lymphatic malformations are vascular anomalies that often arise from congenital defects and can manifest in a variety of locations, including the abdomen. Such lesions are often targeted with sclerotherapy, which has become the first-line treatment due to the limitations of surgical intervention. Sclerotherapy is associated with side effects including local necrosis and edema, with localized tissue destruction seldom reported. Here we describe the case of a neonate female with bowel wall perforation following sclerotherapy with ethanol and doxycycline for macrocystic abdominal lymphatic malformation.
{"title":"Bowel Perforation Following Sclerotherapy of a Massive Intra-Abdominal Lymphatic Malformation","authors":"Andrew Baker, Kevin Wong, Laura Gonzales-Krellwitz, Elizabeth Kim, Graham M Strub","doi":"10.1097/jova.0000000000000081","DOIUrl":"https://doi.org/10.1097/jova.0000000000000081","url":null,"abstract":"Lymphatic malformations are vascular anomalies that often arise from congenital defects and can manifest in a variety of locations, including the abdomen. Such lesions are often targeted with sclerotherapy, which has become the first-line treatment due to the limitations of surgical intervention. Sclerotherapy is associated with side effects including local necrosis and edema, with localized tissue destruction seldom reported. Here we describe the case of a neonate female with bowel wall perforation following sclerotherapy with ethanol and doxycycline for macrocystic abdominal lymphatic malformation.","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"79 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139860845","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-06DOI: 10.1097/jova.0000000000000081
Andrew Baker, Kevin Wong, Laura Gonzales-Krellwitz, Elizabeth Kim, Graham M Strub
Lymphatic malformations are vascular anomalies that often arise from congenital defects and can manifest in a variety of locations, including the abdomen. Such lesions are often targeted with sclerotherapy, which has become the first-line treatment due to the limitations of surgical intervention. Sclerotherapy is associated with side effects including local necrosis and edema, with localized tissue destruction seldom reported. Here we describe the case of a neonate female with bowel wall perforation following sclerotherapy with ethanol and doxycycline for macrocystic abdominal lymphatic malformation.
{"title":"Bowel Perforation Following Sclerotherapy of a Massive Intra-Abdominal Lymphatic Malformation","authors":"Andrew Baker, Kevin Wong, Laura Gonzales-Krellwitz, Elizabeth Kim, Graham M Strub","doi":"10.1097/jova.0000000000000081","DOIUrl":"https://doi.org/10.1097/jova.0000000000000081","url":null,"abstract":"Lymphatic malformations are vascular anomalies that often arise from congenital defects and can manifest in a variety of locations, including the abdomen. Such lesions are often targeted with sclerotherapy, which has become the first-line treatment due to the limitations of surgical intervention. Sclerotherapy is associated with side effects including local necrosis and edema, with localized tissue destruction seldom reported. Here we describe the case of a neonate female with bowel wall perforation following sclerotherapy with ethanol and doxycycline for macrocystic abdominal lymphatic malformation.","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"15 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139801090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-01-10DOI: 10.1097/jova.0000000000000083
Anna M. Kerr, Christine Bereitschaft, Jessica Goldberg, Bryan A. Sisk
The goal of the current study was to gain a deeper understanding of the significant experiences that characterize care for vascular anomalies (VAs). A total of 166 adult patients and 88 caregivers (N = 254) completed an anonymous online cross-sectional survey about their experiences seeking care for their (or their child’s) vascular anomaly. We used thematic analysis to analyze participants’ responses to 3 open-ended questions asking about the biggest challenges, most memorable positive experiences, and any other significant experiences. Participants reported significant healthcare experiences representing 5 primary areas: diagnosis and treatment, healthcare system and logistics, psychosocial consequences, physical consequences, and clinical relationships. The availability of clinical knowledge and information was identified as an overarching theme affecting all 5 categories. The most common negative experiences related to healthcare system and logistics (n = 100). Clinical relationships were commonly identified in both positive (n = 100) and negative (n = 86) experiences. Their responses highlighted the value of a well-organized system of care that promotes productive interactions with expert clinicians and connects patients with support organizations. Unfortunately, VA patients and caregivers often experience long diagnostic journeys, fragmented care, and nonproductive interactions with clinicians due to the pervasive lack of information about VAs. The results indicate the need for systemic changes to address these barriers to care for patients with rare diseases.
{"title":"Significant Experiences Caring for Vascular Anomalies: A Survey of Caregivers and Adult Patients","authors":"Anna M. Kerr, Christine Bereitschaft, Jessica Goldberg, Bryan A. Sisk","doi":"10.1097/jova.0000000000000083","DOIUrl":"https://doi.org/10.1097/jova.0000000000000083","url":null,"abstract":"\u0000 \u0000 The goal of the current study was to gain a deeper understanding of the significant experiences that characterize care for vascular anomalies (VAs).\u0000 \u0000 \u0000 \u0000 A total of 166 adult patients and 88 caregivers (N = 254) completed an anonymous online cross-sectional survey about their experiences seeking care for their (or their child’s) vascular anomaly. We used thematic analysis to analyze participants’ responses to 3 open-ended questions asking about the biggest challenges, most memorable positive experiences, and any other significant experiences.\u0000 \u0000 \u0000 \u0000 Participants reported significant healthcare experiences representing 5 primary areas: diagnosis and treatment, healthcare system and logistics, psychosocial consequences, physical consequences, and clinical relationships. The availability of clinical knowledge and information was identified as an overarching theme affecting all 5 categories. The most common negative experiences related to healthcare system and logistics (n = 100). Clinical relationships were commonly identified in both positive (n = 100) and negative (n = 86) experiences.\u0000 \u0000 \u0000 \u0000 Their responses highlighted the value of a well-organized system of care that promotes productive interactions with expert clinicians and connects patients with support organizations. Unfortunately, VA patients and caregivers often experience long diagnostic journeys, fragmented care, and nonproductive interactions with clinicians due to the pervasive lack of information about VAs. The results indicate the need for systemic changes to address these barriers to care for patients with rare diseases.\u0000","PeriodicalId":74008,"journal":{"name":"Journal of vascular anomalies","volume":"6 10","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139439569","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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