C. Cornacchia, S. Dessalvi, G. Santori, F. Canobbio, G. Atzori, F. De Paoli, R. Diaz, S. Franchelli, M. Gipponi, F. Murelli, M. Sparavigna, F. Pitto, A. Fozza, F. Boccardo, D. Friedman, P. Fregatti
Breast-conserving surgery (BCS) is the standard of care for early-stage breast cancer. We retrospectively enrolled 530 patients (mean age: 62.96 ± 12.69 years) undergoing BCS between January 1, 2018, and December 31, 2019. During the COVID-19 pandemic, all patients with at least 1 year of follow-up were telephonically asked after surgery to provide clinical signs and symptoms attributable to postoperative breast cancer-related lymphedema of the breast (BCRL-B). Thirty-one (5.8%) patients reported breast edema and were visited to measure the tissue dielectric constant (TDC) and to assess the induration of the skin. There was a difference seen in treatment with lumpectomy + ALND performed more frequently in patients with (29%) than without (12%) BCRL-B. In the subgroup of patients with BCRL-B (n=31), significantly higher values of local total water were calculated in the nine patients who underwent Lump + ALND procedure (1.86 ± 0.48 vs. 1.48 ± 0.38; p = 0.046). Among patients with BCRL-B (n=31), in eight patients (25.8%) tissue induration measured with SkinFibroMeter was >0.100 N, thus suggesting tissue fibrosis. Cumulative survival probability at 1-year after surgery was 0.992. No statistical differences in 1-year survival after surgery were found for type of surgery (p = 0.890) or absence/presence of BCRL-B (p = 0.480). In univariate logistic regression, only lumpectomy + ALND surgery (p = 0.009) and any subsequent axillary lymph node removal surgery (p = 0.003) were associated with BCRL-B. Both of these variables were also found to be statistically significant in the multivariate regression model. Further prospective research is warranted to analyze potentential predictors of BCRL-B and to reduce/ prevent this complication.
{"title":"Breast Edema after Conservative Surgery for Early-Stage Breast Cancer: A Retrospective Single-Center Assessment of Risk Factors.","authors":"C. Cornacchia, S. Dessalvi, G. Santori, F. Canobbio, G. Atzori, F. De Paoli, R. Diaz, S. Franchelli, M. Gipponi, F. Murelli, M. Sparavigna, F. Pitto, A. Fozza, F. Boccardo, D. Friedman, P. Fregatti","doi":"10.2458/lymph.5738","DOIUrl":"https://doi.org/10.2458/lymph.5738","url":null,"abstract":"Breast-conserving surgery (BCS) is the standard of care for early-stage breast cancer. We retrospectively enrolled 530 patients (mean age: 62.96 ± 12.69 years) undergoing BCS between January 1, 2018, and December 31, 2019. During the COVID-19 pandemic, all patients with at least 1 year of follow-up were telephonically asked after surgery to provide clinical signs and symptoms attributable to postoperative breast cancer-related lymphedema of the breast (BCRL-B). Thirty-one (5.8%) patients reported breast edema and were visited to measure the tissue dielectric constant (TDC) and to assess the induration of the skin. There was a difference seen in treatment with lumpectomy + ALND performed more frequently in patients with (29%) than without (12%) BCRL-B. In the subgroup of patients with BCRL-B (n=31), significantly higher values of local total water were calculated in the nine patients who underwent Lump + ALND procedure (1.86 ± 0.48 vs. 1.48 ± 0.38; p = 0.046). Among patients with BCRL-B (n=31), in eight patients (25.8%) tissue induration measured with SkinFibroMeter was >0.100 N, thus suggesting tissue fibrosis. Cumulative survival probability at 1-year after surgery was 0.992. No statistical differences in 1-year survival after surgery were found for type of surgery (p = 0.890) or absence/presence of BCRL-B (p = 0.480). In univariate logistic regression, only lumpectomy + ALND surgery (p = 0.009) and any subsequent axillary lymph node removal surgery (p = 0.003) were associated with BCRL-B. Both of these variables were also found to be statistically significant in the multivariate regression model. Further prospective research is warranted to analyze potentential predictors of BCRL-B and to reduce/ prevent this complication.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 4 1","pages":"167-177"},"PeriodicalIF":2.5,"publicationDate":"2023-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49602478","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Chylous ascites (CA), also called chyloperitoneum, is a rare form of ascites in the neonate. It results from the leakage of lymph into the peritoneal cavity. There are congenital and acquired forms of CA. CA may occur during fetal life, and the prognosis will depend on its volume, gestational age at the onset, and the association with other anomalies. Lymphangiectasia is the most common congenital cause, and acquired forms are mainly traumatic and/or post-operative. This review aims to gather the most current information on CA and addresses important aspects regarding etiology, pathophysiology, clinic, diagnostic tools, and treatment.
{"title":"Chylous ascites in the neonate: A narrative review.","authors":"G. Rocha","doi":"10.2458/lymph.5404","DOIUrl":"https://doi.org/10.2458/lymph.5404","url":null,"abstract":"Chylous ascites (CA), also called chyloperitoneum, is a rare form of ascites in the neonate. It results from the leakage of lymph into the peritoneal cavity. There are congenital and acquired forms of CA. CA may occur during fetal life, and the prognosis will depend on its volume, gestational age at the onset, and the association with other anomalies. Lymphangiectasia is the most common congenital cause, and acquired forms are mainly traumatic and/or post-operative. This review aims to gather the most current information on CA and addresses important aspects regarding etiology, pathophysiology, clinic, diagnostic tools, and treatment.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 3 1","pages":"117-128"},"PeriodicalIF":2.5,"publicationDate":"2022-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42896277","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
S. Radu, J. Wilson, A. Bagwell, G. Meyers, A. Nauta
Lymphedema is a debilitating disease characterized by abnormal lymphatic drainage, either due to primary maldevelopment of the lymphatic system or to secondary injury. The clinical features of primary and secondary lymphedema differ, with primary lymphedema more often involving progressive bilateral lower extremity disease as compared to secondary lymphedema characteristically having more localized symptoms related to the origin of injury. This case presentation describes a patient who presented with bilateral lower extremity swelling, left greater than the right, with imaging results to support the diagnosis of lymphedema. During the time he was followed in our clinic, our team witnessed rapid progression of his lymphedema despite compliance with conservative management. We believe that the primary mechanism of systemic damage to our patient's lymphatic system is the lenalidomide and bortezomib therapy prescribed to treat multiple myeloma. This review explores the relationship between lenalidomide, bortezomib, and lymphedema in efforts of understanding this unique pathology of iatrogenic lymphedema mimicking primary nature.
{"title":"Iatrogenic systemic lymphedema following multiple myeloma treatment.","authors":"S. Radu, J. Wilson, A. Bagwell, G. Meyers, A. Nauta","doi":"10.2458/lymph.5403","DOIUrl":"https://doi.org/10.2458/lymph.5403","url":null,"abstract":"Lymphedema is a debilitating disease characterized by abnormal lymphatic drainage, either due to primary maldevelopment of the lymphatic system or to secondary injury. The clinical features of primary and secondary lymphedema differ, with primary lymphedema more often involving progressive bilateral lower extremity disease as compared to secondary lymphedema characteristically having more localized symptoms related to the origin of injury. This case presentation describes a patient who presented with bilateral lower extremity swelling, left greater than the right, with imaging results to support the diagnosis of lymphedema. During the time he was followed in our clinic, our team witnessed rapid progression of his lymphedema despite compliance with conservative management. We believe that the primary mechanism of systemic damage to our patient's lymphatic system is the lenalidomide and bortezomib therapy prescribed to treat multiple myeloma. This review explores the relationship between lenalidomide, bortezomib, and lymphedema in efforts of understanding this unique pathology of iatrogenic lymphedema mimicking primary nature.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 3 1","pages":"110-116"},"PeriodicalIF":2.5,"publicationDate":"2022-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69057293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
P.S. Russell, S. Nachkebia, Victor E. Maldonado-Zimbron, S. Chuklin, G. Gimel'farb, J. Hong, N.D. Martin, M. Itkin, Arj Phillips, J. Windsor
Thoracic duct drainage (TDD) is gaining renewed interest, largely due to accumulation of evidence supporting the gut-lymph model, where toxic mesenteric lymph from the intestine contributes to development of multi-organ failure in acute and critical illness (ACI). Advances in minimally invasive TDD have added to this growing interest. The English TDD literature has been previously reviewed, but the more extensive Eastern European literature has not been available to English readers. Therefore, we undertook a systematic search of Eastern European human TDD studies using Scopus and PubMed databases and Russian language websites. Indications for TDD, clinical outcomes, and complications were reviewed. 113 studies, published between 1965 and 2015, were reviewed. The most common indications for TDD were hepatic failure, acute pancreatitis, and peritonitis. It was often used late and when other treatment options had been exhausted. Human TDD appeared safe and probably effective, especially when combined with lymphosorption. The benefit appeared to correlate with the volume of lymph drained. A randomized controlled trial (and some case-control studies) showed reduced mortality in patients with ACI with TDD. Other benefits included rapid normalization of blood parameters and decreased organ edema. This review provides further support for the gut-lymph model and justification for high quality randomized controlled trials of TDD in ACI. It also highlights other potential indications for TDD, such as bridging patients with liver failure to surgery or transplant.
{"title":"Therapeutic thoracic duct drainage: A systematic review of the Eastern European experience and future potential.","authors":"P.S. Russell, S. Nachkebia, Victor E. Maldonado-Zimbron, S. Chuklin, G. Gimel'farb, J. Hong, N.D. Martin, M. Itkin, Arj Phillips, J. Windsor","doi":"10.2458/lymph.5402","DOIUrl":"https://doi.org/10.2458/lymph.5402","url":null,"abstract":"Thoracic duct drainage (TDD) is gaining renewed interest, largely due to accumulation of evidence supporting the gut-lymph model, where toxic mesenteric lymph from the intestine contributes to development of multi-organ failure in acute and critical illness (ACI). Advances in minimally invasive TDD have added to this growing interest. The English TDD literature has been previously reviewed, but the more extensive Eastern European literature has not been available to English readers. Therefore, we undertook a systematic search of Eastern European human TDD studies using Scopus and PubMed databases and Russian language websites. Indications for TDD, clinical outcomes, and complications were reviewed. 113 studies, published between 1965 and 2015, were reviewed. The most common indications for TDD were hepatic failure, acute pancreatitis, and peritonitis. It was often used late and when other treatment options had been exhausted. Human TDD appeared safe and probably effective, especially when combined with lymphosorption. The benefit appeared to correlate with the volume of lymph drained. A randomized controlled trial (and some case-control studies) showed reduced mortality in patients with ACI with TDD. Other benefits included rapid normalization of blood parameters and decreased organ edema. This review provides further support for the gut-lymph model and justification for high quality randomized controlled trials of TDD in ACI. It also highlights other potential indications for TDD, such as bridging patients with liver failure to surgery or transplant.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 3 1","pages":"86-109"},"PeriodicalIF":2.5,"publicationDate":"2022-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49417881","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
X. Geng, L. Chen, R. Srinivasan, R. J. Kylat, M. Witte, R. Erickson
We have studied the lymphatic phenotypes of 2 mutations, known to cause abnormalities of lymphatics in humans, in mice. The Cx47 R260C mutation (variably penetrant in humans heterozygous for it and causing limb lymphedema) had an adult mouse phenotype of hyperplasia and increased lymph nodes only in homozygous condition but we did not find any anatomical phenotype in day 16.5 homozygous embryos. Mice harboring the Sos1 mutation E846K (causing Noonan's in man which occasionally shows lymphatic dysplasia) had no adult heterozygous phenotype in lymphatic vessel appearance and drainage (homozygotes are early embryonic lethals) while day 16.5 heterozygous embryos also had no detectable anatomical phenotype.
{"title":"Lack of embryonic homozygous or adult heterozygous lymphatic phenotypes for a Sos1 mutation and lack of lymphatic embryonic phenotypes for a homozygous Cx47 mutation in mice.","authors":"X. Geng, L. Chen, R. Srinivasan, R. J. Kylat, M. Witte, R. Erickson","doi":"10.2458/lymph.5405","DOIUrl":"https://doi.org/10.2458/lymph.5405","url":null,"abstract":"We have studied the lymphatic phenotypes of 2 mutations, known to cause abnormalities of lymphatics in humans, in mice. The Cx47 R260C mutation (variably penetrant in humans heterozygous for it and causing limb lymphedema) had an adult mouse phenotype of hyperplasia and increased lymph nodes only in homozygous condition but we did not find any anatomical phenotype in day 16.5 homozygous embryos. Mice harboring the Sos1 mutation E846K (causing Noonan's in man which occasionally shows lymphatic dysplasia) had no adult heterozygous phenotype in lymphatic vessel appearance and drainage (homozygotes are early embryonic lethals) while day 16.5 heterozygous embryos also had no detectable anatomical phenotype.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 3 1","pages":"129-134"},"PeriodicalIF":2.5,"publicationDate":"2022-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41446077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Worldwide, lymphedema can present as a significant health issue. Left untreated, it can have long-term medical and psychological consequences for patients. Cryotherapy is a new physical therapy modality used for many purposes including reduction of pain, inflammation, and edema. It is thought to decrease interstitial fluid volume through many mechanisms. Therefore, it is reasonable to think that cryotherapy might have a positive effect in treatment of lymphedema. The goal of this study was to investigate how local cryotherapy in combination with standard therapy affects patient outcomes. Forty post-mastectomy female patients aged 40-60 years old with lymphedema were referred to the outpatient clinics of the Faculty of Physical Therapy at South Valley University for medical treatment and follow-up by the vascular surgery department. Patients were randomly divided into two groups of equal size. Traditional physical therapy programs (manual lymphatic drainage, pneumatic compression, bandaging, breathing exercises, circulatory exercises, shoulder mobilizations, and ROM exercises) were combined with pulsed local cryotherapy three times per week for 12 weeks in Group (A). For 12 weeks, Group (B) received only traditional physical therapy three times per week. Patients were evaluated using circumferential measurement with tape at the wrist, below the elbow, and above the elbow level, as well as ultrasonography to assess skin thickness before the start of physical therapy, 6 weeks later, and at the end of the treatment (after 12 weeks). Results indicate that cryotherapy is an effective adjunct modality for the treatment of secondary lymphedema and should be added to physical therapy protocols for lymphedema rehabilitation.
{"title":"Addition of local cryotherapy for treatment of post-mastectomy lymphedema.","authors":"Z. M. Askary, M. Elshazly","doi":"10.2458/lymph.5269","DOIUrl":"https://doi.org/10.2458/lymph.5269","url":null,"abstract":"Worldwide, lymphedema can present as a significant health issue. Left untreated, it can have long-term medical and psychological consequences for patients. Cryotherapy is a new physical therapy modality used for many purposes including reduction of pain, inflammation, and edema. It is thought to decrease interstitial fluid volume through many mechanisms. Therefore, it is reasonable to think that cryotherapy might have a positive effect in treatment of lymphedema. The goal of this study was to investigate how local cryotherapy in combination with standard therapy affects patient outcomes. Forty post-mastectomy female patients aged 40-60 years old with lymphedema were referred to the outpatient clinics of the Faculty of Physical Therapy at South Valley University for medical treatment and follow-up by the vascular surgery department. Patients were randomly divided into two groups of equal size. Traditional physical therapy programs (manual lymphatic drainage, pneumatic compression, bandaging, breathing exercises, circulatory exercises, shoulder mobilizations, and ROM exercises) were combined with pulsed local cryotherapy three times per week for 12 weeks in Group (A). For 12 weeks, Group (B) received only traditional physical therapy three times per week. Patients were evaluated using circumferential measurement with tape at the wrist, below the elbow, and above the elbow level, as well as ultrasonography to assess skin thickness before the start of physical therapy, 6 weeks later, and at the end of the treatment (after 12 weeks). Results indicate that cryotherapy is an effective adjunct modality for the treatment of secondary lymphedema and should be added to physical therapy protocols for lymphedema rehabilitation.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 2 1","pages":"70-76"},"PeriodicalIF":2.5,"publicationDate":"2022-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46460039","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
O. Engin, E. Şahin, E. Saribay, B. Dilek, E. Akalın
Cellulitis is one of the most important troubling complications of breast cancer treatment. Therefore, elucidating the risk factors for cellulitis in patients that have undergone breast cancer treatment is crucial. This is a retrospective medical record study among 523 patients who had received breast cancer treatment and were referred to the Lymphedema Clinic. Data on age, height, weight, BMI (body mass index), education level, arm dominance, history of previous surgery, axillary lymph node dissection, radiotherapy, and chemotherapy were noted. The time between operation and onset of lymphedema, duration of lymphedema, history of cellulitis, and number of cellulitis attacks were recorded. Circumference measurements were taken at four points on the upper limb. Univariate analysis showed that longer duration of lymphedema, larger circumference of the unaffected arm and larger circumference of the arm with lymphedema were associated with higher risk of cellulitis (p=0.008, p=0.007, p< 0.001, respectively). The incidence of cellulitis was higher in patients with lymphedema than patients who had no lymphedema (p< 0.001). Moreover, the frequency of cellulitis was higher in patients with lower education level (p=0.015). It was deter-mined that patients with cellulitis needed more compression garments (p< 0.001) and multi-layered bandage therapy (p< 0.001) than those without. Regression analysis revealed that presence of lymphedema (p=0.036), duration of lymphedema (p=0.048), radiotherapy (p=0.01) and educational level (0.019) are significantly associated with developing upper extremity cellulitis. It is important to consider these risk factors for the prevention and management of cellulitis in patients who undergo treatment for breast cancer. Early detection and treatment of lymphedema also remains essential for these patients.
{"title":"Risk factors for developing upper limb cellulitis after breast cancer treatment.","authors":"O. Engin, E. Şahin, E. Saribay, B. Dilek, E. Akalın","doi":"10.2458/lymph.5270","DOIUrl":"https://doi.org/10.2458/lymph.5270","url":null,"abstract":"Cellulitis is one of the most important troubling complications of breast cancer treatment. Therefore, elucidating the risk factors for cellulitis in patients that have undergone breast cancer treatment is crucial. This is a retrospective medical record study among 523 patients who had received breast cancer treatment and were referred to the Lymphedema Clinic. Data on age, height, weight, BMI (body mass index), education level, arm dominance, history of previous surgery, axillary lymph node dissection, radiotherapy, and chemotherapy were noted. The time between operation and onset of lymphedema, duration of lymphedema, history of cellulitis, and number of cellulitis attacks were recorded. Circumference measurements were taken at four points on the upper limb. Univariate analysis showed that longer duration of lymphedema, larger circumference of the unaffected arm and larger circumference of the arm with lymphedema were associated with higher risk of cellulitis (p=0.008, p=0.007, p< 0.001, respectively). The incidence of cellulitis was higher in patients with lymphedema than patients who had no lymphedema (p< 0.001). Moreover, the frequency of cellulitis was higher in patients with lower education level (p=0.015). It was deter-mined that patients with cellulitis needed more compression garments (p< 0.001) and multi-layered bandage therapy (p< 0.001) than those without. Regression analysis revealed that presence of lymphedema (p=0.036), duration of lymphedema (p=0.048), radiotherapy (p=0.01) and educational level (0.019) are significantly associated with developing upper extremity cellulitis. It is important to consider these risk factors for the prevention and management of cellulitis in patients who undergo treatment for breast cancer. Early detection and treatment of lymphedema also remains essential for these patients.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 2 1","pages":"77-83"},"PeriodicalIF":2.5,"publicationDate":"2022-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44704503","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Plastic bronchitis, more appropriately termed chyloptysis, is a rare and potentially fatal condition caused by chylous coating of the airways. These cast coating can dislodge and become an obstructive mass in the patient's airway, necessitating rapid intervention. PB is well described to occur following single ventricle physiology heart disease corrective procedures, particularly following Fontan procedures. It is less commonly seen in traumatic settings. We present the youngest known case of a traumatic injury induced plastic bronchitis. A 19-year-old man was involved in a motor vehicle accident with airbag deployment. The airbags struck him in the chest; however, the patient felt well at the time and did not seek medical attention. Several months later the patient began coughing up milky white masses identified as casts. He was initially diagnosed with asthma but did not respond to therapy. He ultimately was found to have evidence of thoracic duct injury. Options for therapy were discussed, including possible thoracic duct ligation. The patient opted to continue a lowfat diet and has remained cast free. This case highlights the importance of considering plastic bronchitis in patients with cast production and a history of trauma to the chest.
{"title":"Plastic bronchitis: A rare complication following a motor vehicle collision.","authors":"J. Lee, K. Stanley, M. C. Lowe","doi":"10.2458/lymph.5268","DOIUrl":"https://doi.org/10.2458/lymph.5268","url":null,"abstract":"Plastic bronchitis, more appropriately termed chyloptysis, is a rare and potentially fatal condition caused by chylous coating of the airways. These cast coating can dislodge and become an obstructive mass in the patient's airway, necessitating rapid intervention. PB is well described to occur following single ventricle physiology heart disease corrective procedures, particularly following Fontan procedures. It is less commonly seen in traumatic settings. We present the youngest known case of a traumatic injury induced plastic bronchitis. A 19-year-old man was involved in a motor vehicle accident with airbag deployment. The airbags struck him in the chest; however, the patient felt well at the time and did not seek medical attention. Several months later the patient began coughing up milky white masses identified as casts. He was initially diagnosed with asthma but did not respond to therapy. He ultimately was found to have evidence of thoracic duct injury. Options for therapy were discussed, including possible thoracic duct ligation. The patient opted to continue a lowfat diet and has remained cast free. This case highlights the importance of considering plastic bronchitis in patients with cast production and a history of trauma to the chest.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 2 1","pages":"65-69"},"PeriodicalIF":2.5,"publicationDate":"2022-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45387092","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Milroy disease, known as primary congenital lymphedema, is characterized by chronic tissue swelling due to impaired lymphatic drainage and is inherited in an autosomal dominant manner. This study reports a rare case of Milroy disease affecting siblings from unaffected parents. A one-month-old female infant presented with swelling of the bilateral calf and the dorsum of the feet which had been present since birth. Her 14-month-old brother had a similar presentation since birth with swelling of the bilateral calf and the dorsum of the feet. Milroy disease was diagnosed based on the clinical findings of bilateral lower limb swelling and confirmed by molecular genetic testing. The patient and her family, including her brother, parents, and maternal grandfather, were genetically tested, and two novel missense mutations (NM_182925.4: c.2534T>C; p.Leu845Pro, c.4006G>A; p.Glu1336Lys) were found in the Fms-related tyrosine kinase (FLT4) gene. Mutations segregated by the parents who carried each mutation in the heterozygous state were identified in the patient and her brother. The present case report in which Milroy disease developed in all offspring of parents with a normal phenotype suggests the possibility of a compound heterozygous mutation or non-penetrance during the process of inheritance of Milroy disease.
Milroy病，被称为原发性先天性淋巴水肿，其特征是由于淋巴排水受损导致的慢性组织肿胀，并以常染色体显性方式遗传。本研究报告了一例罕见的米罗伊氏病，影响未受影响的父母的兄弟姐妹。一个月大的女婴表现为双侧小腿和足背肿胀，自出生以来一直存在。她14个月大的弟弟自出生以来也有类似的表现，双侧小腿和脚背肿胀。根据双侧下肢肿胀的临床表现诊断Milroy病，并通过分子基因检测确诊。对患者及其家人(包括其兄弟、父母和外祖父)进行了基因检测，发现两个新的错义突变(NM_182925.4: C . 2534t >C;p.Leu845Pro, c.4006G >;p.Glu1336Lys)在fms相关酪氨酸激酶(FLT4)基因中发现。在患者和她的兄弟身上发现了由携带杂合状态突变的父母分离的突变。本病例报告表明，在Milroy病的遗传过程中，可能存在复合杂合突变或非外显性。
{"title":"Compound heterozygosity for a variably penetrant variant and a variant of unknown significance in FLT4 causes fully penetrant Milroy's lymphedema.","authors":"J. Kim, S. Y. Lim","doi":"10.2458/lymph.5264","DOIUrl":"https://doi.org/10.2458/lymph.5264","url":null,"abstract":"Milroy disease, known as primary congenital lymphedema, is characterized by chronic tissue swelling due to impaired lymphatic drainage and is inherited in an autosomal dominant manner. This study reports a rare case of Milroy disease affecting siblings from unaffected parents. A one-month-old female infant presented with swelling of the bilateral calf and the dorsum of the feet which had been present since birth. Her 14-month-old brother had a similar presentation since birth with swelling of the bilateral calf and the dorsum of the feet. Milroy disease was diagnosed based on the clinical findings of bilateral lower limb swelling and confirmed by molecular genetic testing. The patient and her family, including her brother, parents, and maternal grandfather, were genetically tested, and two novel missense mutations (NM_182925.4: c.2534T>C; p.Leu845Pro, c.4006G>A; p.Glu1336Lys) were found in the Fms-related tyrosine kinase (FLT4) gene. Mutations segregated by the parents who carried each mutation in the heterozygous state were identified in the patient and her brother. The present case report in which Milroy disease developed in all offspring of parents with a normal phenotype suggests the possibility of a compound heterozygous mutation or non-penetrance during the process of inheritance of Milroy disease.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 2 1","pages":"41-46"},"PeriodicalIF":2.5,"publicationDate":"2022-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45068032","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
E. Rannikko, Ida-Maria Leppäpuska, M. Laukka, A. Saarikko, P. Hartiala
Vascularized lymph node transfer surgery (VLNT) can provide benefit to lymphedema patients. Cytokines may play a role in the development of lymphedema and in the regeneration of lymphatic vessels after VLNT. Our primary aim was to investigate whether the VLNT patients have a specific cytokine profile. Our secondary aim was to see whether the preoperative lymphedema or severity affects the postoperative cytokine response. Wound exudate was gathered from 18 patients undergoing VLNT on the first and sixth postoperative day (POD). The concentrations of IL-10, TNF-α, TGF-β1 and VEGF-C were analyzed using enzymelinked immune-sorbent assays. A general score was generated to assess the benefit of the surgery. The changes in cytokine concentrations (1st POD-6th POD) were correlated with the pre- and postoperative lymphedema related factors. A shorter duration of lymphedema preoperatively correlated with an increase in the concentration of IL-10 and TNF-β during the first six PODs (IL-10: r=0.495, p=0.051; TNF-α: r=0.737, p=0.006) and a decrease in the concentration of TGF-ß1 (r= -0.613, p=0.020). The increase of the concentration of TNF-α during the first six PODs also correlated with a greater total general score (r=0.775, p=0.005) and hence indicated a better response to the surgery. The patients with a shorter duration of lymphedema preoperatively had a more favorable cytokine response during the first six PODs after VLNT.
{"title":"Short duration of upper extremity lymphedema correlates with a favorable cytokine response after lymph node transfer surgery.","authors":"E. Rannikko, Ida-Maria Leppäpuska, M. Laukka, A. Saarikko, P. Hartiala","doi":"10.2458/lymph.5266","DOIUrl":"https://doi.org/10.2458/lymph.5266","url":null,"abstract":"Vascularized lymph node transfer surgery (VLNT) can provide benefit to lymphedema patients. Cytokines may play a role in the development of lymphedema and in the regeneration of lymphatic vessels after VLNT. Our primary aim was to investigate whether the VLNT patients have a specific cytokine profile. Our secondary aim was to see whether the preoperative lymphedema or severity affects the postoperative cytokine response. Wound exudate was gathered from 18 patients undergoing VLNT on the first and sixth postoperative day (POD). The concentrations of IL-10, TNF-α, TGF-β1 and VEGF-C were analyzed using enzymelinked immune-sorbent assays. A general score was generated to assess the benefit of the surgery. The changes in cytokine concentrations (1st POD-6th POD) were correlated with the pre- and postoperative lymphedema related factors. A shorter duration of lymphedema preoperatively correlated with an increase in the concentration of IL-10 and TNF-β during the first six PODs (IL-10: r=0.495, p=0.051; TNF-α: r=0.737, p=0.006) and a decrease in the concentration of TGF-ß1 (r= -0.613, p=0.020). The increase of the concentration of TNF-α during the first six PODs also correlated with a greater total general score (r=0.775, p=0.005) and hence indicated a better response to the surgery. The patients with a shorter duration of lymphedema preoperatively had a more favorable cytokine response during the first six PODs after VLNT.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 2 1","pages":"54-64"},"PeriodicalIF":2.5,"publicationDate":"2022-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45427540","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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