Chubby Infant – Should One Worry? An Infant with Primary lymphedema – Mini Review and Case Report

2020 ◽  
Author(s):  
Joanna Magdalena Kaczmarek ◽  
Karolina Anna Graczykowska ◽  
Iwona Szymkuć-Bukowska ◽  
Ewa Łoś-Rycharska ◽  
Aneta Krogulska
Keyword(s):  
Case Report ◽  
Lymphatic System ◽  
Subcutaneous Tissue ◽  
Pediatric Population ◽  
Tissue Fluid ◽  
Fat Tissue ◽  
Primary Lymphedema ◽  
Progressive Accumulation ◽  
Secondary Causes ◽  
Localized Form

AbstractLymphedema is a localized form of tissue swelling, characterized by a progressive accumulation of a tissue fluid in the interstitial compartment as a result of the lymphatic system dysfunction. It is a rare disease in the pediatric population and in the majority of cases it is a consequence of an abnormal formation of the lymphatic system, which is called primary lymphedema. Although its epidemiology is not precise, it is assumed that 1:100 000 children suffer from primary lymphedema. The diagnosis can be made by a proper clinical examination after ruling out secondary causes of lymphedema, particularly in cases with a more asymmetric swelling of the extremities. In this very article we present a case report of an 8-months-old infant with primary lymphedema, who had presented swelling of the extremities from birth and yet no pathology was suspected before. The purpose of this article is to draw attention to the fact that a baby with excessive subcutaneous tissue is not always a healthy, chubby infant with considerable amount of fat tissue.

Effect of skin concavity on subcutaneous tissue fluid pressure

1991 ◽  
Vol 261 (2) ◽  
pp. H349-H353 ◽  
Author(s):  
T. H. Adair ◽  
G. A. Vance ◽  
J. P. Montani ◽  
A. C. Guyton
Keyword(s):  
Linear Regression ◽  
Entire Range ◽  
Lymphatic System ◽  
Subcutaneous Tissue ◽  
Fluid Pressure ◽  
Tissue Fluid ◽  
Average Depth ◽  
Factors Associated ◽  
Mechanical Factors ◽  
Fascial Plane

We tested the hypothesis that mechanical factors associated, with a skin concavity can cause the local tissue fluid pressure to become more negative. Perforated Teflon collars, 26 mm in diameter and having various heights (5, 10, 13, and 16 mm), were implanted into the fascial plane of the inguinal and abdominal areas of six sheep. After several weeks, visible signs of edema were no longer apparent, and the skin formed a concavity within the center of each collar. The depth of each concavity was measured using an electronic micrometer, and the tissue fluid pressure beneath the concavity was measured using a needle method. Over the entire range of collar heights, the average depth of the concavities ranged from 1.1 to 4.7 mm in the abdominal tissues and from 1.8 to 5.5 mm in the inguinal tissues. The respective values of tissue fluid pressure averaged -4.6 to -13.0 and -5.7 to -12.8 mmHg. The results therefore indicate that implanting deeper collars leads to the formation of deeper concavities in the skin and also to greater negativity in the free tissue fluid pressure beneath the skin. Linear regression extrapolation to a collar height of 0 mm corresponded to a tissue fluid pressure of -1.0 mmHg in the abdominal tissue and -2.4 mmHg in the inguinal tissues. A model based on excessive pumping of the lymphatic system in the vicinity of a concavity is provided to explain this newly described phenomenon. We conclude that mechanical factors associated with the formation of a skin concavity cause or permit the tissue fluid pressure to reach levels of negativity far greater than those that exist in the absence of a concavity.

scholarly journals Linfedema Primário em Membro Superior Esquerdo em Paciente Idosa / Primary Lymphedema in Upper Left Member in Elderly Patient

2016 ◽  
Vol 6 (3) ◽  
pp. 133-141
Author(s):  
Melissa Andreia de Moraes Silva ◽  
Camila Bueno da Silva ◽  
Isabella João Milan ◽  
Seleno Glauber de Jesus-Silva ◽  
Rodolfo Souza Cardoso
Keyword(s):  
Quality Of Life ◽  
Elderly Patient ◽  
Case Report ◽  
Early Diagnosis ◽  
Rare Case ◽  
Lymphatic System ◽  
Multidisciplinary Treatment ◽  
Primary Lymphedema ◽  
Patient Prognosis

Introdução: O Linfedema Primário (LP) é caracterizado por disfunção do sistema linfático de etiologia idiopática. Este pode ser dividido em congênito, precoce e tardio, sendo o tipo congênito o mais raro. O diagnóstico desta patologia é predominantemente clínico,  podendo  ser  solicitado  linfocintilografia quando  há  dúvida  diagnóstica. O diagnóstico  precoce  está  diretamente  relacionado  com  o  melhor  prognóstico  do paciente, postergando limitações físicas e psico­sociais. O tratamento do LP é clínico e multiprofissional. Relato do Caso: Relatou­se caso raro de uma paciente de 78 anos de idade, sexo feminino, portadora de Linfedema Primário em membro superior esquerdo desde o nascimento, o qual foi investigado durante a infância, porém com resultados inconclusivos. Conclusão: Embora seja pequena a quantidade de trabalhos sobre o tema, este relato confirma que o diagnóstico precoce é fundamental e que o tratamento igualmente precoce e multiprofissional melhora a qualidade de vida dos pacientes.Palavras­chave:  Linfedema, Sistema Linfático, Extremidade Superior, Idoso, CintilografiaABSTRACTIntroduction: Primary lymphedema (PL) is characterized by dysfunction of the lymphatic system of idiopathic etiology. It can be divided into congenital, early and late,  with  the congenital type being the rarest. The diagnosis of this pathology is predominantly clinical, and a lymphoscintilography may be requested when the diagnostic is uncertain. Early diagnosis is directly related to better patient prognosis, delaying physical and psychosocial limitations. Treatment of PL is clinical and multiprofessional. Case Report: The aim of this paper is to describe a rare case of a 78­ year­old female with primary lymphedema in the left arm since her birth, which was investigated during childhood, but with inconclusive results. Conclusion: Although there is small  amount of work on the topic, this report confirms that early diagnosis is crucial and also that  early and multidisciplinary treatment improves the quality of life of patients.Keywords: Lymphedema, Lymphatic System, Upper Extremity, Elderly, Radionuclide Imaging  

Surgical treatment of alveolar echinococcosis: a single centre experience and systematic review of the literature

2019 ◽  
Vol 98 (4) ◽  
pp. 167-173
Keyword(s):  
Case Report ◽  
Echinococcus Multilocularis ◽  
Alveolar Echinococcosis ◽  
Lymphatic System ◽  
Surgical Intervention ◽  
Clinical Symptoms ◽  
Safety Margin ◽  
Surgical Removal ◽  
Single Centre Experience ◽  
Therapeutic Procedures

Introduction: Alveolar echinococcosis (AE) is a zoonosis caused by Echinococcus multilocularis. AE is primarily localised in the liver. Echinococcus multilocularis imitates tumour-like behaviour. It can metastasise through blood or lymphatic system to distant organs. Echinococcosis often remains asymptomatic due to its long incubation period and indistinct symptoms. Clinical symptoms are determined by the parasite’s location. Diagnosis of echinococcosis is based on medical history, clinical symptoms, laboratory tests, serology results, imaging methods and final histology findings. Surgical removal of the cyst with a safety margin, followed by chemotherapy is the therapeutic method of choice. Case report: We present a case report of alveolar echinococcosis in a thirty-year-old female patient in whom we surgically removed multiple liver foci of alveolar echinococcosis. The disease recurred after two years and required another surgical intervention. Conclusions: Alveolar echinococcosis is a disease with a high potential for a complete cure provided that it is diagnosed early and that the recommended therapeutic procedures are strictly adhered to.

scholarly journals Is it always Wilms’ tumor? Localized cystic disease of the kidney in an infant. An extremely rare case report and review of the literature

2020 ◽  
Vol 12 (1) ◽  
Author(s):  
Christos Kaselas ◽  
Charikleia Demiri ◽  
Vasilios Mouravas ◽  
Eleni Koutra ◽  
Kleanthis Anastasiadis ◽  
...  
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Wilms Tumor ◽  
Pediatric Population ◽  
Benign Disease ◽  
Cystic Disease ◽  
Proper Treatment ◽  
Review Of The Literature ◽  
Rare Case Report ◽  
Renal Malignancies

Localized Cystic Disease of the Kidney (LCDK) is an extremely rare benign disease in pediatric population. Although its management is conservative and generally requires no treatment, the unfamiliarity with the disease can expose such patients to misdiagnosis as renal malignancies or uncertainty for proper treatment. We report such a case in an infant and review the current literature.

Primary Cutaneous CD30-Positive Anaplastic Large Cell Lymphoma in Childhood: Report of 4 Cases and Review of the Literature

2005 ◽  
Vol 8 (1) ◽  
pp. 52-60 ◽  
Author(s):  
Shimareet Kumar ◽  
Stefania Pittaluga ◽  
Mark Raffeld ◽  
Michael Guerrera ◽  
Nita L. Seibel ◽  
...  
Keyword(s):  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Subcutaneous Tissue ◽  
Pediatric Population ◽  
Large Cell ◽  
Lymphoid Cells ◽  
Cell Phenotype ◽  
Anaplastic Lymphoma ◽  
Large Cell Lymphoma

We present the clinicopathologic findings in 4 children with primary cutaneous anaplastic large cell lymphoma (C-ALCL). The patients ranged in age from 13 months to 8 years, with 3 females and 1 male. All presented with a rapidly enlarging mass involving the skin and subcutaneous tissue. Histologic evaluation showed sheets of large pleomorphic lymphoid cells that were diffusely and strongly CD30+. Tumor cells were CD45+ in 1 of 4 cases. Cells were of T-cell phenotype, with variable positivity for CD3 (3 of 4 cases) and CD5 (2 of 4 cases). All 4 cases were positive for CD4 and clusterin. Staining for anaplastic lymphoma kinase was negative in all cases. No evidence of systemic involvement was noted at initial presentation or over a follow-up of 5 to 78 months, although 3 patients had cutaneous recurrences. Primary C-ALCL has only rarely been described in the pediatric population. The high-grade histologic appearance of this lymphoma belies its generally favorable clinical course and prognosis. Recognition of this entity and its differentiation from other T-cell lymphomas that secondarily involve the skin is important to avoid unnecessarily aggressive therapy in these children.

scholarly journals Unifocal Granuloma of Femur due to Langerhans' Cell Histiocytosis: A Case Report and Review of the Literature

2010 ◽  
Vol 2010 ◽  
pp. 1-3 ◽  
Author(s):  
Harpreet Singh ◽  
Satnam Kaur ◽  
P. Yuvarajan ◽  
Nishant Jain ◽  
Lalit Maini
Keyword(s):  
Case Report ◽  
Clinical Data ◽  
Case History ◽  
Langerhans Cell Histiocytosis ◽  
Eosinophilic Granuloma ◽  
Pediatric Population ◽  
Langerhans Cell ◽  
Long Bones ◽  
Review Of The Literature ◽  
Osteolytic Lesions

The radiological diagnosis of osteolytic lesions of the long bones in pediatric population constitutes a challenge when the case history and clinical data are uncharacteristic. We believe that the description of few clinically and histologically proven cases to verify the existence of radiological signs useful for diagnosis may be of interest. Here, we describe a case of Langerhans' cell histiocytosis (LCH) presenting as unifocal eosinophilic granuloma of femur along with a brief review of the literature.

An occult ectopic parathyroid adenoma in a pediatric patient: a case report and management algorithm

2017 ◽  
Vol 30 (9) ◽  
Author(s):  
Brent D. Bauman ◽  
Maria Evasovich ◽  
Amanda Louiselle ◽  
Eugene Zheng ◽  
Kevin Goodwin ◽  
...  
Keyword(s):  
Case Report ◽  
Parathyroid Adenoma ◽  
Pediatric Patient ◽  
Pediatric Population ◽  
Preoperative Imaging ◽  
Management Algorithm ◽  
Postoperative Mri ◽  
Complex Patients ◽  
Multiple Imaging ◽  
Parathyroid Adenomas

AbstractBackground:Hyperparathyroidism (HPT) is a rare disease in the pediatric population, and optimal management may be unclear if it is due to an occult parathyroid adenoma. We present a case report of a pediatric patient with an occult, ectopic, supernumerary, parathyroid adenoma.Case presentation:A 13-year-old female who initially presented with anxiety was diagnosed with HPT. Preoperative imaging and bilateral neck exploration with four-gland biopsy were negative for any parathyroid adenoma. Postoperative MRI identified a thymic mass. She subsequently underwent video-assisted thoracoscopic thymectomy with resection of an intrathymic parathyroid adenoma.Conclusions:The diagnosis of pediatric HPT is increasing. Supernumerary or occult parathyroid adenomas are rare and add complexity to presurgical planning and management. Our case represents the rare occurrence of a pediatric ectopic supernumerary occult parathyroid adenoma treated with a two-stage approach utilizing multiple imaging studies. We provide a review of the pathology and propose an algorithmic approach to manage these complex patients.

scholarly journals Platelets Disseminate Extracellular Vesicles in Lymph in Rheumatoid Arthritis

2020 ◽  
Vol 40 (4) ◽  
pp. 929-942 ◽  
Author(s):  
Nicolas Tessandier ◽  
Imene Melki ◽  
Nathalie Cloutier ◽  
Isabelle Allaeys ◽  
Adam Miszta ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Extracellular Vesicles ◽  
Lymphatic System ◽  
Circulatory System ◽  
Donor Cell ◽  
Vascular Leakage ◽  
In Vivo Model ◽  
Interstitial Tissue ◽  
Tissue Fluid

Objective: The lymphatic system is a circulatory system that unidirectionally drains the interstitial tissue fluid back to blood circulation. Although lymph is utilized by leukocytes for immune surveillance, it remains inaccessible to platelets and erythrocytes. Activated cells release submicron extracellular vesicles (EV) that transport molecules from the donor cell. In rheumatoid arthritis, EV accumulate in the joint where they can interact with numerous cellular lineages. However, whether EV can exit the inflamed tissue to recirculate is unknown. Here, we investigated whether vascular leakage that occurs during inflammation could favor EV access to the lymphatic system. Approach and Results: Using an in vivo model of autoimmune inflammatory arthritis, we show that there is an influx of platelet EV, but not EV from erythrocytes or leukocytes, in joint-draining lymph. In contrast to blood platelet EV, lymph platelet EV lacked mitochondrial organelles and failed to promote coagulation. Platelet EV influx in lymph was consistent with joint vascular leakage and implicated the fibrinogen receptor α2bβ 3 and platelet-derived serotonin. Conclusions: These findings show that platelets can disseminate their EV in fluid that is inaccessible to platelets and beyond the joint in this disease.

scholarly journals Supratentorial Tanycytic Ependymoma in an Adult Male: Case Report and Review of Literature

2015 ◽  
Vol 8 (1) ◽  
pp. 159-163 ◽  
Author(s):  
Giselle Lopez ◽  
Roger E. McLendon ◽  
Katherine B. Peters
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Adult Male ◽  
Clear Cell ◽  
Pediatric Population ◽  
Ependymal Cells ◽  
Review Of Literature ◽  
Tanycytic Ependymoma ◽  
Central Nervous System Neoplasm

Ependymomas, tumors of the ependymal cells, are very rare and usually present in the pediatric population. Furthermore, there are even rarer variants of ependymomas that can include cellular, papillary, clear cell, and tanycytic subtypes. We present a case of a supratentorial tanycytic ependymoma in an adult male and review the literature in regard to this rare primary central nervous system neoplasm.

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