Presentation
Abstract Fetal edema can present as limited subcutaneous edema, fluid accumulation in body cavities or hydrops fetalis. [ncbi.nlm.nih.gov]
Most affected individuals presented with bilateral congenital lower-limb lymphedema. [doi.org]
The information presented here is not intended to diagnose, treat, cure or prevent any disease. Read disclaimer. [gulfdoctor.net]
Entire Body System
- Lymphedema of the Lower Extremity
A seven-year-old girl with chronic hereditary lymphedema of the lower extremities had bilateral conjunctival lymphedema at birth. A swelling of the lower extremities developed shortly thereafter. [jhu.pure.elsevier.com]
Lymphangiosarcoma (LAS) may occur as a rare complication of primary lymphedema. A case of LAS in hereditary lymphedema of the lower extremity in a 36-year old female is reported. [ncbi.nlm.nih.gov]
Lymphedema of the lower extremity, initially involving the foot, gradually progresses up the leg so that the entire limb becomes edematous (marked by edema). In the early stages of lymphedema, the edema is soft and pits easily with pressure. [healthcentral.com]
Lymphedema of the lower extremity: is it genetic or nongenetic? Clin Pediatr (Phila). 2007 Nov;46(9):835-41. Epub 2007 Jul 19. [ghr.nlm.nih.gov]
- Localized Edema
2017 2018 2019 Billable/Specific Code Applicable To Fluid retention NOS legs R60.0 ICD-10-CM Diagnosis Code R60.0 Localized edema 2016 2017 2018 2019 Billable/Specific Code hereditary Q82.0 chronic hereditary Q82.0 familial, hereditary Q82.0 hereditary [icd10data.com]
- Hyperthermia
Despite of chemotherapy, local hyperthermia and later amputation of the extremity the patient died of progressive disease due to pulmonary metastasis. [ncbi.nlm.nih.gov]
Jaw & Teeth
- High Arched Palate
The Turner syndrome phenotype includes short stature, stature disproportion, primary amenorrhea, neck webbing, congenital lymphedema of the hands and feet, high-arched palate, short metacarpals, scoliosis, Madelung deformity, hearing difficulties, cardiac [ncbi.nlm.nih.gov]
Workup
"Lymphedema: Diagnostic workup and management". Journal of the American Academy of Dermatology. 77 (6): 995–1006. doi : 10.1016/j.jaad.2017.03.021. ISSN 1097-6787. [en.wikipedia.org]
Treatment
Treatment Decongestive therapy is the most widely accepted form of treatment. There is no cure for Milroy's but the condition can be managed by early diagnosis and treatment. [lymphedemapeople.com]
MEDIATORS OF PRURITUSDRUG TREATMENT OF PRURITUS; FUTURE DIRECTIONS; REFERENCES; Chapter 7. [worldcat.org]
I’d love to hear your experiences in receiving diagnosis and treatment in the comments! [thelymphielife.com]
Treatment is aimed at reducing swelling and preventing infection. [rarediseases.org]
Continued Treatment Your doctor may recommend: Compression garments: These fabric sleeves apply pressure to the affected limb to help lymph fluid circulate. [webmd.com]
Prognosis
In fact, although the prognosis of this neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can increase the possibility of survival of these patients. [ncbi.nlm.nih.gov]
Prognosis Long term prognosis is excellent is the condition is identified early and treatment begins so after the diagnosis is made ======================================================= National Organization for Rare Disorders, Inc. [lymphedemapeople.com]
In fact, although the prognosis of this neoplasm is very poor, prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can increase the possibility of survival of these patients. [sc.ehu.es]
The other one-third of patients have a poor prognosis and very often need interventions. Lymphedema praecox, the most common form of primary lymphedema, is seen mostly in females. [cincinnatichildrens.org]
Etiology
Hydrops fetalis is the end stage of a variety of fetal/maternal disorders and nonimmune etiology represents more than 3/4 of cases. [ncbi.nlm.nih.gov]
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
The findings support the recommendation that when peripheral lymphedema of undetermined etiology is found, further careful, comprehensive clinical, including detailed dysmorphic, evaluation along with lymphatic imaging with subsequent syndromic classification [arizona.pure.elsevier.com]
Epidemiology
Erythroderma; INTRODUCTION; HISTORY; EPIDEMIOLOGY; PATHOGENESIS; CLINICAL FEATURES; PATHOLOGY; DIFFERENTIAL DIAGNOSIS; TREATMENT; REFERENCES. Chapter 12. [worldcat.org]
Thelma, Expansion mutation frequency and CGG/GCC repeat polymorphism inFMR1 andFMR2 genes in an Indian population, Genetic Epidemiology, 20, 1, (129), (2001). [dx.doi.org]
Epidemiology of elephantiasis with special emphasis on podoconiosis in Ethiopia: A literature review. J Vector Borne Dis. 2015 Jun. 52 (2):111-5. [Medline]. Elgendy IY, Lo MC. [emedicine.medscape.com]
"The patient record in epidemiology". Scientific American. 245 (4): 54–63. doi : 10.1038/scientificamerican1081-54. ISSN 0036-8733. PMID 7027437. ^ a b c d e f Grada, Ayman A.; Phillips, Tania J. (December 2017). [en.wikipedia.org]
Pathophysiology
Modes of inheritance, associated clinical features and images, and specific organ involvement were inventoried and suggested pathophysiologic mechanisms noted. [arizona.pure.elsevier.com]
Modes o f inheritance, associated clinical features and images, and specific organ involvement were inventoried and suggested pathophysiologic mechanisms noted. [eurolymphology.org]
Arnold Publishers (pg. 82 - 86 ) 24, Lymph Stasis: Pathophysiology, Diagnosis, and Treatment, 1991 Boca Raton, FL CRC Press (pg. 157 - 190 ) 25, Lymph Stasis: Pathophysiology, Diagnosis, and Treatment, 1991 Boca Raton, FL CRC Press (pg. 191 - 210 ) 26 [hmg.oxfordjournals.org]
Prevention
Treatment Patients with lymphedema of the lower extremities must be instructed to take meticulous care of their feet to prevent recurrent lymphangitis. Skin hygiene is important and emollients can be used to prevent drying. [healthcentral.com]
Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates [icdlist.com]
You may also wear them to help prevent lymph fluid from going back into your affected limb. [webmd.com]