Edit concept Question Editor Create issue ticket

Lymphatic Malformation


Presentation

  • We discuss the causes of delayed presentation of these congenital lesions. An overview of the causation, natural history, diagnosis, and treatment options is presented.[ncbi.nlm.nih.gov]
  • In this study, we present a case report of a pediatric lymphatic malformation of the sphenoid sinus.[ncbi.nlm.nih.gov]
  • They are uncommon and can present in various ways.[ncbi.nlm.nih.gov]
  • We report a neonate with a lymphatic malformation (lymphangioma) initially confined to the larynx that presented with stridor and progressive respiratory failure.[ncbi.nlm.nih.gov]
  • The case presented in this report is a rare solitary gingival involvement of a microcystic-type lymphatic malformation. The lesion presented clinically as a small vesicular swelling of a buccal interdental papilla in a 16-year-old girl.[ncbi.nlm.nih.gov]
Steatorrhea
  • She did not have common symptoms of intestinal vascular and lymphatic malformation, such as gastrointestinal bleeding, steatorrhea, and hypoalbuminemia.[ncbi.nlm.nih.gov]
Pelvic Mass
  • This case is different from the 3 cases reported before, for it is only presented with a palpable pelvic mass and the lesion did not penetrate full thickness of bladder.[ncbi.nlm.nih.gov]
Petechiae
  • Examination under anesthesia and vaginoscopy showed a congested appearance of the hymen and vaginal petechiae.[ncbi.nlm.nih.gov]
Miosis
  • Twenty-one weeks after initial treatment, ophthalmic examination showed complete resolution of the blepharoptosis and pupillary miosis.[ncbi.nlm.nih.gov]
Strabismus
  • Amblyopia, astigmatism, and strabismus were not improved after treatment. All of the patients had excellent aesthetic improvement and corrected blepharoptosis.[ncbi.nlm.nih.gov]
  • If it is localized around the eye, it may cause proptosis (push the eye forward), increased intraocular pressure, strabismus, diplopia and even vision loss.[texaschildrens.org]
Blurred Vision
  • A 41-year-old woman presented with several days of right eye pain and blurred vision. Examination and radiologic workup were consistent with a veno-lymphatic malformation, including demonstration of cystic fluid-fluid levels on imaging.[ncbi.nlm.nih.gov]
Chemosis
  • OBJECTIVES: Periorbital microcystic lymphatic malformations (LM) can cause severe symptoms, such as blepharoptosis, amblyopia, chemosis, strabismus, diminished vision, and blindness.[ncbi.nlm.nih.gov]
Unilateral Proptosis
  • Prenatal ultrasound revealed prominence of the left orbital soft tissue, and at birth, the patient was noted to have unilateral proptosis, tortuous retinal vessels, and intraretinal hemorrhages in all 4 quadrants in the left eye.[ncbi.nlm.nih.gov]
Vaginal Bleeding
  • Premenarchal vaginal bleeding can present a diagnostic challenge and has not been previously reported in association with congenital venous lymphatic malformation. A 3-year-old girl presented with intermittent vaginal bleeding since birth.[ncbi.nlm.nih.gov]
Abnormal Gait
  • We report two cases of giant LM with occasional high fever and pain or abnormal gait. To overcome dose limitation and to prevent systematic toxicities, we performed both injection of absolute ethanol and aspiration of it after 5 min exposure to LM.[ncbi.nlm.nih.gov]

Workup

  • Examination and radiologic workup were consistent with a veno-lymphatic malformation, including demonstration of cystic fluid-fluid levels on imaging.[ncbi.nlm.nih.gov]
  • Genioplasty Indications horizontal mandibular excess or asymmetry, need for mandibular advancement Contraindications co-morbidities increasing surgical risk Setup supine, nasal intubation, general anesthesia, Inferior alveolar nerve block Preoperative Workup[csurgeries.com]
  • Click here to go to a related article (Appropriate Diagnostic Workup for Birthmarks).[birthmarks.us]
  • […] histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed “diffuse pulmonary lymphangiomatosis” (DPL). 3 Recognition of the disease requires a high index of suspicion and an extensive workup[lgdalliance.org]
Human Herpesvirus 8
  • All cases tested showed negative staining for Ki-67 and human herpesvirus 8 latent nuclear antigen. LIMITATIONS: There are no limitations.[ncbi.nlm.nih.gov]
  • Histopathological examination and immunohistochemical staining of CD31, D2-40, Prox1, Wilms tumor 1 (WT-1) and human herpesvirus-8 (HHV-8) were performed for all cases.[ncbi.nlm.nih.gov]

Treatment

  • No treatment modality clearly resulted in superior treatment outcome. Complications were more frequent in surgical series but were reported inconsistently in sclerotherapy studies.[ncbi.nlm.nih.gov]
  • Because no specific treatment has yet been approved for MLM, and as a step before the use of aggressive systemic or intralesional treatments, it was decided to initiate treatment with 1% rapamycin ointment.[ncbi.nlm.nih.gov]
  • The mean follow-up time was 47 months after treatment. At the most recent clinic evaluation, 13 patients (50%) were symptom free, 8 (31%) were stable and improved without need for future treatment, and 5 (19%) required further treatment.[ncbi.nlm.nih.gov]
  • It is very rare at birth and its treatment remains controversial.[ncbi.nlm.nih.gov]
  • Promisingly, few case studies indicate topical sirolimus as a potential treatment for the lymphatic malformations.[ncbi.nlm.nih.gov]

Prognosis

  • Surgical excision is ideal to treat LM, and the prognosis is good. Although asymptomatic horseshoe kidney need not be treated, it is important for patients to receive regular follow-up because of the propensity for various complications.[ncbi.nlm.nih.gov]
  • Once excised completely, the prognosis of the disease is good.[ncbi.nlm.nih.gov]
  • The prognosis of primary lymphedema of the lower limbs. Arch Surg . 1981. Google Scholar 15. Williams WH, McNeill GC, Witte MH, et al. Evaluation of peripheral lymphedema by longitudinal lymphangioscintigraphy. Lymphology . 2002;35.[link.springer.com]
  • It is also generally true that facial and oropharyngeal involvement are associated with a poor prognosis.[edmondsmd.com]
  • [ edit ] The prognosis for lymphangioma circumscriptum and cavernous lymphangioma is generally excellent.[en.wikipedia.org]

Etiology

  • Stridor in a neonate evokes a series of prompt evaluations to determine the etiology. Common etiologies include inherent structural defects (such as laryngomalacia), vocal fold paralysis, acquired infectious etiologies, or extrinsic compressions.[ncbi.nlm.nih.gov]
  • METHODS: We present an unusual location for the development of a cystic lymphangioma with emphasis on etiology, clinical implications, and current treatment.[ncbi.nlm.nih.gov]
  • We hypothesized that they might share the similar etiologic factors.[ncbi.nlm.nih.gov]
  • Congenital venous lymphatic malformations can rarely be associated with premenarchal vaginal bleeding and should be considered when all more common etiologies have been ruled out.[ncbi.nlm.nih.gov]
  • An overview of the etiology, diagnosis, histology, and options for the management of such lesions is outlined.[ncbi.nlm.nih.gov]

Epidemiology

  • A short review of epidemiology, clinical manifestation and treatment is given.[ncbi.nlm.nih.gov]
  • Summary Epidemiology Prevalence is unknown.[orpha.net]
  • Neurofibroma Lymphoproliferative Lymphoma Langherhans cell histiocytosis Xanthogranuloma Mesenchymal Rhabdomyosarcoma Fibrous histiocytoma Meningioma Optic nerve glioma Neurofibroma Metastasis Lung, Breast, Prostate Neuroblastoma Leukemia Summary Chart Epidemiology[eyerounds.org]
  • Discussion Epidemiology Lymphangiomas account for about 5% of benign tumors seen in infants and children. 1,2 The sites most commonly involved are the neck (75%) and axillae (20%). 3,4 Less than 1% of lymphangiomas are found in the gastrointestinal tract[gastroenterologyandhepatology.net]
Sex distribution
Age distribution

Pathophysiology

  • The purpose of this study was to determine the natural progression of LMs to facilitate patient counseling, gain insight into pathophysiology, and guide therapy.[ncbi.nlm.nih.gov]
  • The pathophysiology of lymphedema. Cancer . 1998. Google Scholar 13. Papendieck CM. Lymphangiomatosis and dermoepidermal disturbances of lymphangioadenodysplasias. Lymphology . 2002;35. Google Scholar 14. Wolfe JH, Kinmonth JB.[link.springer.com]
  • A similar phenotype is seen in the Chy mouse, which carries a spontaneous heterozygous point mutation in Vegfr3 and is a model to study the pathophysiology and to perform preclinical trials of Nonne-Milroy lymphedema.[jci.org]
  • Pathophysiologic considerations and management recommendations. Ophthalmology. 1990 Dec;97(12):1583-92. PubMed PMID: 2087290. Sires BS, Goins CR, Anderson RL, Holds JB. Systemic corticosteroid use in orbital lymphangioma.[eyerounds.org]

Prevention

  • The treatment is complete surgical excision, in order to prevent recurrence.[ncbi.nlm.nih.gov]
  • To overcome dose limitation and to prevent systematic toxicities, we performed both injection of absolute ethanol and aspiration of it after 5 min exposure to LM.[ncbi.nlm.nih.gov]
  • CONCLUSION: RFA ablates the lesions and achieves hemostasis while the sclerosant injected in and around the lesion reaches the deeper vascular lesions, preventing recurrence.[ncbi.nlm.nih.gov]
  • Since a lymphatic malformation will never go away on its own, expert treatment is required to heal this condition, prevent infection and prevent complications.[spectrumhealth.org]
  • Can they be prevented? No, as they occur so early in pregnancy, they cannot be prevented. However, the effects of the lymphatic malformation can be reduced or prevented.[gosh.nhs.uk]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!