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Results for “Lymphatic Abnormalities”

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PubMed1989

Lymphatic abnormalities in fetuses with posterior cervical cystic hygroma.

We studied the structure and number of lymph vessels in 12 spontaneously aborted previable fetuses with posterior cervical cystic hygroma and generalized edema of variable origin (monosomy X, trisomy 21, trisomy 13, suspected Noonan syndrome, and lethal multiple pterygium syndrome) and compared them to 5 therapeutically aborted, apparently normal fetuses. We found that in the non-45,X fetuses with cystic hygroma and edema the lymphatic vessels at all studied sites were dilated and appeared increased in number. The 45,X fetuses had no recognizable lymphatic vessels in the edematous cutaneous tissue of the limbs, and only occasional dilated vessels in the wall of the nuchal cystic hygroma and in the lungs. These findings may be useful in differentiating between monosomy X and other conditions causing nuchal cystic hygroma in specimens in which the fetus was incomplete and/or cytogenetic study could not be done.

Aorta↗
PubMed1996

Ultrasonographic evidence of abnormal lymphatic vessels in young men with adult Wuchereria bancrofti infection in the scrotal area.

PURPOSE: We determined the prevalence and magnitude of dilatation of the lymphatic vessels of the spermatic cord in men infected with Wuchereria bancrofti, which is known major cause of hydrocele in the tropics. MATERIALS AND METHODS: Scrotal ultrasound was performed with a 7.5 MHz, transducer in 78 men from Recife, Brazil (endemic for filariasis) and in 15 from a nonendemic area. RESULTS: Among men from Recife the lymphatic vessels were dilated (1.3 to 15.0 mm., mean 3.8) at the location of the adult worm. Vessel diameter was not associated with hydrocele. CONCLUSIONS: Lymphatic dilation was observed in all men with ultrasonographically detectable W. bancrofti infection, even those who were asymptomatic.

Adolescent↗
PubMed2002

Abnormal lymphatic vessel development in neuropilin 2 mutant mice.

Neuropilin 2 is a receptor for class III semaphorins and for certain members of the vascular endothelial growth factor family. Targeted inactivation of the neuropilin 2 gene (Nrp2) has previously shown its role in neural development. We report that neuropilin 2 expression in the vascular system is restricted to veins and lymphatic vessels. Homozygous Nrp2 mutants show absence or severe reduction of small lymphatic vessels and capillaries during development. This correlated with a reduction of DNA synthesis in the lymphatic endothelial cells of the mutants. Arteries, veins and larger, collecting lymphatic vessels developed normally, suggesting that neuropilin 2 is selectively required for the formation of small lymphatic vessels and capillaries.

Animals↗
PubMed1979

Prenatal diagnosis of fetal lymphatic system abnormalities by ultrasound.

Antenatal ultrasound diagnoses of gross lymphatic system abnormalities were made in three fetuses. Although the diagnosis was made in only 1 fetus at a stage early enough in pregnancy to allow selective termination, knowledge of the abnormality in the remaining 2 fetuses proved to be valuable for subsequent management of those pregnancies. It is stressed that the extent of the abnormality must be carefully assessed because of the possibility of corrective surgery should the lesion be small and that the parents must be given detailed counseling before any definitive measures are taken.

Abortion, Therapeutic↗
PubMed2000

Polymicrogyria associated with scalp and limb defects: variant of Adams-Oliver syndrome.

We describe cortical malformations in two siblings who also had features of Adams-Oliver syndrome (AOS, MIM 100300). The parents were first cousins and showed no signs of either disorder, suggesting autosomal recessive inheritance. Psychomotor delay was present in both sibs, and cerebral imaging was indicative of polymicrogyria (PMG). One sib had aplasia cutis congenita of the scalp and transverse limb defects, and the other had short fingers and toes and also developed lymphedema of the right leg. CNS abnormalities and lymphatic abnormalities are rare manifestations of AOS, and we suggest that these sibs have a rare variant of AOS with probable recessive inheritance.

Abnormalities, Multiple↗
PubMed1982

Ultrasonographic diagnoses of major lymphatic system abnormalities prior to 20 weeks of pregnancy.

Prenatal ultrasound diagnoses of severe lymphatic system abnormalities were obtained in three fetuses. In all three cases the diagnosis was established prior to 20 wk of pregnancy, i.e. early enough to allow selective termination. In all three cases male fetuses with a chromosomal anomaly were found. Alpha-fetoprotein values were not elevated. In all cases the fetal abnormalities were of such size that surgical excision could not be taken into consideration to provide satisfactory results for the infants.

Abortion, Therapeutic↗
PubMed1993

Congenital pulmonary lymphangiectasis with chylothorax: a heterogeneous lymphatic vessel abnormality.

We report on 7 perinatal autopsy cases of primary congenital pulmonary lymphangiectasis (CPL) with bilateral chylothorax. This study demonstrates that primary CPL is often complicated by chylous pleural effusions with ensuing pulmonary hypoplasia. Conversely, CPL appears to be a constant pathological finding in spontaneous congenital chylothorax. These observations indicate a common pathogenesis for both disorders. The basic defect is not an intrinsic lung abnormality, but a developmental error of the lymphatic system resulting in a pulmonary lymphatic obstruction sequence. The cause of CPL is heterogeneous. Apparently, most cases are sporadic occurrences. We report the second instance of CPL in sibs. This indicates that some cases are genetically determined with autosomal recessive inheritance. CPL may also be part of a multiple congenital anomalies (MCA) syndrome such as Noonan, Ullrich-Turner, and Down syndrome.

Abnormalities, Multiple↗
PubMed1995

Evaluation of lymphatic function: abnormal lymph drainage in venous disease.

The essential function of the lymphatic system is to return to the vascular system extravascular molecules and colloids too large to re-enter directly. Quantitative lymphoscintigraphy employs this principle and has proved useful in the differential diagnosis of chronic limb swelling, in the identification of subtle or incipient lymphoedema and in edema of compound origin where a lymphatic component would otherwise go unnoticed. In a study exploring the contribution of lymphatic insufficiency to poor wound healing in chronic venous leg ulceration 32 patients were compared to 22 normal control subjects using quantitative lymphoscintigraphy. In subjects less than 65 years lymphatic function was reduced in the ulcerated limbs compared to normal limbs (p<0.0001). In those aged more than 65 years lymphatic function was lower in ulcerated limbs but not significantly so, owing to a decline in lymph drainage with age in normal controls (r = 0.62, p = 0.0001). In patients with unilateral leg ulceration lymphatic function was reduced in the ulcerated limb compared with the contralateral leg (<65 years, p = 0.05; >65 years, p = 0.03). The finding of impaired lymph drainage with chronic venous insufficiency suggests that lymphatic pathology may be as important as venous pathology in the "chronic venous leg ulcer" and treatment should be aimed at improving lymphatic as well as venous function.

Adult↗
PubMedDate not supplied

[Lymphedemas of the lower limbs: a lymphoscintigraphic study].

UNLABELLED: Patients with lymphoedema of the lower limbs (LLL) are usually affected by recurrent erysipelas. This bacterial infection is usually admitted as an aetiological factor, but it can be a consequence of some previous lymphatic abnormality. Classically, the commonest causative factors of LLL are bacterial infection, venous ulcers, trauma (leading to secondary lymphoedema) and congenital disorders of the lymphatic system (primary lymphoedema--praecox or tarda). PURPOSE: To identify previous lymphatics abnormalities in patients with LLL, admitted as having secondary lymphatic lymphoedema--as consequences of infection, trauma or other factors--, by using lymphoscintigraphic method. To observe advantages of this approach in practical assistance and evaluation of LLL cases. METHODS: Twelve patients with LLL, supposed to have primary (congenital) or secondary disorder affecting one or both lower extremities were submitted to lymphoscintigraphy at the Escola Paulista de Medicina of Sao Paulo, Brazil. Each patient received an intradermal injection of labelled Dextran (Dx-99mTc) at the first interdigital space of each foot and, after one to three hours, images of lower extremities, pelvic and abdominal areas were obtained with Gammatome CGR. RESULTS: Examination of all 24 lower extremities disclosed clinical diagnosis of lymphoedema in 17 (70.8%), being five (41.6%) with clinical signs of lymphoedema of both lower limbs and seven (58.3%) of a single one. The lymphoscintigraphic images revealed lymphatic disorders in 22 of the 24 extremities (91.7%). Lymphoscintigraphic abnormalities in clinically normal lower extremities were observed in five cases (41.3%). CONCLUSION: Lymphoscintigraphy is a non-invasive useful method to study LLL, with involvement of one or both limbs. It does not differentiate between primary and secondary lymphoedema, but makes possible to detect cases of normal appearance of the limbs with previous lymphatic defect(s), changing aetiological diagnosis. Some cases that appear to be secondary lymphoedema could be, in fact, associated with congenital abnormality(ies) of lymphatics, triggered by factors like trauma or venous ulcers.

Adult↗
PubMed1998

Lymphoscintigraphy and lymphangiography of lymphangiectasia.

Chronic genital edema secondary to lymphangiectasia and chylous reflux in a 23-yr-old man with Noonan syndrome was investigated by 99mTc sulfur nanocolloid lymphoscintigraphy and bipedal contrast lymphangiography. Lymphoscintigraphy showed a delayed lymphatic flow pattern in the pelvis, abdomen and chest consistent with lymphangiectasia and abnormal lymphatic flow dynamics. Lymphangiography showed dilated and tortuous abnormal lymphatics in the abdomen and pelvis. Ligation of incompetent retroperitoneal lymph vessels and lymphaticovenous anastamosis were performed, resulting in clinical improvement. Lymphangiectasia has been described previously in Noonan syndrome, but it is relatively uncommon below the diaphragm. This case demonstrates the use of lymphoscintigraphy and lymphangiography in providing important physiological and anatomical information before surgical intervention. Careful presurgical planning using such tests also allows the most appropriate operation to be performed.

Adult↗