Pulmonary Lymphangiectasia

Pulmonary Lymphangiectasia

DEFINITION:

A congenital disease involving the lung characterized by greatly dilated lymphatic ducts throughout the lung.

EPIDEMIOLOGY:

incidence:

age of onset:neonatal period and after the neonatal periodrisk factors:Noonan's SyndromeTurner's Syndrome

TYPES

(after Noonon (1970))*:

1. Group Idilated pulmonary lymphatics are part of a generalized form of lymphangiectasis: thoracic and extrathoracic (intestinal) lymphangiectasiaassociated with
lymphedemahemihypertrophydiffuse angiomatosis in which bone is the most common site of involvementless severe form and has a much better prognosis than the other two forms

2. Group IIdue to a cardiac lesiondilated pulmonary lymphatics are acquired in utero due to obstruction of the pulmonary venous flow secondary to a cardiac lesion which may interfere with the normal regression of the lymphatic tissue elements after the 16th week of fetal lifevery poor prognosis

3. Group IIIdue to a defect in the primary development of the lungLawrence (1955) postulated that lymphangectasis is due to a developmental error in which the normal regression of connective tissue elements in the lung fails to occur after the 16th week of life. Subsequently the lymph vessels do not regress in size and remain enlarged in relation to the parenchyma of the lung (Noonan, 1970).male predominancemay be associated with congenital malformations including cardiac lesionsclinical picture characterized by over expanded lungs with alveolar hypoventilation and periodic wheezing and a relapsing coursedilated lymphatics may result in marked restriction of respiratory movements poor prognosis

*first report of CPL by Virchow (1856) and first named by Lawarence in 1955

CLINICAL FEATURES:

presents in two forms:1. Neonatal Periodpresents with respiratory distress and cyanosis at birth(usually term) with rapid demisecan also present as a stillbirth2. Post Neonatal Periodpresents with respiratory difficultiesprogressive relapsing courseassociated with chylopericardium, chylothorax, chronic cough, congestive heart failure3. Associated Abnormalitiescongenital abnormalities in 50% of cases:1. CardiacTAPVR, mitral valve atresia, hypoplastic left heart, aortic coarctation, VSD, PDA, ASD

2. Otherscystic hygromas, diffuse angiomatosis (bones), pneumothoraxINVESTIGATIONS:
Diagnostic

1. Lung Biopsycystic dilation of the lymphatics in the subpleural and intralobular regions and perivascular spaces give the lungs a subpleural reticulated pattern of fine interconnecting white lines with air-filled cystic areas

2. Imaging Studies

Chest X-Rayhyperexpansiondiffuse reticular pattern

MANAGEMENT:

supportive

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Pulmonary Lymphangiectasia:

Viewcases of pulmonary lymphangiectasis

Clinical:

Pulmonary lymphangiectasia is a rare disorder that is seen more frequently in males. It occurs secondary to a developmental defect which results in obstructive, dilated lymphatics and subsequently "wet lungs" (ie: there is arrested lymphatic development). Patients may present with severe neonatal respiratory distress, cyanosis, and death. There are questionable associations with familial cases, Noonan's syndrome, and Ichthyosis congenita [1]. There are 3 forms of the disorder:

1- Isolated Pulmonary: The developmental defect is isolated to the pulmonary lymphatics. Felt to be related to failure of regression of the pulmonary lymphatics after the 16th week of gestation. It results in severe interstitial lung disease and has a poor prognosis. Rarely children may live beyond the neonatal period.

2- Secondary to Pulmonary Venous Obstruction: This form is associated with congenital heart disease and accounts for 30% of cases. Cardiac abnormalities include: Hypoplastic left heart, total anomalous pulmonary venous return type III, and pulmonic vein atresia.

3- Generalized: (Systemic)- Characterized by lymphangiectasia throughout the body. The pulmonary manifestations are typically mild. Patients may have an associated lymphangioma.

Article

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Congenital dilatation of the pulmonary lymphatics

SN Javett, I Webster and JL Braudo904 Medical Centre Jeppe Street, Johannesburg, South Africa

The disease variously referred to as congenital dilatation of the pulmonary, lymphatics congenital pulmonary cystic lymphangiectasis, and congenital lymphangiomatosis of the lung is a rarity known almost exclusively to pediatric pathologists. Up to 1959, 21 cases had appeared in world literature, with only two reports in clinical journals. Congenital dilatation of the pulmonary lymphatics has been observed as a chance finding in fetuses dying in utero, stillborn infants, and neonates, without any mention being made as to its possible significance. The great majority of those born alive have suffered in the immediate neonatal period from an acute respiratory disease exhibiting dyspnea and cyanosis, death following within a few hours. As a consequence, the opportunity for clinical observation and investigation has been strictly limited. Confirmation has been possible only by autopsy. With virtually no clinical information to work on, the pathologists have centered interest almost entirely on the identification of lymphatics, the histopathology, and theories of causation without being in a position to offer correlation between histologic findings and symptoms. In the absence of any clinical build-up, too, it is not surprising that the disease has escaped the attention of most clinicians. We have had the opportunity of observing and investigating a patient with congenital dilatation of the pulmonary lymphatics, still alive, albeit not well, at the age of 8 months. It is the purpose of this paper to describe as a clinical entity a condition a clinical entity a condition hitherto regarded as a pathological curiosity.

Article

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Primary pulmonary lymphangiectasia in infancy and childhood

P.M. Barker1, C.R. Esther, Jr1, L.A. Fordham2, S.J. Maygarden3 and W.K. Funkhouser3
Depts of 1 Paediatrics (Division of Paediatric Pulmonology), 2 Radiology, and 3 Pathology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA Correspondence: P.M. Barker, Division of Paediatric Pulmonology, Dept of Paediatrics, 200 Mason Farm Road, CB 7220, University of North Carolina at Chapel Hill, Chapel Hill, NC, 27599, USA. Fax: 1 9199666179. E-mail: Pierre_Barker@med.unc.edu

Keywords: Growth, pathology, radiology, spirometry

Received: February 4, 2004Accepted May 20, 2004

C.R. Esther Jr is supported by a Parker B. Francis Pulmonary Fellowship.

Primary pulmonary lymphangiectasia (PPL) is a rare disorder of unknown aetiology characterised by dilatation of the pulmonary lymphatics. PPL is widely reported to have a poor prognosis in the neonatal period and little is known about the clinical features of patients who survive the newborn period.

The current authors report the outcome in nine patients diagnosed in infancy with PPL over a 15-yr period at a single university-based hospital clinic and followed for a median of 6 yrs.

Although all of the patients initially experienced respiratory distress, respiratory symptoms improved in most patients after infancy and were notably better by the age of 6 yrs. Many patients had poor weight gain in the first years of life, which eventually improved. Radiological scans showed progressive resolution of neonatal infiltrates, but were characterised by hyperinflation and increased interstitial markings in older children. Most patients had evidence of bronchitis and grew pathogenic organisms from quantitative bronchoalveolar lavage culture. Pulmonary function tests showed predominantly obstructive disease that did not deteriorate over time.

In conclusion, these results suggest that primary pulmonary lymphangiectasia does not have as dismal a prognosis as previously described and symptoms and clinical findings improve after the first year of life.

ersjournals

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Pulmonary Lymphangiectasia

Paediapaedia: Neonatal Chest DiseasesMichael P. D'Alessandro, M.D

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Congenital Pulmonary Lymphangiectasia

New England Journal of Medicine

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Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy

Find Articles

Renal Lymphangiectasia

Renal Lymphangiectasia

Lorenz T. Ramseyer, MD

1 From Bass Baptist Memorial Hospital, 600 S Monroe,
Enid, OK 73701. Received May 28, 1999; revision requested July 20;
revision received August 26; accepted August 30. Address

correspondence to the author (e-mail: lramseyer@peakonline.com

Index terms: Diagnosis Please • Kidney, CT, 81.12112 • Lymphatic system, abnormalities, 994.829 • Lymphatic system, CT, 994.12912 • Urography, 81.11

History

A 28-year-old obese man had microscopic
hematuria. Physical examination results were
normal. His medical history was unremarkable,
and there was no pertinent family history. Excretory
urography (Fig 1) and computed tomography

(CT) of the abdomen (Fig 2) were
performed after oral and intravenous
administration of contrast material.

Imaging Findings

The excretory urogram demonstrated
bilateral nephromegaly with distortion of
the pelvocaliceal systems (Fig 1). The CT
scan of the abdomen (Fig 2) showed fluid
collections in the perinephric space bilaterally,
surrounding the renal cortex. In addition, there
were peripelvic fluid collections bilaterally,
with distortion of the pelvocaliceal systems.

There were fluid collections in the
retroperitoneum that crossed the midline
at the level of the renal hila, adjacent to the
abdominal aorta and the inferior vena cava.

Discussion

Several differential diagnostic possibilities are to
be considered with bilateral nephromegaly with
pelvocaliceal splaying and distortion. Adult polycystic
kidney disease, lymphoma, nephroblastomatosis,
and other causes of multiple renal masses, such as
von Hippel-Lindau disease and tuberous sclerosis,
are considerations. Adult polycystic kidney disease
has characteristic findings of numerous bilateral renal
cysts, with or without hepatic or pancreatic cysts.

The cysts typically vary in size and are scattered
throughout the parenchyma. Lymphoma and other
malignancies can demonstrate soft-tissue masses
involving the kidneys, pelvocaliceal systems, or
retroperitoneum. The fluid attenuation of the perinephric
collections in the test case, as evidenced by attenuation
measurements of 0–10 HU in Figure 2c, exclude
lymphoma and other soft-tissue masses. Perinephric and
retroperitoneal soft-tissue masses can be seen in

retroperitoneal fibrosis but are again excluded by the
fluid attenuation in the test case. Nephroblastomatosis
is a cause of nephromegaly in children and is characterized
by multiple subcapsular and parenchymal soft-tissue nodules
composed of metanephric blastema (1).

Renal lymphangiectasia is a rare disorder.
Patient symptoms described in the literature (2–4)include
hematuria, flank pain, and abdominal pain. The
condition has been found in children and in adults
(2,4–7). The origin of this disorder is speculative.

There is a familial association in some cases,
which argues for a congenital cause (6). There was
no known family history of renal lymphangiectasia
in the test case. Others argue for an acquired cause,
which suggests that the lymphatic vessels may become
blocked owing to inflammation or other obstruction
and so cause lymphatic ectasia (8). Others have
suggested that these lesions may be a true
neoplasm (9). The nomenclature of this disorder
is confusing and has evolved in recent years. Other
names have included "renal lymphangiomatosis"

(3,6), "renal lymphangioma" (5), "peripelvic
lymphangiectasia" (2), and "renal peripelvic multicystic
lymphangiectasia" (8). "Renal lymphangiectasia" is the
preferred name, given that the disorder is characterized
by ectatic perirenal, peripelvic, and intrarenal
lymphatic vessels (10,11).

Imaging findings of renal lymphangiectasia include
peripelvic cysts and perirenal fluid collections. The
finding of retroperitoneal fluid collections, presumably
dilated lymphatic vessels, is a more variable finding
but has been noted in multiple cases in the literature (2–4,6).

Renal lymphangiectasia has been found associated with
renal venous thrombosis and hypertension (4,6,12).
Ascites and large perinephric fluid collections have
been found and are exacerbated by pregnancy (6).

The natural history of this disorder is not completely
understood. In a neonatal case, partial regression
was reported (7).

The diagnosis of renal lymphangiectasia can be
confirmed with needle aspiration of chylous fluid
from the perinephric fluid collections (4). However,
the ultrasonographic and CT findings are characteristic
for this disease and allow the diagnosis to be made
confidently (2,6). Treatment is not usually necessary.

Complicated cases may be treated with nephrectomy,
percutaneous drainage, or marsupialization (4).

In the case presented here, the diagnosis of renal
lymphangiectasia was based on the characteristic
CT findings. The perirenal, peripelvic, and retroperitoneal
collections had attenuation measurements consistent
with fluid (0–10 HU) rather than with the soft-tissue
attenuation seen with lymphoma or other causes of
bilateral renal soft-tissue masses (Fig 2c). No invasive
procedures to confirm diagnosis were deemed
appropriate for this patient.

Our congratulations to the 26 individuals who
submitted the most likely diagnosis (renal lymphangiectasia)
for Diagnosis Please, Case 34. The names and locations
of the individuals, as submitted, are as follows:

Marc P. Banner, Philadelphia, Pa
Lawrence R. Bigongiari, MD, Hope, Ark
Marc G. de Baets, MD, Lugano, Switzerland
Kemal Demir, MD, Ataköy,stanbul, Turkey
Giovanna Demurtas, MD, Cagliari, Italy
Luis E. Fajre, MD, Tucuman, Argentina
Sandra K. Fernbach, Chicago, Ill
Milton R. Fuentealba, MD, General Roca, Rio Negro, Argentina
Celso Ichihara, Brazil
Kartik Jhaveri, MD, Mumbai, India
Douglas S. Katz, MD, Mineola, NY
Glenn Krinsky, MD, New York, NY
Prof. Dr. Luis Mendez Uriburu, Tucuman, Argentina
Sergio J. Moguillansky, MD, Cipolletti, Rio Negro, Argentina
Adalberto Montanhini Júnior, Brazil
Steven Perlmutter, MD, Mineola, NY
Timothy J. Phalen, MD, Cincinnati, Ohio
Dr. Arturo Ramos-Pablos, Cd. Obregón, Son., México
Luiz Antonio Rossi, São Paulo, Brazil
Anthony J. Scuderi, MD, Johnstown, Pa
Matt Shapiro, MD, Lowell, Mass
Paolo Siotto, MD, Cagliari, Italy
Douglas L. Teich, MD, Brookline, Mass
Christopher Vittore, MD, Rockford, Ill
Keith Wittenberg, MD, Rochester, Minn
Joe Yut, Olathe, Kan

Footnotes

Part 1 of this case appeared 4 months previously
and may contain larger images.

Article

Lymphangiectasia Information

What is Lymphangiectasia?

Lymphangiectasia

Last Updated: November 8, 2005

Synonyms and related keywords: acquired lymphangioma, acquired lymphangiectasia, secondary lymphangioma, lymphangioma circumscriptum

Author: Geover Fernandez, MD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School

Geover Fernandez, MD, is a member of the following medical societies: Alpha Omega Alpha

Editor(s): James Fulton, Jr, MD, PhD, Medical Director, Fulton Skin Institute; David F Butler, MD, Professor, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Rosalie Elenitsas, MD, Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; and Dirk M Elston, MD, Teaching Faculty, Department of Dermatology, Geisinger Medical Center

Introduction

Background:

Lymphangiectases represent superficial lymphatic dilatation caused by a wide range of scarring processes. Lymphangiectasia occurs as a consequence of lymphatic damage by an external cause, leading to obstruction of local lymphatic drainage. Lymphangiectases are also termed acquired lymphangiomas. Acquired lymphangiomas most commonly occur in adults as a late sequela of mastectomy and radiation therapy. Patients usually present with numerous translucent vesicles in a chronic lymphedematous area several years after surgery with or without radiation therapy.

Some authors apply the terms acquired lymphangioma and lymphangioma circumscriptum interchangeably. In both conditions, the typical cutaneous lesions are groups of small translucent vesicles, often compared with frog spawn. Although both share similar clinical and histologic features, the authors believe that they are 2 distinct entities. The term acquired lymphangioma (lymphangiectasia) is used when dilated lymphatic channels arise following damage to previously normal deep lymphatics, whereas lymphangioma circumscriptum is used when lymphatic channel dilation occurs because of congenital malformations of the lymphatic system involving the skin and the subcutaneous tissues.
Pathophysiology:

The pathogenesis of lymphangiectasia is not known; however, the vesicles associated with lymphangiectasia are suggested to represent saccular dilations of local superficial lymphatics. These vesicles develop secondary to increased intralymphatic pressure as a result of buildup of lymph in the superficial vessels caused by damage to previously normal deep lymphatics. This mechanism explains the accompanying lymphedema seen in most patients with lymphangiectasia. The lymphedema usually arises as a result of obstructed lymphatic drainage after mastectomy, radiation therapy, or tumor mass compression.

Frequency:

In the US: The true incidence of this disease is not known. Although lymphangiectasia has been reported in the literature with increased frequency in the past 2 decades, the disease remains rare.

Mortality/Morbidity:

Lymphangiectasia is a nonfatal disease associated with a high tendency for local recurrence after treatment.

Lymphangiectasia may be complicated by chronic copious drainage, pain, and recurrent bouts of cellulitis. In addition, lesions are often cosmetically undesirable.

Acquired lymphangiomas are not believed to have malignant potential, although associated chronic lymphedema places the patient at risk for lymphangiosarcoma (Stewart-Treves syndrome), which is an aggressive tumor with a dismal prognosis.

Race: No racial predominance has been reported.

Sex: No sexual predominance has been reported.

Age: In contrast to lymphangioma circumscriptum, acquired lymphangioma is more common in adults than in children. More generally, the condition occurs in patients between their fifth and seventh decades of life.

Clinical:

History:

Patients typically present with numerous fluid-filled vesicles in a chronic lymphedematous area several years after surgery, more commonly due to a malignancy.

The cutaneous lesions can range from clear, fluid-filled blisters to smooth, flesh-colored nodules, often appearing along an incisional scar.

Coexisting lymphedema is present in most patients with acquired lymphangioma.

Patients can present with localized wetness or copious drainage of clear or milky fluid from ruptured vesicles.

Pain and recurrent cellulitis are complications associated with lymphangiectasia.

Physical:

Clinically, lymphangiectasia consists of several clusters of translucent, thick-walled, fluid-filled vesicles. The vesicles typically measure 2-10 mm in diameter.

The affected area appears to be speckled by numerous translucent vesicles with normal-appearing skin among the lesions.

Some lesions may become pedunculated with a hyperkeratotic verrucous surface mimicking a wart.

Although many patients without chronic lymphedema have been reported, it is a common physical finding in patients with acquired lymphangioma.

Causes: Acquired lymphangiomas can arise from a large number of external factors that cause structural damage to previously normal deep lymphatics.

Lymphangiectases have been reported following radical mastectomy with or without radiation therapy; irradiation alone for various malignancies; metastatic lymph node obstruction; and various scarring processes, such as infections, keloids, scleroderma, and scrofuloderma.

Lymphangiectases have been described in the penis and the scrotum after removal of a sacrococcygeal tumor; they may also arise on the vulva and the inner thigh after surgery for cervical cancer.

Acquired lymphangiomas have been reported in the genital region of elderly patients without evidence of lymphatic obstruction.

Differentials

Dermatitis Herpetiformis Herpes Simplex
Herpes Zoster Lymphangioma Malignant Melanoma Metastatic Carcinoma of the Skin Stewart-Treves Syndrome Warts, Genital Warts, Nongenital

Other Problems to be Considered:

A lymphangiectatic variant of pilomatricoma has been described (del Pozo, 2004).

Other Tests:

Immunohistochemistry

This study is important in differentiating lymphangiomas from hemangiomas in difficult cases.

Factor VIII–related antigen testing demonstrates positive results in the endothelial cells of hemangiomas but negative or weakly positive results in the endothelium of lymphangiomas.

Ulex europaeus I testing results are positive in the endothelial cells of lymphangiectases and hemangiomas.

Immunoperoxide staining with QBEnd10 (anti-CD34) antibodies shows positive reactivity results only in endothelial cells lining the blood vessels of the dermis.

Procedures:

Skin biopsy: The diagnosis of lymphangiectasia is primarily based on clinical history and conventional light microscopy findings.

Histologic Findings: On microscopic examination, lymphangiectases are characterized by large, dilated lymph vessels lined by a single layer of endothelial cells. They are usually found in the papillary dermis and the reticular dermis. Involvement in the deeper dermis is rare. The overlying epidermis is usually associated with acanthosis and hyperkeratosis but is less marked than in angiokeratomas. At times, acquired lymphangiectases grow above the level of adjacent skin.

Lymphangiectases lack the subcutaneous muscle-coated cisternae characteristic of lymphangioma circumscriptum. Although no specific histologic criteria exist with which to differentiate lymphangioma circumscriptum from lymphangiectasia, some believe that the absence of subcutaneous cisternae in lymphangiectases is a true histologic difference and that this finding reflects the difference in the pathogenesis of both disorders.

Treatments

Medical Care:

No medical care has been proven to be effective for acquired lymphangiomas because the responsible lymphatic vessels must be either excised or sealed to prevent recurrence.

Treatment for lymphangiectases can be difficult, but it is important because of the risk that ruptured vesicles may provide a portal of entry for infection and subsequent cellulitis. To prevent superinfection of ruptured vesicles, cleansing the affected area daily with topical antibacterial agents and applying mupirocin ointment or silver sulfadiazine cream are advisable.

Surgical Care:

Many surgical treatment modalities have been advocated in the care of lymphangiectases; these modalities include electrodesiccation, laser therapy, sclerotherapy, cryotherapy, and surgical excision.

Daily compression through bandaging or hosiery, in accessible areas, has yielded acceptable results.

Followup

Further Inpatient Care:

At times, severe recurrent cellulitis may warrant hospitalization in patients with lymphangiectasia, especially in patients who are immunocompromised. Intravenous antibiotic is required in patients with severe cellulitis.

Further Outpatient Care:

Follow-up care is essential for early treatment of recurrences.

Lymphangiosarcoma (Stewart-Treves syndrome) may occur in chronic edematous limbs, and early detection is critical.

Complications:

Lymphangiectases are often complicated by pain, copious fluid drainage, and recurrent attacks of cellulitis.

Prognosis:

Lymphangiectasia has a good prognosis because most conditions respond well to treatment modalities.

Patient Education:

Patients should be reassured.

Lymphangiectases pose no potential for malignant transformation.

For excellent patient education resources, visit eMedicine's Women's Health Center and Cancer and Tumors Center. Also, see eMedicine's patient education articles Mastectomy and Breast Cancer

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