PULMONARY LYMPHANGIECTASIA
-----------------------------------
CONGENITAL 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 period
risk factors:
Noonan's Syndrome
Turner's Syndrome
TYPES
(after Noonon (1970))*:
1. Group I
dilated pulmonary lymphatics are part of a generalized form of lymphangiectasis:
thoracic and extrathoracic (intestinal)
lymphangiectasia
associated with
lymphedema
hemihypertrophy
diffuse angiomatosis in which bone is the most common site of
involvement
less severe form and has a much better prognosis than the other two
forms
2. Group II
due to a cardiac
lesion
dilated 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
life
very poor prognosis
3. Group III
due to a defect in the primary development of the lung
Lawrence (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 predominance
may be associated with congenital
malformations including cardiac lesions
clinical picture characterized by over expanded lungs with alveolar
hypoventilation and periodic wheezing and a relapsing course
dilated 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 Period
presents with respiratory distress and cyanosis at birth
(usually term) with rapid demise
can also present as a stillbirth
2. Post Neonatal Period
presents with respiratory difficulties
progressive relapsing course
associated with chylopericardium,
chylothorax,
chronic cough,
congestive heart
failure
3. Associated Abnormalities
congenital abnormalities in 50% of cases:
1. Cardiac
TAPVR, mitral valve atresia, hypoplastic left heart, aortic
coarctation, VSD,
PDA, ASD
2. Others
cystic hygromas, diffuse
angiomatosis
(bones),
pneumothorax
INVESTIGATIONS:
1. Diagnostic
1.
Lung
Biopsy
cystic 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
1. Chest
X-Ray
hyperexpansion
diffuse reticular pattern
MANAGEMENT:
supportive
..............................................
Pulmonary Lymphangiectasia, congenital
Introduction
Lymphatic fluid in the lung is derived from normal leakage of fluid out
of the
blood capillaries in the lung and the intestines. In PL the channels
(lymphatics)
that carry lymph fluid are not properly connected and become dilated
with fluid
(lymphangiectasia). This fluid makes the lungs less elastic, which this
causes
breathing difficulties Mostly PL presents with severe respiratory
distress in
the newborn period, although later onset forms of PL have been
described in a
number of cases. The mortality of neonatal PL was previously reported
to be
nearly 100%. However, most of these reports were published nearly 30
years ago
and neonatal care has advanced significantly since that time. Several
recent
case reports and case studies indicate that infants are now surviving
the severe
neonatal form of PL and the outcome is good for infants who present
after the
neonatal period. This report describes the latest concepts on what may
cause
this condition, its clinical presentation, how to make a diagnosis of
PL, what
successful strategies have been used to treat PL, and the outcome of PL.
Incidence
The overall incidence of pulmonary lymphangiectasia is unknown. Autopsy
studies
suggest that approximately 1% of infants who are stillborn or die in
the
neonatal period have pulmonary lymphangiectasia. Most case series have
reported
that more males than females are affected. In most cases, there is no
familial
pattern, and in some PL may occur together with a number of genetic
syndromes
including Noonan, Down, Turner, and Fryns, yellow nail syndrome and
congenital
icthyosis. Most of these syndromes are associated with generalized
abnormalities
of the lymphatic system.
Classification
and Causes
Pulmonary lymphangiectasia is broadly divided into 2 categories:
primary
pulmonary lymphangiectasia where there is an intrinsic abnormality of
the lymph
channels, and secondary pulmonary lymphangiectasia, where the
lymph channels are
normal, but there is some downstream blockage to lymph flow.
Primary
PL can be subdivided into
generalized, pulmonary, and syndromic forms. The generalized form
involves lymph
vessel changes in many organ systems including the subcutaneous tissue,
intestine, and lung. The major symptoms include generalized
edema and
protein
loss from intestinal
lymph
vessels. Hemihypertrophy (enlargement of one limb) is
common. Lung involvement in typically not severe, and survival in
patients with
pulmonary involvement is reported to be better than with the other
forms of
pulmonary lymphangiectasia. The primary pulmonary form of
lymphangiectasia
includes those patients with disease confined to the lung. This form is
the
classic congenital pulmonary lymphangiectasia previously described as
having a
very poor prognosis. In the syndromic form, PL is associated with
multiple other
unrelated congenital anomalies including the genetic syndromes
mentioned
earlier.
The cause of primary PL is not known. There is some evidence that the
lung
lymphatics are not properly “programmed” as they are developing in the
fetus, so that the
lymph systems in different parts of the lungs are never
connected to the large channels that drain the fluid away. In some
cases, there
is evidence that the
lymphatics may be normal at birth, and that subsequent
abnormalities may result from a lung infection.
Secondary PL includes those forms that are due to downstream blockage of the lymphatics (e.g. surgery, trauma, lung disease) or congestion of the pulmonary veins, (usually from abnormal pulmonary veins on an abnormal heart).
Clinical
Presentations
The clinical symptoms of pulmonary lymphangiectasia can present
prenatally, at
birth (neonatal) or after an asymptomatic period (post-neonatal). In
the
prenatal period, pulmonary lymphangiectasia can be a cause of swelling
of the
fetus (non-immune hydrops fetalis) and excess amniotic fluid
(polyhydramnios),
and fluid in the chest cavity (pleural effusions). Many infants are
stillborn.
The neonatal presentation manifests as respiratory distress at, or
within a few
hours of birth. Mechanical ventilation is often required. The
post-neonatal
presentation can occur after weeks or months of mild or absent
respiratory
symptoms. A few cases have presented for the first time in childhood or
adolescence. Typical presenting symptoms include rapid breathing, and
recurrent
cough or wheeze. Examination of the patient usually reveals the
presence of fine
crackles throughout the lungs. Although pleural effusions are reported
in up to
15% of patients.
Pleural effusions typically contain lymph (cloudy) fluid, but
may be clear, particularly if the newborn infant has not yet been fed.
Investigations
Diagnosis of pulmonary lymphangiectasia can be difficult, as many of
the
respiratory symptoms and radiological (X-rays, CT scan etc) findings
are
non-specific. Chest X-rays are usually abnormal, reflecting fluid in
the chest
cavity or lung tissue in infants, and overexpansion of the lungs in
older
infants and children. Chest
computerized tomography (CT) scans of patients with
pulmonary lymphangiectasia show patchy areas of fluid and over
inflation of the
lungs.
Magnetic resonance imaging
(MRI) may be used in the future for making a
diagnosis of PL, but is not yet of proven value.
In many patients an open lung biopsy is helpful to distinguish pulmonary lymphangiectasia from other forms of lung disease. Care must be taken with the pathologic evaluation of the biopsy as the changes of pulmonary lymphangiectasia can be mistaken for interstitial emphysema or overlooked. Although dilated pleural lymphatics present a theoretical risk of chylothorax at the time of lung biopsy (leakage of lymph fluid from the incision site), this complication has not been reported .
In experienced hands, the diagnosis of PL can, in many cases, be made through a combination clinical and radiological evaluation, but a lung biopsy may be the only way to make the diagnosis of PL, particularly if there is concern that there may be another specific diagnosis (e.g. another type of interstitial lung disease).
Lung function studies show varying patterns of obstructive and restrictive disease with no deterioration over time. In infancy, patients present with recurrent with bronchitis, often caused by easily treatable pathogens including Streptococcus pneumonia and Moraxella cattharalis.
Clinical
Course
While there are undoubtedly infants whose PL is so severe that they
cannot be
saved, it is likely that many of the infants reported to have died
previously
with uncomplicated PL would have survived with current practices of
neonatal
intensive care. The oldest reported survivors in recent case series are
12 to 13
years old and are predicted to live into adulthood. Most patients who
survive
the neonatal and early infancy period continue to have respiratory
problems
(recurrent cough, wheeze and pneumonia) in the first several years of
life with
increased rates of hospitalization. A few patients require home
supplemental
oxygen for a period of time. Overall, respiratory condition and X-ray
abnormalities improve during infancy and childhood, with many patients
having
minimal symptoms by age six. Of note, no deaths have occurred in the
recent
reported cohorts of patients who survived infancy.
Many patients with pulmonary lymphangiectasia who survive beyond infancy have other medical problems common to patients with chronic lung disease. Gastroesophageal reflux is frequent, poor growth is also not uncommon in the first few years of life, although most patients resume normal growth patterns by age 3 years. Both reflux and poor growth may be secondary to the increased work of breathing typical of patients with pulmonary lymphangiectasia rather than any specific abnormality.
Treatment
There is no specific treatment for PL. The main strategy is to support
the
patient by treating respiratory failure in the neonatal period and lung
infections later on in the expectation that he condition will improve
over time.
Mechanical ventilation is often required in the neonatal form of PL,
and
specific strategies (e.g. pressure support with high mean airway
pressures) have
been successfully used as a strategy for neonatal respiratory failure.
Some
infants develop rapidly expanding pleural effusions that require
placement of
chest tube that may drain large volumes of fluid over days or weeks,
but in most
cases, this problem resolves over time. Other specific therapies
including
nutritional therapy with
medium chain triglycerides and total parenteral
nutrition have been successfully employed for treatment of chylothorax.
Conclusion:
Pulmonary lymphangiectasia can occur as a result of an intrinsic
developmental
anomaly, or from postnatal obstruction of pulmonary lymphatic or venous
drainage. The timing and severity of the initial presentation is
variable.
Advances in neonatal care have significantly improved the outcome in
even the
most severe cases. PL can be diagnosed with a combination of clinical
assessment, radiological studies and lung biopsy. Treatment is
essentially
supportive. The long-term outcome of PL is uncertain, but current
evidence
suggests that for the majority of patients with either neonatal or
post-neonatal
presentations, there is gradual improvement of clinical status over
time,
particularly if there are no significant co-existing abnormalities.
British Paediatric Orphan Lung Diseases (BPOLD)
..............................................
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
http://www.auntminnie.com/ScottWilliamsMD2/Chest/Congenital/Lymphangiec/Lymphangiectasia.htm
..............................................
Pulmonary Lymphangiectasia
Congenital
pulmonary lymphangiectasia (PL) is a rare developmental disorder
involving the
lung and characterized by pulmonary subpleural, interlobar,
perivascular, and
peribronchial lymphatic dilatation. The prevalence is unknown. PL
presents at
birth with severe respiratory distress, tachypnea and cyanosis, with a
very high
mortality rate at or within a few hours of birth. Most reported cases
are
sporadic and the etiology is not completely understood. It has been
suggested
that PL lymphatic channels of the fetal lung do not undergo the normal
regression process at 20 weeks of gestation. Secondary PL may be caused
by a
cardiac lesion. The diagnostic approach includes obtaining a complete
family and
obstetric history, conventional radiologic studies, ultrasound and
magnetic
resonance studies,
lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy,
and pleural effusion examination. During the prenatal period, all
causes leading to
hydrops fetalis should be considered in the diagnosis of PL.
Fetal ultrasound evaluation plays a key role in the antenatal diagnosis
of PL.
At birth, mechanical ventilation and pleural drainage are nearly always
necessary to obtain a favorable outcome of respiratory distress. Home
supplemental oxygen therapy and symptomatic treatment of recurrent
cough and
wheeze are often necessary during childhood, sometimes associated with
prolonged
pleural drainage. Recent advances in intensive neonatal care have
changed the
previously nearly fatal outcome of PL at birth. Patients affected by PL
who
survive infancy, present medical problems which are characteristic of
chronic
lung disease. *Authors: Drs C. Bellini, F. Boccardo, C. Campisi, E.
Bonioli
(October 2006)*. Reproduced from Congenital
pulmonary
lymphangiectasia. Orphanet J Rare Dis. 2006;1(1):43.
ICD10
Synonym
- Pulmonary
lymphangiomatosis
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=2414
Full length article
http://www.ojrd.com/content/1/1/43
...............................................
Division of Pulmonary and Critical Care Medicine and the Department of Pathology, Stanford University School of Medicine, Stanford, California; and the Department of Pathology, Mayo Clinic, Scottsdale, Arizona
http://ajrccm.atsjournals.org/cgi/content/full/161/3/1037
..............................................
SN
Javett, I Webster and JL Braudo 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 hitherto regarded as a
pathological
curiosity.
http://pediatrics.aappublications.org/cgi/content/abstract/31/3/416 .............................................. 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, 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. http://erj.ersjournals.com/cgi/content/abstract/24/3/413 .............................................. A female
infant born at 28 weeks' gestation was
found to have mild hydrops foetalis. Initial echocardiography showed a
structurally normal heart. During the first week of life, episodic
atrial
tachycardia with 1:1 or 2:1 conduction was seen, requiring therapy with
digoxin.
The infant remained ventilator dependent, with a persistent, chylous
pleural
effusion which contained a preponderance of lymphocytes. Congenital
pulmonary
lymphangiectasia (CPL) was confirmed histologically. Worsening episodes
of
atrial tachycardia, including episodes of atrial fibrillation, were
further
investigated and a repeat echocardiogram revealed thickening of the
entire right
atrial wall. The cardiac findings of a thickened right atrial wall with
the
histological signs of myocarditis were thought to be the cause of
paroxysms of
atrial fibrillation, an extremely rare arrhythmia in the neonatal
period. To the
authors' knowledge there have been no previous reports of CPL in
association
with the cardiac abnormalities described herein. ======================= External
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904 Medical Centre Jeppe Street, Johannesburg, South Africa
Primary
pulmonary
lymphangiectasia in infancy and childhood
Accepted May 20, 2004
Atrial fibrillation with
neonatal pulmonary lymphangiectasia
TAPVR, ASD,
Pulmonary Lymphangiectasia
http://www.tchin.org/portraits/michael-3.htm
Ackowledgement: Congenital Heart Information Network
..............................................
National
Congenital Pulmonary Lymphangiectasia Foundation
National network. Founded 1995.Provides support and information for
families and
friends of persons with congenital pulmonary lymphangiectasia. Funds
research
projects. Information and referrals, phone support, pen pals,
literature.
WRITE:
Nat'l CPL Fdn.
1202 S. Columbia Ave.
Somerset, PA 15501-9387
CALL: 814-445-3686
E-mail: vbrf@twd.net
VERIFIED: 10/17/2002
http://healthlink.uhseast.com/Library/HealthGuide/SelfHelp/topic.asp?hwid=shc29cpl
Paediapaedia: Neonatal Chest Diseases
Michael P. D'Alessandro, M.D.
http://www.vh.org/pediatric/provider/radiology/PAP/NeonatalChestDiseases/PulmonLymphang.html
..............................................
Congenital Pulmonary
Lymphangiectasia
New England Journal of Medicine
http://content.nejm.org/cgi/reprint/349/22/e21.pdf
..............................................
http://www.findarticles.com/cf_dls/m0950/6_109/87564139/p1/article.jhtml?term=
www.auntminnie.com/ScottWilliamsMD2/Chest/Congenital/
Lymphangiec/Lymphangiectasia.htm
..............................................
CONGENITAL
PULMONARY LYMPHANGIECTASIA ASSOCIATED WITH CONGENITAL HEART
DISEASE.
Chest, Oct, 2000, by JT Tuladhar, SA Storgion, MC Bugnitz, G Stidham, O
Lasater
http://www.findarticles.com/cf_0/m0984/4_118/71127145/p1/article.jhtml
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Subscribe:
AdvocatesforLymphedema-subscribe@yahoogroups.com
The time has come for families, parents, caregivers to have a support
group of
their own. Support group for parents, families and caregivers of
chilren with
lymphedema. Sharing information on coping, diagnosis, treatment and
prognosis.
Sponsored by Lymphedema People.
http://health.groups.yahoo.com/group/childrenwithlymphedema/
Subscribe: childrenwithlymphedema-subscribe@yahoogroups.com
......................
No matter how you spell it, this is another very little understood and
totally
frustrating conditions out there. This will be a support group for
those
suffering with lipedema/lipodema. A place for information, sharing
experiences,
exploring treatment options and coping.
Come join, be a part of the family!
http://health.groups.yahoo.com/group/lipedema_lipodema_lipoedema/?yguid=209645515
Subscribe: lipedema_lipodema_lipoedema-subscribe@yahoogroups.com
http://health.groups.yahoo.com/group/menwithlymphedema/
Subscribe: menwithlymphedema-subscribe@yahoogroups.com
Support group for parents, patients, children who suffer from all forms
of
lymphangiectasia. This condition is caused by dilation of the
lymphatics. It can
affect the intestinal tract, lungs and other critical body areas.
http://health.groups.yahoo.com/group/allaboutlymphangiectasia/
Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com
While we have a number of support groups for lymphedema... there is
nothing out
there for other lymphatic disorders. Because we have one of the most
comprehensive information sites on all lymphatic disorders, I thought
perhaps,
it is time that one be offered.
DISCRIPTION
Information and support for rare and unusual disorders affecting the
lymph
system. Includes lymphangiomas, lymphatic malformations,
telangiectasia,
hennekam's syndrome, distichiasis, Figueroa
syndrome, ptosis syndrome, plus many more. Extensive database of
information
available through sister site Lymphedema People.
http://health.groups.yahoo.com/group/lymphaticdisorders/
Subscribe: lymphaticdisorders-subscribe@yahoogroups.com
For our Google fans, we have just created this online support group in
Google
Groups:
Homepage: http://groups-beta.google.com/group/All-About-Lymphedema
Group email: All-About-Lymphedema@googlegroups.com
http://groups.aol.com/lymphedemafriend
If you an AOL fan and looking for a
support group in AOL
Groups, come and join us there.