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981
Case Report
H
I
Pulmonary Alveolar Microlithiasis: High-Resolution CT M. A. Korn,1
We report
H. Schurawitzki,1
W. Kbepetko,2
the high-resolution
CT findings
and
in a case of
[1 ]. The high rate of occurrence
within families (>50%) suggests an autosomal-recessive hereditary factor [1]. The radiographic appearance is pathognomonic [2], but open lung biopsy is sometimes required. Plain chest radiographs show a white lung or sandstorm lung consisting of
fine sandlike
microcalcifications,
diffusely
scattered
through-
out both lungs with higher density at the lung bases [1-5]. The heart borders and the diaphragm are usually obliterated.
Typical findings include small bublae in the apexes and a black pleural line, described by Felson [2] as a zone of increased translucence between the lung parenchyma and the ribs. However, high-resolution CT scans show that this black pleural
line is due to thin-walled
subpbeural
Cysts.
studies revealed the typical features total
lung
exercise, Chest
A 26-year-old woman was referred to our hospital because of recurrent pneurnothoraces, which had been treated by tube thoraEleven
diagnosis
of pulmonary
tion she
had
no
years
earlier, alveolar
shortness
an open microlithiasis.
of breath
lung
biopsy At the
at rest.
confirmed time
a
of evalua-
Pulmonary
function
and
Arterial
PA02
May
1992 036i-803X/92/i
585-0981
C American
Roentgen
capacity
was
of restrictive but
normal
thiasis and a small pneumothorax
defect with reduced
without
at rest
evidence
and
features
of airflow
deteriorated
during
of alveolar
rnicroli-
on the right side. Heart borders
and diaphragm were obscured, small apical bullae were visible, and a small zone of radiolucency was present between the lung parenchyma and the ribs (Fig. 1A). High-resolution CT was performed on a Somatom DR3 (Siemens, Erlangen, Germany) scanner. All images were obtained at maximal inspiration using 2-mm collimation, 1 25 kV, 90 mA, and a 5-sec acquisition time. Scans were taken at 20-mm intervals in the supine position; at the lower levels, additional scans were obtained in the
prone position. Images were reconstructed by using bone algorithm and a 512 x 512 matrix. All scans were photographed with window/ level settings of 2000/-200 and 900/-650. Apical bullae were visible on both sides, but they were larger on the right. In addition, small thin-walled subpleural cysts, ranging from 5 to 1 0 mm
in diameter,
were
seen.
The
cysts
were
along the rnediastinal and panetal from the apex to the costodiaphragrnal
itself was
thickened
and irregularly
ribbonlike
and
pleura on both sides sinus (Fig. 1 B). The
delineated,
containing
punctate calcifications. Additionally, a small residual pneumothorax was visible in the right apex 3 days after the chest tube was removed. Lung parenchyma showed high attenuation, increasing from the
apex to the lung base. In the upper and middle lung zones, the high attenuation
was
renchyma; involvement,
in the lower lung higher attenuation
distributed
Received September 4, 1991 ; accepted after revision October 23, 1991. This work was supported in part by the Ludwig Boitzmann Institute for Radiological Tumor Diagnosis. 1 Department of Radiology, University of Vienna, AKH, Wbhringer GUrtel 18-20, A-i 090 Wien, Austria. 2 Second Surgical Department, University of Vienna, AKH, Spitalgasse 23, A-i 090 Wien, Austria. 3 Department of Internal Medicine IV, Division of Pulmonary Medicine, University of Vienna, AKH, Wbhringer AJR 158:981-982,
vital
indicating diffusion impairment. radiographs showed the typical
arranged extending
Report
costomy.
capacity
obstruction.
pleura Case
on
0. C. Burghuber3
pulmonary alveolar microlithiasis, a disease characterized by widespread, sandlike intraalveolar calcifications. A rare lung disease, it has been reported fewer than 1 60 times in the literature. Although the cause is unknown, one possibility is
an inborn error of metabolism
Findings
Ray Society
homogeneously
GUrtel
throughout
the
lung
pa-
zones, however, instead of diffuse was seen in the dorsal zones (Fig.
18-20,
A-i 090 Wien,
Austria.
KORN
Downloaded from www.ajronline.org by 125.77.209.163 on 11/02/15 from IP address 125.77.209.163. Copyright ARRS. For personal use only; all rights reserved
982
Fig. 1.-26-year-old
woman with biopsy-proved
pulmonary
alveolar
ET AL.
AJA:i58,
microlithiasis.
A, Chest radiograph shows sandstorm lung with black pleural line (arrows) between the ribs and lung parenchyma. B, High-resolution CT scan (middle lung zone) shows subpleural cysts (arrows) along the parietal and mediastinal pleura. parenchyma is due to alveolar calcifications. Soft-tissue emphysema followed right-sided pneumothorax. C, High-resolution CT scan of lower lung zone shows greater involvement than in middle lung zone (B), especially posteriad arrows show subpleural cysts.
1 C) and the higher attenuation persisted in the prone position, causing us to rule out hypostasis as a reason for the increased density. In addition to the high attenuation of the lung parenchyma, small (
981
Case Report
H
I
Pulmonary Alveolar Microlithiasis: High-Resolution CT M. A. Korn,1
We report
H. Schurawitzki,1
W. Kbepetko,2
the high-resolution
CT findings
and
in a case of
[1 ]. The high rate of occurrence
within families (>50%) suggests an autosomal-recessive hereditary factor [1]. The radiographic appearance is pathognomonic [2], but open lung biopsy is sometimes required. Plain chest radiographs show a white lung or sandstorm lung consisting of
fine sandlike
microcalcifications,
diffusely
scattered
through-
out both lungs with higher density at the lung bases [1-5]. The heart borders and the diaphragm are usually obliterated.
Typical findings include small bublae in the apexes and a black pleural line, described by Felson [2] as a zone of increased translucence between the lung parenchyma and the ribs. However, high-resolution CT scans show that this black pleural
line is due to thin-walled
subpbeural
Cysts.
studies revealed the typical features total
lung
exercise, Chest
A 26-year-old woman was referred to our hospital because of recurrent pneurnothoraces, which had been treated by tube thoraEleven
diagnosis
of pulmonary
tion she
had
no
years
earlier, alveolar
shortness
an open microlithiasis.
of breath
lung
biopsy At the
at rest.
confirmed time
a
of evalua-
Pulmonary
function
and
Arterial
PA02
May
1992 036i-803X/92/i
585-0981
C American
Roentgen
capacity
was
of restrictive but
normal
thiasis and a small pneumothorax
defect with reduced
without
at rest
evidence
and
features
of airflow
deteriorated
during
of alveolar
rnicroli-
on the right side. Heart borders
and diaphragm were obscured, small apical bullae were visible, and a small zone of radiolucency was present between the lung parenchyma and the ribs (Fig. 1A). High-resolution CT was performed on a Somatom DR3 (Siemens, Erlangen, Germany) scanner. All images were obtained at maximal inspiration using 2-mm collimation, 1 25 kV, 90 mA, and a 5-sec acquisition time. Scans were taken at 20-mm intervals in the supine position; at the lower levels, additional scans were obtained in the
prone position. Images were reconstructed by using bone algorithm and a 512 x 512 matrix. All scans were photographed with window/ level settings of 2000/-200 and 900/-650. Apical bullae were visible on both sides, but they were larger on the right. In addition, small thin-walled subpleural cysts, ranging from 5 to 1 0 mm
in diameter,
were
seen.
The
cysts
were
along the rnediastinal and panetal from the apex to the costodiaphragrnal
itself was
thickened
and irregularly
ribbonlike
and
pleura on both sides sinus (Fig. 1 B). The
delineated,
containing
punctate calcifications. Additionally, a small residual pneumothorax was visible in the right apex 3 days after the chest tube was removed. Lung parenchyma showed high attenuation, increasing from the
apex to the lung base. In the upper and middle lung zones, the high attenuation
was
renchyma; involvement,
in the lower lung higher attenuation
distributed
Received September 4, 1991 ; accepted after revision October 23, 1991. This work was supported in part by the Ludwig Boitzmann Institute for Radiological Tumor Diagnosis. 1 Department of Radiology, University of Vienna, AKH, Wbhringer GUrtel 18-20, A-i 090 Wien, Austria. 2 Second Surgical Department, University of Vienna, AKH, Spitalgasse 23, A-i 090 Wien, Austria. 3 Department of Internal Medicine IV, Division of Pulmonary Medicine, University of Vienna, AKH, Wbhringer AJR 158:981-982,
vital
indicating diffusion impairment. radiographs showed the typical
arranged extending
Report
costomy.
capacity
obstruction.
pleura Case
on
0. C. Burghuber3
pulmonary alveolar microlithiasis, a disease characterized by widespread, sandlike intraalveolar calcifications. A rare lung disease, it has been reported fewer than 1 60 times in the literature. Although the cause is unknown, one possibility is
an inborn error of metabolism
Findings
Ray Society
homogeneously
GUrtel
throughout
the
lung
pa-
zones, however, instead of diffuse was seen in the dorsal zones (Fig.
18-20,
A-i 090 Wien,
Austria.
KORN
Downloaded from www.ajronline.org by 125.77.209.163 on 11/02/15 from IP address 125.77.209.163. Copyright ARRS. For personal use only; all rights reserved
982
Fig. 1.-26-year-old
woman with biopsy-proved
pulmonary
alveolar
ET AL.
AJA:i58,
microlithiasis.
A, Chest radiograph shows sandstorm lung with black pleural line (arrows) between the ribs and lung parenchyma. B, High-resolution CT scan (middle lung zone) shows subpleural cysts (arrows) along the parietal and mediastinal pleura. parenchyma is due to alveolar calcifications. Soft-tissue emphysema followed right-sided pneumothorax. C, High-resolution CT scan of lower lung zone shows greater involvement than in middle lung zone (B), especially posteriad arrows show subpleural cysts.
1 C) and the higher attenuation persisted in the prone position, causing us to rule out hypostasis as a reason for the increased density. In addition to the high attenuation of the lung parenchyma, small (
