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(1)

Report

of

a Case

with

Unilateral

Pulmonary

Arterial

and

Venous

Stenosis

and

Atriovenous

Occlusion

By Israel Diamond, M.D.

Departments of Pathology of the Children’s Hospital and time University of Louisville Sc/moo! of Medicine

(Accepted February 28, 1958; submitted December 26, 1957.)

ADDRESS: 226 East Chestnut Street, Louisville, Kentucky.

PEDIATRICS, August 1958

THE

HAMMAN-RICH

SYNDROME

IN CHILDHOOD

279

I

N 19:35 Himiimiiimi and Rich’ described

four cases of bilateral (hiffuse interstitial

fibrosis of the lungs, of unknown etiology.

Simice that time a numuber of case reports

have appeare(1,2 7 the first appearing 10

years hater. In 1952, Rubin, Kahn and

Pecker5 reported a case and summarized the literature. They listed 14 other patients

ranging in age between 21 and 68 years,

and pointed out that the duration of life

from the onset of symptoms to death ranged

from 31 days to 3% years. In 1953, Peabody,

Buechner and Anderson9 reported three

cases and reviewed the literature, making a

total of 21 cases including the three

re-ported by them. They summarized the

clini-cal features of the Hamman-Rich syndrome

as follows: The svmptomatology is marked

by cough, dyspnea and cyanosis associated

with minimal physical findings. There is a

progressive, essentially afebrile course

lead-imig to progressive dyspnea and eventual

asphyxial death.

Roentgenograms of the chest show

paren-chymal and hilar densities which eventually

develop into bilateral diffuse involvement.

The pathologic fimidings are characterized

by interstitial pulmonary fibrosis with

ab-sence of signs of acute inflammatory

re-sponse. The pulmonary arterioles not

infre-quently show subintimal proliferation. Half of the patients develop cor pulmonale.

Ra-diologically the syndrome cannot be

differ-entiate(l froln other diseases producing

dif-fuse reticular amid nodular shadows. The

diagmiosis is established by exclusion and

by hung biopsy.

Cases have 1)eefl reported in identical amii

nonidentical twins and in members of the

same family.”7 Until 1956 when Bradley’’

reported the condition in a 9-year-old white

male child, the youngest patient had been

21 years of age. In 1957, a subsequent case in a child was reported by Baar and Braid.”

This concerned a 3-year-old white male

whose disease began at 7 months of age

with whooping cough. The cough persisted

and there was progressive (levelopment of

cyanosis and infiltration of the lung

radio-logically, more marked in the right than in

the left lung. This child died with cor put1-monale. Necropsy revealed the

characteris-tic picture of bilateral diffuse interstitial pulmonary fibrosis. Feinerman and Harris’2

recently reported a case in a 7-month-old girl with necropsy and an 8-year-old boy

with findings in lung biopsy consistent with

the Hamman-Rich syndrome. The aumthors speculate that these cases are a variant of the syndrome with lesser degree of involve-ment.

The etiology of the syndrome is

com-pletely undetermined. It has been suggested

that bacterial, viral or chemical agents may

be factors. Callahan1 suggests that chronic pulmonary lymphedema may also

contrib-ute to the picture. The term “pulmonary

keloid”T has been used to suggest that

cer-tain individuals are more prone to pul-monary scarring.

This report deals with a 4-year-old Negro

male whose clinical and pathologic features

(2)

280 HAMMAN-RICH SYNDROME

At the time of the first admission to the

Fu;. I(Lcft). Roentgenogramii of chest at 15 months of age.

FIG. 2 (Right). Roentgenogranl of chest at years of age.

is stenosis of the right puhmiiomitry artery and

comiiphete occlusion of the right pulmiionary

vein coniphicating the picture.

History

CASE REPORT

Time 1)dtielit, a Negro male, as the product

of a 9-,llonth, UnCOIflj)hicated pregmiancv and

uneventful (lehiVerV. The birth weight was

3,178 gin and the mieonatal course was

umievent-fiil. The family history was noncontributorv.

The illmless began at 4 months of age with

cough. fever and failure to gain weight. He

VtS seen re1)eate(llV iii a pediatric clinic diurimig

the subsequent ear, with little change in svmp-tolliS.

At 1 year of age the weight was 7.4 kg amid

the length 7:3.6 cm. The chest was

hvperreso-namit andi filled with coarse rales and rhonchi.

A roentgenogram showed moderate mediastimial

shift to the right, extensive umiiform

infiltra-tion of

tue

right lumig and emphysema of the

left lumig. There were questionable infiltrates

in the left upper lolw. Slight clubbing of the

fingers was noted and a history of sleeping in

knee-chest positiomi was elicited. The child

could crawl, hut could not sit up unassisted.

Food! intake was poor amid stools were bulky.

Physical Findings

hiosji;tal it 15 months of age the eight was

still 7.4 kg. The systolic blood pressure was

100 mm Hg, temperature 38#{176}Cand head

cir-cumference 44.5 cm. Tile anterior fontanelle

vas closed, four teeth were present amid! he

Vi15 able to say only two words. He poorly

developed amid poorl nourished. A slight

vesi-cular skirt rash was found on

tue

buttocks and

thighs. The chest signs were unchanged.

Neuro-logic examination was negative.

Laboratory Findings

Roentgemiogramn of the chest (Fig. 1) showed

ami increase in the reticular infiltration of the

right lumig. The emphysema of the left lung

persistedi with expansion of the left heniithorax.

There were coarse infiltrates in tile left lung.

Nasopharmigeal cumlture grew nomlilemnolytic

Staphylococcus aureus. The heukocvte count

was 19,600/mm3, 67% neutrophils, 29%

lymph-ocytes, 1% eosinophils, 1% monocvtes. The

con-centration of hemoglobin was 9.6 gm/100 ml.

Trvptic activity of the stool was 4 lilus; trvptic

activity of duodenal drainage was 5% of

nor-mal. V.D.R.L. was negative.

Course

The child was treated with antibiotics, .oumghi

syrup, nose drops and pancreatic granules and

was transfused with 120 ml of whole blood.

He was discharged after 9 days. The mother

(3)

daily admimiistration of Mol-iron#{174}, pancreatic

granules, Gantrisin#{174} and cehiac diet.

Later History

During the next 13 years the patient was

repeatedly seen in clinic and admitted to the

hospital three times. The symptoms were

es-sen tinIly unchanged and the roentgenographic

findings iii the chest became more extensive.

Costophremiic septal lines were noted on the

right 5i(le. Trvptic activity of duodenal

drain-age was I 00% of normal. A single slight bout

of hemoptvsis occurre(l. At 2 years of age he

cOtltIacte(1 intmnips and! at 2 ears, chicken

pox. At (hifferemit times nasopharyngeal cultures

yielded! various organisms-Staphylococcus

mu-reus, Bacillus pvocyaneus. amid Hemophilus

in-fluenzae.

At 3’#{176},‘ears of age he contracted

polio-m’elitis and was admitted to the hospital with

a 2-day history of abdominal pain, fever, poor

appetite and inal)ihty to walk. He weighed 11

kg and his temperature was 31#{176}C.There were

crepitant rales in the right lower lobe. The

liver was palpable 2 cm below the right costal

margin in the mid-clavicular line. There were

spontaneous movements in the upper

extremi-ties. The lower extremities were markedly weak

and he could not support his weight. The neck

and back were stiff. Kernig’s sign was

nega-tive. There was absence of the right knee jerk

and both ankle jerks. Only slight movement of

the lower extremities was elicited by pain.

Later Laboratory Findings

Cerebrospinal fluid contained 11

polymor-phonuclears/mm, 31 lymphocytes/mm3.

Con-centrations of sugar, total protein and chloride

were 56 mg/100 ml, 24 mg/100 ml and 126

mEq/l, respectively. Cultures of cerebrospinal

fluid and blood yielded no growth. The

con-centration of hemoglobin was 8.8 gm/100 ml.

Urine was negative for porphobilinogen.

Reti-cuhocyte count was 1.2/100 erythrocytes. Stools

were negative for ova and parasites. Tubercu-lin (P.P.D.) skin test was negative; histoplasmin

skin test resulted in a 12 mnm area of erythema

and! indluration.

Roentgenograin of tile chest (Fig. 2) showed

progression and the infiltrates ill tile right humig

now had a nodular appearance. The

media-stina! shift, the emphysema amid infiltration of

tile left lung hadi persisted. An increase in

heart size with a globular appearance

sug-gested cor pulmonale. Roentgenograms of the pelvis and hip joints were normal except for slight bowing of the femoral shafts, probably developmental in nature. The spine was normal except for some straightening of the normal lordotic curvature, suggesting spasm of the as-sociated muscle. The hands and feet showed no osseous or articular abnormality, bitt growth

retardation was notezi.

Subsequent Course

During the first 10 das iii the hospital he

became less irritai)le. His temperature

fiuictim-,ited between 37#{176}Cand 39#{176}C.The following

laboratory data were obtained : concentration

of chloride in sweat was 17.6 mEq/l,

concen-tration of total protein in the serum was 7.5

gm/100 ml (albumin 3.6 gm/100 ml, globulin

3.9 gm/100 ml); concentratiomi of gamma

glob-ulin (electrophoretic) normal; tryptic activity

of duodenal drainage 100% of normal; stool was

negative for occult blood; urine was negative

for porphobihinogen. Another duode’ial drain-age showed normal trptic, hipase and amvlase

activity. Two gastric washings for hernosiderin

were negative.

During the second 10 days in the hospital

the temperature continued unchanged and

muscle spasm diminished greatly. He started

sitting by himself and stood with assistance.

No increased respiratory difficult’ was noted

and repeated roentgenograms of the chest

failed to disclose an change.

The patient was improving slowly, bitt on the forty-second hospital day rapid

respira-tions, subcostal retractions and fever developed.

The chest was full of rhonchi. The liver edge was palpable 6 cm below the costal margin.

No cyanosis was noted. Leukocyte count was 22,500/mm3, 65% neutrophils, 33%

lympho-cytes, 2% monocytes. The concentration of

he-moglobin was 10.6 gm/100 ml. Nasopharyngeal

culture yielded pneumococcus. The

concentra-tion of cholesterol in the blood was 189 mg/

100 ml. Roentgenogram of the chest was un-changed. He was given penicillin, tetracycline and oxygen. Antibiotics were discontinued after

2 weeks.

Through tiliS period! the patiemit regained

most of the function of the lower extremities

and was able to pull up, to a standing position

and then walk. After 52 days the child was

discharged.

(4)

282 HAMMAN-RICH SYNDROME

Ftc. :3.Rocntgellograln of chest taken shortly be-fore (leatllat 4 years of age.

sixth and filial timiie. \Veight was 1:3 kg and temiiperature was :37#{176}C.Physical examination

showed tile following: There was a

barrel-chest (leforniit\; the chest was hperresonant

to percussion, breath sounds were coarse with crepitamit rales bilaterally; tile edge of the liver

was palpable 5 ciii below the costa! margin imi

the right rnid-clavicuhar line. Roen tgenogram

of tile chest was unchanged.

Biopsy of the lung and of a mediastinah node

prOvidedl pathologic diagnoses of interstitial

pulmonary fibrosis and obliterative 1)ulmomlar\’

arteriolosclerosis.

Postoperativeh’ the I)atiemit was given

peni-cilhin, streptomvcin and fluids intra’enousl’.

Repeated roentgenograms (Fig. 3) showed the

left bug to be markedly einphysematous, the

right lung showed some compressiomi from the

shifted mediastirmumn. A small pnemnnothorax

was noted on the right on one occasion. Four

day’s after biopsy his getieral comidition

deteri-orated rapidly and respirations became labored!

with flaring nostrils. Pulse becamne more rapid!

and the liver was tender and the lower edge

palpable 6 cm below the costal margimi. He was

digitalized with digoxin. Electrocardiogram

showed sinus tachycardia andl right ventricular

hpertroph’.

The face and upper extremities became

puffy while the lower extreniities showed no

edema. The child expired on December 10,

1956, 2 dlavs before his fourth birthdlay.

Necropsy

GROSS FINDINGS: The significant fimidings

were in the chest. The thoracic amiteroposterior

diameter was imicreased amid the chest was

barrel-shaped!. The lumigs (Fig. 4) filled the

respective pleural cavities amid almost mnet in

the midilimie. Each of the pleural cavities

con-tamed 20 mnl of pale, clear, straw-colored! fluid.

The pleura was smooth and glistenimig except

over the site of biopsy. The right lung weighed

170 gm, the left 230 gm. (The expected normal

Fmc. 4 (Left). Anterior view of heart and lung. Note the dilatation of the pulmonary artery and bulging right ventricle. The lungs arevoluminous and retain their shape.

Hc. 5 (Right). Posterior view of heart and lungs showing the pulmollary hilar vessels. 1. Stenotic right

pulmonary vein. 2. Stenotic right pulnionary’ artery. 3. Left atrium. 4. Dilated leftpulmonary vein.

(5)

weights are 90 gm amid 85 gm.) No crepitation

\‘as elicited except at the very’ apex of the left

lung. Both lungs were firm in comisistency’. The

right lung was grey’ with reddish-purple

mot-thug posteriorly, the heft lung red with

scat-tered purple blotches. There was no

medias-tinal shift. The trachea was empty and

pre-sented a iiale glistening mucosa. In the major

bronchi the mucous membranes were slightly

reddlenedi.

The heart ‘as opened in situ (Fig. 5). The

right atrium was markedl’ dilated and the

right ventricle was at least twice as large as the

left. The left ventricular vahl measured from

0.7 to 1.0 cm iii thickness. The right ventricular

wall measuredi from 0.8 to 0.9 cm in thickness.

There were mio septal defects and no evidence

of valvular disease. The valve circumferences

were as follows: tricuspid 6.5 cm, pulmonic

5.0 cm, aortic 3.9 cm, mitral 5.5 cm. The

pul-monarv artery was slightly larger than the

aorta. The left pulmonary artery measured 1.4

cm in diameter. The main and left pulmonary

arteries were pliable. The right pulmonar’

ar-terv was firm and thickened. All of the middle

mediastinal adventitial tissue was thickened

l)umt this was more marked on the right side

than on the left.

The left pulmonary’ artery and aorta were

dissected without difficulty although some

re-sistance was encountered along the right side

of the aortic arch. The adventitial tissue around

the right pulmonary artery was markedly

thickened and the reduction in size of this

vessel became even more apparent as one

ap-proached the hihus of the right lung. The main

artery and its lobar branches were thickened

and their lumens were much reddened.

Section-ing of the main artery almost at its bifurcation

revealed a wall 1 mm in thickness and a lumen

2 mm in diameter. The right pulmonary vein

amid the lohar veins of which only two were

identified were markedly reduced in size,

thickened and just barely admitted a fine

probe. On tracing the right pulmonary vein to

the left atrium, its lumen reduced progres-sively in size and at its junction with the left

atrium there was no lumen whatever, and a

fine probe could not be passed from vein into

atrium. The left pulmonary vein and its lobar

bramiches were dilated and filled with blood. The bronchial arteries were not remarkable.

The right lung was firm, and presented a

striking pale grey color with a yellowish tinge.

There was fine anthracotic mottling and a few

petechial hemorrhages. On the lateral surface

of the upper lobe, just above the fissure, was the site of lung biopsy, closed by interrupted

sutures. There was very little pleural reaction.

On the lateral aspect of the upper lobe just

below the apex there was a projecting, firm,

hemispherical nodule measuring approximately’

1 cm in diameter at its base and 0.5 cm in

height. Section revealed a yellow caseous focus

encapsulated by a grey’ line of fibrous tissue.

The secondary and smaller bronchi presented

slightly’ reddened mucosa. The bronchiolar

mu-cosa was roughened and yellow. The bronchi

were distinctly dilated and cuild be dissected

easily almost to the pleura. There was also

ribbing and early sacculation. Here amid there

flecks of muco-pus were seen. The cut surface

of the right hung (Fig. 6) was yellow-gre and

fairly homogeneous except for the bronchi that

stood out with their thickened walls. The

strik-ing feature was the absence of discernible

yes-sels.

The heft lung was red to red-purple. The

pleural surface was smooth and mottled by

small lobular hemorrhages. Dissection of the

bronchi revealed a distinctly reddened mucosa

throughout. There was bronchiectasis, but not

as marked as the right side. In contrast to the

right side, the bronchi were filled with a thin

frothy fluid and it was only in the lower lobe

that a little muco-pus was identified. The cut surface (Fig. 7) was wet, very’ dark red in color and the dilatation of arteries and veins was striking.

The

mediastinal

nodes

were red, enlarged

and varied in size from 1 cm in diameter to

3.0 X 2.0

x

1.0 cm.

The

nodes on the right

side were far more enlarged, prominent and

matted than those on the left. On cut surface

all nodes were slightly lobulated, moist and

reddish-brown. A few nodes on the right side

contained scattered tiny 1 mm caseous yellow

foci. Some of these were calcified.

Cultures of heart and lung y’ielded n growth. Culture taken from the right main

bronchus yielded Friedlander’s bacillus and

alpha hemolvtic streptococcus.

MIcRoScOPIc FINDINGS: Both lungs pre-sented widespeard, diffuse, interstitial pneu-monia and interstitial fibrosis (Fig. 8).

The

right lung contained very little air; possibly 10% of its volume was air-containing. The left

(6)

284 HAMMAN-RICH SYNDROME

Fic. 6 (Upper). Cut surface of right lung showing an almost COIilphete absence of

discernible vessels.

F’mc. 7 (Lower). Cut surface of left lung showing congestion and dilatation of

vessels.

volume. Tile fibrosis was characterized by’

broad bands of moderately cellular

fibrocolla-gen separating small, irregular. air-filled spaces

hued by cuboida! or flattened!, proliferated

bronchiolar epithehiuum. Arm mud the bronchi,

bronchioles and pulnionarv essels the fibrosis

was more mnarked. The scam’ tissue was

mnoder-atelv to heavily infiltrated by lymphocytes,

plasma cells amid a small number of reticulum

cells amid neutrophils. The air spaces were

either empty or contained small chumps of

eosinophilic macrophages. Occasional spaces

were filled with plugs of pus amid these also

occluded a few bronchiolar lumens. The

bron-chiolar amid bromichial walls were diffusely

in-filtrated by lymphocytes and plasma cells with a

smaller number of admixed mieutrophils. Imi the

right lung only rare areas of pneumonitis with

little or no scarring were found. In the left

(7)

mod-Fmc. 8 (Upper). Photomicrograph of lung showing interstitial fibrosis. The

air-contain-ing spaces are lined by proliferated bronchiolar epithelium. Some of the lumens

con-tain macrophages.

(x

160.)

Ftc. 9 (Lower). Photomicrograph of small pulmonary artery showing lumenal stenosis

(8)

286

HAM

MAN-RIdI

SYNDROME

Complete obstructiomi of right pulmonary

crate to mninimnal pmletmmiiomlitis, congestion and

no scarring. It must be emphasized, however,

that these scattered umiscarred areas accounted

for a relativel small amount of lung. The

subpleural and septal lmphatics were

mark-edhlv dilated. Occasional subpheura!

emphyse-mnatoums blebs were seen.

A striking feature in the right lung was the

vascular sclerosis, prepond!erantly arterial but

also involving the veins. Large and medium

sized vessels showed marked intimal fibrosis

with severe narrowimig or occlusion of the

lu-mnens (Fig. 9). Arterioles amid small arteries

presented marked medial hyperplasia with the

saune severe narrowing or occlusion of the

hummen. Occasionally’, the smaller vessels

pre-sentedi intimal pol’s’morphonuclear infiltration

and, rarely, there was complete pits cell

iii-filtration and necrosis of the wall. The right

puhmnomiary artery sectioned close to its

bifurca-tiomm revealed marked eccentric intimal

fibro-sis; amid the fibrotic area contaimied a few

scat-tered h’mphoc’tes and pus cells, patches of

edema amid sonic proliferating capillaries. The

right pulmonary vein showed marked

colla-genous imitimal thickening. In the left lung the

large and medium sized vessels were, for the

most part, dilated. However, approximately 50% of the arterioles showed medial

hyperpla-sia with severe narrowing or occlusion of the

lumen.

Microscopic examination of the heart

re-vealed marked right vemitricular hpertrophy.

The left vemitricular muscle fibers did not

ap-P’ emilarged.

FINAL DIAGNOSES: The fimial diagnoses were

as follows:

Pumlmomiary fibrosis, imiterstitial, diffuse, more

marked in right lung.

Chronic interstitial pneumomiia, marked imi

right, moderate in left.

Bronchiectasis, saccular in right side, cylin-(Irical in left.

Pulmonary arteriosclerosis and

phlebosclero-sis.

Obliterative arterioloscherosis, severe in

right, slight to moderate in left.

Acute necrotizing angii’tis, right; very

slight in left.

Stemiosis and sclerosis of right pulmonary

artery amid! major branches.

Stemiosis amid sclerosis, right pulmonary

veins.

venous return at atrial junction.

Dilatation of pulmonary artery, left

pumhmon-ary artery and branches.

Pulmonary edema and cangestion, chronic,

left.

Pulmonary hemorrhages, recent and old, left.

Cor pulmonale.

Right ventricular hypertrophy and

dilata-tion

Right atrial hypertrophy and dilatation.

Chronic passive hepatic congestion, slight.

Hepatic fatty metamorphosis,

centrilobu-lar.

Hydropericardium, slight.

Hvdrothorax, bilateral, slight.

Ascites, slight.

Edema, peripheral amid mediastiuial, slight.

Clubbing of fingers.

Erythroid normoblastic hperplasia of

marrow, slight.

Acute suppurative, obstructive bronchiohitis,

slight, bilateral.

Chronic bronchitis and pen-bronchitis,

bilat-eral.

Caseous granulomatous primary complex

(histoplasmosis, partially healed), right lung amid

mediastinal nodes.

Barrel-chest deformity.

Thoracotomy and lung biopsy, postoperative.

Poliomyelitis, residual, with minimal

en-cephalitic changes.

Atrophy of upper amid lower extremities.

Posterior root ganglionitis, due to residumal

pohiomyehitis.

Chronic focal sialadenitis.

Ectopic pancreatic tissue, intestinal.

DISCUSSION

The

patient’s

course

was

one of increasing

dyspnea and ultimate development of cor

pulmonale.

The

progressive

pneumonits,

fi-brosis of undetermined etiology and the

pulmonary arteriolosclerosis explain the

symptoms and are typical of the

Hamman-Rich syndrome.

The progressive shunting of blood from the right to the heft lung and the disparity in content of blood between the two lungs

is atypical. Also atypical is the fact that the

fibrosis was more advanced on the right side than on the left. The preponderance of

disease in the right side was seen

(9)

tlii(I Braid.’ The evidemice strongly suggests

that

death occurred when a critical number

of arteriolar lumens were stenosed or

ob-hiterated in the left lung, the right lung

l)eing virtually ischemic at this time.

The hilar findings at the right lung were

surprising and are possibly unique. I have

been unable to find a reported similar

in-stamice. Stenosis of main pulmonary vessels

are almost always congenital anomalies, but

there are objections to assuming that these

iiilar vascular lesions are congenital. One

cannot explain how blood left the right

king.

The bronchial veins were not

promi-nent; there were no accessory veins and no

pleural adhesions. The bronchial arteries

showed no evidence of hyperplasia and the

right bronchial tree was not hypoplastic.

There was no interference in the growth of

the lung. The disparity in the size and

weight of the two lungs could be accounted

for by the increased fibrosis in the right

lung and the increased blood volume in the

left. While it is true that the right lung had

omily two lobes, this hardly constitutes an

anomaly amid no other anomalies were

pres-cut.

It is suggested, rather, that the stenosis

of the right pulmonary artery and vein are

1)d)th inflammatory in origin. Gross

examina-tion revealed mediastinal scarring on the

right side, particularly around the vessels,

and the hilar lymph nodes were matted. It

seems likely that the initial inflammatory

process of the lungs involved the hilar

areo-lar tissue, possibly directly and possibly via

the lymph nodes. Inflammation may even

have involved the vessels directly at one

time. At any rate, the cicatrization would

gradually narrow the vessels. It is

diffi-cult to see how disuse atrophy subsequent

to arteriolosclerosis would produce such

markedi sclerosis and stenosis although it

miiay have played a part in the atriovenous

occlusion. During the patient’s course some

of the roentgenograms of the chest showed

the presence of costophrenic septal lines in

the right lower lung field. Bruwer, Ellis and

Kirkhin14 attribute the presence of such lines

to pulmonary venous hypertension. Their

presence unilaterally, therefore, wo uld

sup-port the idea that there was pulmonary

venous hypertension due to progressive

mar-rowing and sclerosis of the right pulmonary

vein. Given a lung with a stemotic

pulmo-nary vein and artery, it is possible to

postu-late that, as the arteriolosclerosis progressed!,

a point might be reached where insufficient

blood left the lung through the vein and the

atrial block might have occurred on the

basis of stasis.

The history suggests a pulmonary

infec-tion starting at 4 months of age. It may have

originated in the right lung or have been

bilateral but preponderant on the right side.

A

tempting

hypothesis

is that

a

bout

of

aspiration may have initiated the process

and that Friedlander’s bacillus may have

been responsible for the pulmonary

scar-ring.

SUMMARY

The Hamman-Rich syndrome is described in a 4-year-old Negro male. The clinical

picture was that of persistent cough and

progressive dyspnea beginning at 4 months

of age. Diagnosis was made ante mortem

by lung biopsy. The fibrotic process and

arteriolosclerosis were more markedi in

tue

right lung. The disease may have been

ini-tiated by a bout of aspiration. There was accompanying stenosis of the right

pulmo-nary artery and vein and occlusion of the

lumen of the right pulmonary vein at its en-trance to the left atrium. The hilar vascular

findings are believed to be secondary to

hilar areolar inflammation.

REFERENCES

1. Hamman, L., and Rich, A.: Fulmimiating

diffuse interstitial fibrosis of the kings.

Tr. Am. Chin. & Chimatol. A., 51:154,

1935.

2. Eder, H., Hawn, C., and Thormi, G.:

Re-port of a case of acute interstitial

fibro-sis of the lungs. Bull. Johmis Hopkins

Hosp., 76:163, 1945.

3. Potter, B. P., and Gerber, I. E.: Acute

diffuse interstitial fibrosis of the lungs.

Arch.

mt.

Med., 82:113, 1948.

4. Peabody,

J.

W., Peabody,

J.

W., Jr., Hay’es,

E. W., and Hayes, E. W., Jr.: Idiopathic

(10)

288

HAMMAN-RICH

SYNDROME

i(leultica! twiui sisters. I)is. Chest, 18:

:330, 1950.

5. Fluniphrevs. F. NI. : (imrouiic progressive

pulmonary fibrosis. NI. (liii. North

America, 35: 169, 1951.

6. Peabody, H. D., Moersch, H.

J.,

amid

Ed-wards,

J.

E. : Clinically indeterminate

pulmomiary fibrosis; a pathological study.

J.

Thoracic Surg., 21:519, 1951.

7. MacMillan,

J.

M. : Familial pulmonary

fi-brosis. Dis. Chest, 20:426, 1951.

8. Rubin, E. H., Kahn, B. S., and Pecker, D.:

Diffuse interstitial fibrosis of the lungs.

Ann.

mt.

Mccl., 36:827, 1952.

9. Peabody,

J.

W. , Jr., Buechner, H. A., and

Amiderson, A. E. : Hamnman-Rich

syn-drome; amialysis of current concepts and

report of three precipitous deaths

follow-ing cortisone and corticotropin (ACTH)

withdrawal. Arch.

mt.

Med., 92:802,

1953.

10. Bradley, C. A. : Diffuse interstitial fibrosis

of the lungs iii childremi.

J.

Pediat., 48:

442, 1956.

1 1. Baar, H., and Braid, F. : Diffuse

progres-sive interstitial fibrosis of the lungs in

childhood. Arch. Dis. Childhood, 32:199,

1957.

12. Feinerman, B., and Harris, L. E. : Unusual

interstitial pneumonitis; report of two

cases occurrimig iii children. Proc. Staff

Meet., Mayo Chin., 32:637, 1957.

13. Callahan, W. P., Sutherland,

J.

C., Fulton,

J.

K., and Kline,

J.

R.: Acute diffuse

in-terstitial fibrosis of the lungs. Arch. Int.

Med., 90:468, 1952.

14. Bruwer, A.

J.,

Ellis, F. H., and Kirklin,

J.

XV.: Costophrenic septal hues in

pul-monary vemious hypertension.

Circula-tiomi, 12:807, 1955.

SUMMARIO IN INTERLINGUA

Syndrome

Dc

Hamman-Rich

In Pueritia

Le syndrome de Hamman-Rich consiste de

bilateral fibrosis interstitial diffuse del

pul-momies. Su etiologia es imicognoscite. Illo se

manifesta chimiicamemite per tusse, dyspmiea, e

cyanosis, associate con un minimno de

consta-tationes physic. Le curso del sy’uidrome es

es-sentiahmemite afebril. Le dvspmiea progrede e

resulta in morte per asphyxia. Le

roentgeno-gramma momistra dlensitates paremichymal e

hilar, e istos-in he curso del tempore-se

dis-veloppa in diffuse affectiomies bilateral. Le

comis-tatationes pathologic consiste die fibrosis

puul-monar interstitial comm vane grados de

pneu-11101 H intersti tial e (Ic sclerosis arteriohar.

Us(1uue I 956, oiiiiie le CmI5OS reportate esseva

cmt505 de adlultos. Depost ihle tempore, 4 casos

in juveniles ha essite describite. Le etates de

iste patientes esseva imlter 7 menses e 9 annos.

Le presente reporto describe he syndrome de

Hamman-Rich in un masculo de racia negre de

4 annos de etate. Le diagnose esseva establite

ante morte per biopsia pulmonar. Esseva

tro-vate, in plus, stenosis del arteria

dextero-pul-monar e occlusion complete del vena

dextero-pulmonar. Istos rendeva he tableau plus

compli-cate.

Le maladia del patiente comenciava a! etate

de 4 menses con tusse, febre, e infiltrationes

roemitgemiographic del pulmon dextere, con

moderate grados de displaciamento dextrorse

del mediastino. Le crescentia e he

disveloppa-memito general del patiente esseva

compromit-tite, e quando ille habeva 1 anno de etate, su

roentgenogramma thoracic monstrava grossier

infiltrationes bilateral. Le dyspnea progredeva,

e a! etate de 23 annos, lineas septal

costo-phrenic del latere dextere esseva notate in he

roentgenogramma del thorace. Repetite essayos

a determinar le etiohogia de iste mnorbo

pul-monar remaneva sin successo. Duo nienses ante

su morte, he patiente contraheva pohiomyelitis,

sed ab isto ille se restabhiva quasi

complete-mente. Terminalmente ille disveloppava corde

pulmonal, e ille moriva per asphyxia.

Le necropsia revelava sever grados bilateral

de fibrosis interstitial e vane intemisitates de

pneumonia interstitial que alficeva Ic integre

pulmon dextere e le plus grande parte del

pul-mon sinistre. Arterioloscherosis oblitterative

es-seva marcate in le pulmon dextere. Illo esseva

moderatemente sever in he pulmon sinistre. In

tanto que le vasos se contraheva, de plums in

plus sanguine esseva shuntate verso he pulmon

sinistre. Es suggerite in plus que he

obhittera-tive arteriosclerosis pulmonar esseva causate in

parte per inflammation e in parte per

hyper-tension pulmonar secundari a! ob!itteration del

capillatura per he morbo inflammatori e

cica-trical. Le asphyxia mortal supervemiiva qumando

un numero critic de arteriolas del pulmon

sinistre esseva obhitterate.

Le datos colligite durante le numerose

hos-pitahisationes del patiente non permitteva

(11)

1958;22;279

Pediatrics

Israel Diamond

Unilateral Pulmonary Arterial and Venous Stenosis and Atriovenous Occlusion

THE HAMMAN-RICH SYNDROME IN CHILDHOOD: Report of a Case with

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1958;22;279

Pediatrics

Israel Diamond

Unilateral Pulmonary Arterial and Venous Stenosis and Atriovenous Occlusion

THE HAMMAN-RICH SYNDROME IN CHILDHOOD: Report of a Case with

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