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Aplasia of the Diaphragm


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hematogenous osteomyelitis, have little or no

effect against pseudomonas species. The

poly-mixin group of antibiotics, in addition to their

renal toxicity, penetrate tissues poorly which

limits their effectiveness in situations where

this is desirable.6 Gentamicin appears to be a

promising agent against pseudomonas but

ex-perience in osteomyelitis is limited.7 Both

oto-toxicity and renal toxicity have been associated

with its use. Recently, carbenicillin8 has been

advocated specifically for its effect against

pseumonas species. In certain situations it

shows synergism7 when used with gentamicin,

but rapid emergence of resistance9 has already

been reported when it has been used alone.

\Vhile pseudomonas species are usually

resis-taut to less toxic agents, this should not

pre-elude appropriate sensitivity testing in an

at-tempt to circumvent some of the problems

mentioned. In this disease long-term

hospital-ization is the rule with residual radiologic

changes and/or orthopedic deficits persisting in

some cases.

Why pseudomonas species are the pathogens

in the patients reported thus far, is not clear. It

may be that antibiotic therapy aimed at the

usual causative agents is a factor in selecting

the organism. Most of the patients have had

such therapy for varying periods prior to

defini-tive diagnosis. Whether pseudomonas species

can on occasion be part of the normal skin flora of the foot is speculative. If this were the case

the puncture wound might serve to directly

in-oculate the bone involved with this organism.

It is evident that while most puncture

wounds of the foot resolve without any

se-quele, on occcasion complications ensue. A

puncture wound of the foot in which pain and

inflammation fail to resolve or in which such

signs and symptoms recur should be reason for

a vigorous diagnostic approach. An awareness

that traumatic osteomyelitis can follow such

in-juries, that the signs and symptoms contrast

markedly from those seen in hematogenous

os-teomvelitis, and that pseudomonas species may

be the pathogen, will hopefully modify the

ab-sence of appropriate therapy which thus far has

typified the early management of such cases

and decrease the overall morbidity.


This paper reports three cases of

pseudo-monas osteomyelitis complicating puncture

wounds of the foot. The clinical features which serve to differentiate this entity from

hematog-enous osteomyelitis are presented along with

the therapeutic considerations.



Departments of Pediatrics and


The Johns Hopkins Hospital University

School of Medicine

Baltimore, Maryland 21205

David H. Carver is a Kennedy Scholar.

This investigation was supported b Grant

HD-00091-05 from the National Institute of Child

Health and Human Development.


1. Griffin, P. P.:Bone and joint infections in

cliii-dren. Pediat. Clin. N. Amer., 14:533, 1967.

2. Johanson, P. H. : Pseudomonas infections of the

foot following puncture wounds. J.A.M.A.,

204:170, 1968.

3. Swick, H. M., Maxwell, E., Charache, P., and Levin, S. : Peritoneal dialysis in colistin

intoxi-cation: Report of a case. J. Pediat., 74:976,


4. Nunnery, A. W., and Riley, H. D., Jr. : Gentami-cm: Clinical and laboratory studies in infants

and children. J. Infect. Dis., 119:460, 1969.

5. Feigin, R. D., McAlister, W. H., San Joaquin,

U. H., and Middlekamp, J. N. : Osteomvelitis

of the calcaneus. Amer. J. Dis. Child., 119:61,


6. Jawetz, E. : Polymyxin, Neomycin, Bacitracin.

New York,: Medical Encyclopedia, Inc., pp.

11-35, 1956.

7. Smith, C. B., Dans, P. E., Wilfert, J. N., and

Finland, M. : Use of gentamicin in conibina-tions with other antibiotics. J. Infect. Dis.,

119:370, 1969.

8. Neu, H. C., and Swarz, H. : Carbenicillin:

Clini-cal and laboratory experience with a

paren-terally administered penicillin for treatment

of pseudomonas infections. Ann. Intern.

Med., 71:903, 1969.

9. Lowbury, E. J. L., Kidson, A., Lilly, H. A.,

Ay-liffe, C. A. J., and Jones, R. J.:Sensitivity of

pseudomonas aeruginosa to antibiotics : emer-gence of strains highly resistant to

carbenicil-lin. Lancet 2:448, 1969.


of the Diaphragm

According to Butler and Claireaux,1



ing death during the first few days of life are

secondary to diaphragmatic defects. The

re-ported incidence is 1 out of every 2,200 births.2

To our knowledge, diaphragmatic defects in

siblings, specifically aplasia of the diaphragm,

have not been reported in the American

litera-ture although there have been six reported

families in the European literature.38 Passarge,

et have recently reported a family and

re-viewed the literature. We would like to report

another family with aplasia of the diaphragm

and stress the importance of proper genetic



The first infant was the product of the first

preg-nancy of a 25-year-old mother and a 27-year-old

father. There was no exposure to any infectious

diseases, radiation, or drugs except for an

antiem-etic. Other than a weight gain of fifty pounds and

decreased fetal activity, the pregnancy was normal. Delivery was also normal and an increased amount

of amniotic fluid was noted. Birth weight was 8 lbs

5 oz. The infant had severe respiratory distress at

birth and died shortly thereafter. Pertinent findings

at postmortem examination included a small atelec-tatic lung on the left with the spleen, part of the stomach, all of the small intestine and colon except

for the sigmoid and rectum located in the left

tho-rax. The extact nature of the diaphragmatic defect

was not fully detailed. The right diaphragm was

normal and the right lung was somewhat

com-pressed. The heart was normal as were the liver

and spleen except for increased congestion. The

uterus was bicornuate. On microscopic examination

there was marked atelectasis of the lungs and

con-gestion of the liver and kidney. The second

preg-nancy resulted in a normal female. In the third

pregnancy, which occurred in 1969, there was no

exposure to any infections, radiation, or drugs cx-cept for an antiemetic. The mother gained 35 pounds and noted decreased fetal activity. An 8-lbs

12-oz male was delivered normally but died shortly

after birth. Pertinent findings at postmortem

exami-nation revealed almost complete absence of the

diaphragm on the left side. The pleural and

perito-neal cavities were distended with air, possibly

see-ondary to a ruptured lung cyst. The spleen,

stom-ach, and part of the transverse colon were in the

left chest cavity. On the right, about one third of

the diaphragm was present and the liver was

buig-ing into the chest cavity. The appendix and splenic fiexure were on the left but the lower part of the

colon was on the right. It was felt that the partial

malrotation might be secondary to the trapping of

the transverse colon in the left chest. The lungs

were small and not expanded and the left lower

lobe contained a cyst filled with air. The heart was

of normal size with a small left atrium and a large

right atrium. There was a single ventricle and the

great vessels leading from the heart were derived

from a common trunk which served as both an

aorta and pulmonary artery. The rest of the organs

were normal.

There was no history of diaphragmatic defects in

three generations of this family although one infant

died at birth of an unknown cause. Chromosomal

analysis of the mother was normal and the

derma-toglyphics of tile mother and father were also nor-mal.


Passarge, Halset, and German7 suggest that

unilateral agenesis of the diaphragm should be

differentiated from other types of

diaphrag-marie defects. The most common type of

dia-phragmatic defect in infants is herniation

through the foramen of Bochdalek which

in-volves the posterolateral aspects of the

dia-phragm. The left side is more frequently

in-volved than the right. Another less common

type of diaphragmatic hernia is herniation

through the anterior aspect of the diaphragm

near the sterno-costal junction or through the

foramen of Morgagni. Eventration of the

dia-phragm may also occur and in these cases there

is no hernial orifice, but the leaf of the

dia-phragm is stretched out because of deficiency

of muscle. Congenital absence of the

dia-phragm is very rare and in his series of 117

cases of diaphragmatic hernias in infants and

children at Children’s Memorial Hospital, Baffes did not encounter a single case.#{176} Pas-sarge and his co-workers postulate that

unilat-eral agenesis of the diaphragm does not

rep-resent part of a spectrum of diaphragmatic

defects but constitutes a separate genetic entity.

The embryological abnormality causing

agene-sis of the diaphragm differs from the

abnormal-ity causing the nonfamilial form of

diaphrag-matic hernia. The most frequently described

anatomic defect in the familial foi-m suggests

that three of the four parts of the left

dia-phragm anlage do not develop and only the

fourth portion, the marginal in growths of

body-wall muscles, is present. In our first

pa-tient the exact nature of the diaphragmatic

de-feet is not known. Our second patient had

al-most complete agenesis of the left diaphragm

and agenesis of two thirds of the right

dia-phragm. This bilaterality has not been

previ-ously described and, although we cannot be



the familial form. Our second patient also had

an associated congenital heart disease which

has not been previously reported. The mother

did not take any of the drugs which have been

suggested as possible causes of diaphragmatic

defects in the fetus, namely thalidomide,’0

qui-nine, or phenmetracine hydrochloride.6

Vita-mm A deficiency induced in rats has been

re-ported by Warkany12 to produce

diaphrag-matie defects in their offspring.

In infants with diaphragmatic abnormalities,

the specific type of defect should be

deter-mined and if aplasia of the diaphragm is

pres-ent, the possibility of recurrence in subsequent

siblings (perhaps as high as 25%) should be

discussed with the parents. Agenesis of the

dia-phragm appears to be inherited as an

autoso-mal recessive trait although there are still too

few reported cases to be certain. Consanguinity

was not present in any of the reported families,

nor in our family, and this is unusual in such a

rare autosomal recessive condition. Ten Kate

and Anders8 raise the possibility of a

multifac-tonal genetic causation, although there is no

good evidence for this hypothesis.


Department of Pediatrics

Tufts University School of Medicine


Center for Genetic Counseling and

Birth Defect Evaluation

Boston Floating Hospital

for Infants and Children

20 Ash Street

Boston, Massachusetts 02111

Postmorten examinations were performed by

Richard A. Durham, M.D., Lowell General Hospi-tal, Lowell, Massachusetts.


1. Butler, M., and Claireaux, A. E. : Congenital

diaphragmatic hernia as a cause of perinatal mortality. Lancet, 1 :659, 1962.

2. Rubin, A. : Handbook of Congenital

Malforma-tion. Philadelphia: W. B. Saunders, 1969.

3. M#{228}kelii, V. : Hernia diaphragmayica congenita spuria. Finsk. Lakaresalisk. Handl., 58:1107, 1916.

4. Philipp, E. E., and Skelton, NI. D. : Congenital diaphragmatic hernia in siblings. Brit. Med.

J., 1:1283, 1952.

5. Mertins, H. : Uber eine familiare Zverchfell-mill bildung. Zbl. Gyn#{227}ek., 74:951, 1952.

6. Powell, P. D., and Johnstone, J. M. :

Phennie-trazine and foetal abnormalities. Brit. Med.

J.,2:1327, 1962.

7. Passarge, E., Halset, H., and German J.:

Uni-lateral agenesis of the diaphragm.

Human-genetik, 5:226, 1968.

8. Ten Kate, L. P., and Anders, G. J. P. A. : Uni-lateral agenesis of the diaphragm. Human-genetik, 8:366, 1970.

9. Mustard, W. T., Ravitch, M. NI., Snyder,

W. H., Jr., Welch, K. J., and Benson, C. D.:

Pediatric Surgery, ed. 2. Chicago: Year Book

Medical Publishers, Inc., 1969.

10. Hobolth, N. : Drugs and foetal abnormalities.

Lancet, 2:1333, 1962.

11. Zolcinski, A., Heimrath, T., and Ujec, M. :

Chi-nina jako przyczyna wad rozwojowych

plodu. Ginek. Pol., 36:935, 1965.

12. Warkany, J., Roth, C. B., and Wilson, J. C.:

Multiple congenital malformations: A

consid-eration of etiologic factors. PEDIAriucs. 1:

462, 1948.

A Dangerous








to an Infant

in a Baby


One method of elevating the ambient oxygen

of a small baby during the admission

proce-dure is to place his head in an oxygenated

hood. We describe here a situation recently

en-countered in which a premature infant who

was being provided 68% oxygen environment,

administered with the aid of a head hood in an

infant warmer, and was effectively cut off from the source of radiant heat.

The hood which was utilized was made of

3” plexiglass, 8”


9” x 5” high and was

pro-vided with a cutout on one side which normally

fitted loosely about the child’s neck when in

use.#{176} When used with normal size infants the

hood only covers the head. However, when an

extremely small infant is being treated, the

hood may cover a significant portion of his body.

Indeed some active infants have squirmed

about until the entire body was inside the

hood. The danger arises from the use of this

type of hood in conjunction with a radiantly

heated infant warmer. The plexiglass absorbs

almost all of the radiation from the heating

element and thus an infant whose body is mostly inside the hood receives very little

0Our 1100(1 was homemade, l)tlt there are se-eral commercial units available which are made of




Murray Feingold

Aplasia of the Diaphragm


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Aplasia of the Diaphragm


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