Talk:Paper - Congenital deficiency of the pericardium (1916)
CONGENITAL DEFICIENCY OF THE PERICARDIUM. By Martin R. Cuasz, M.D.
(From the Anatomical Laboratory of the Northwestern University Medical School.!)
A CONSIDERABLE number of cases of congenita] deficiency of the pericardium have been recorded. Faber in 1878 collected ten cases. Ebstein, 1910, found thirty-two cases reported. Perna, 1910,and Plaut, 1913, have recently summarised the literature on the subject, and reported additional cases. The reader is referred to their papers for a complete consideration of the literature.
It is the purpose of this paper to present a short description of a case found in this laboratory. At the time the body came under our observa- tion, all parts except the chest had been dissected, thus losing the identity of the subject.
The chest was that of a fairly well-developed adult male presenting a marked kypho-scoliosis. On opening the right pleural cavity it was noted that there were extensive adhesions between visceral and parietal pleura, and evidence of tuberculous involvement of the right lung. The mediastinal partition seemed normal.
On opening the left pleural cavity, however, the left lung and the heart came clearly into view, lying in a common serous cavity. Examination shows that this is due to a large defect of the left half of the parietal pericardium. The visceral pericardium is normal. Around the base of the heart is a more or less sickle-shaped fold with a free border, which almost encircles and partially encloses the base of the heart. This fold begins on the superior aspect of the root of the left lung as a reduplication of the serous membrane about 1°5 cm. wide, having its attachment along a line passing horizontally forward, across the ascending aorta to the mediastinal partition, thence downward as a reduplication of the mediastinal pleura to reach the diaphragm just to the left of the sternal attachment of the 6th left costal cartilage. From this point.a continuation extends upwards over the dome of the diaphragm, passing to the left of the inferior vena cava, to blend with the left pulmonary ligament.
Taken as a whole, the left portion of the pericardium may be considered as a reduplication of the serous lining of the common pleuro-pericardial
1 Contribution No. 39. 800 Dr Martin R. Chase
cavity, encircling the base of the heart from the upper aspect of the root of the lung to the pulmonary ligament on the inferior aspect of the pulmonary
Root of lung.
! | 1 | 1 ! | 1
Phrenic nerve. --—+|—.--------=
Phrenic nerve. — ==
Drawing of heart from in front and to the right, showing cut section of pulmonary root, and rudimentary fold of pericardium with phrenic nerve. Owing to the deformity of the spinal column the aorta appears to bend sharply to the right.
root. It contains on the right side where it is attached to the mediastinal pleura, and at its anterior attachment to the diaphragm, thickenings of Congenital Deficiency of the Pericardium 301
fatty tissue which in places assume the shape of tags of fat covered with serosa.
The fold varies in width from 2°5 em. along the mediastinum to 3°5 cm. where it is attached to the diaphragm. To the left and in front the visceral pericardium is directly in contact with the visceral pleura on the mediastinal surface of the left lung. More anteriorly and in the cardiac notch of the lung it lies against parietal costal pleura. Below, the heart is in contact with the diaphragmatic pleura.
The heart itself is of normal size and appearance. It is covered with serous pericardium which continues over the roots of the great vessels in a normal manner. The transverse and oblique sinuses of the pericardium are present. There are present no adhesions of the visceral pericardium with parietal pleura or lung, as was found in many of the cases reported, the serosa of the common pleuro-pericardial cavity being smooth and free in all parts. The heart shows no congenital defects. The ductus arteriosus and the foramen ovale are closed.
The phrenic nerve enters the thorax behind the left innominate vein, courses downward to the left of the innominate vein, crosses the arch of the aorta anterior and a little to the left of the origin of the left common carotid artery, and enters the fold of pericardium at its attachment just below the arch of the aorta. It then turns to the right along the free border of the fold, which it follows to the diaphragm. In some of the cases reported no rudiment of pericardium was found. In others rudiments were present ranging from tag-like pads about the base of the heart to crescentic folds completely enclosing a part of the heart. In a case reported by Keith, in a foetus presenting many other congenital anomalies, a small aperture was seen in the left side of the pericardium through which the left auricular appendage protruded. In Powell’s case there was an opening only one-eighth of an inch in diameter in the left side of the pericardium, which had smooth edges and showed no signs of erosion. In his subject the condition of pneumo-pyothorax was present, and the pericardial sac contained air and pus.
The course of the phrenic nerve was, in all cases except Perna’s, to the right of the deficiency. In cases of well-marked folds it ran in the fold near the free border. Where the fold was small or absent it ran in the mediastinal partition, between the two pleural sacs. In the cases of Versé and Picchi (quoted from Plaut) it ran in a fold behind the sternum.
In Perna’s case the phrenic took a course across the root of the lung VOL. L. (THIRD SER. VOL. XI.)—JULY 19:6. 20 302 Congenital Deficiency of the Pericardium
posterior to the pulmonary artery to reach the posterior part of the diaphragm.
So far as known, the condition has no clinical significance and has never been diagnosed ante-mortem.
Keith thinks the explanation of the condition is that the lung-bud grows through the pleuro-pericardial foramen, expanding that opening, and pre- venting its normal closure.
Perna suggests that early establishment of the anastomosis between the left and right anterior cardinal veins results in early atrophy of the left duct of Cuvier (common cardinal vein). Inasmuch as the pleuro-pericardial membrane, which normally completes the division of the pericardial cavity from the pleural cavity, is carried by the duct of Cuvier, early atrophy of the left vein would result in incomplete development of the left membrane and persistence and enlargement of the left pleuro-pericardial foramen. The fact that all known cases of congenital deficiency of the pericardium occurred on the left side would support this view.
M°Garry discusses the embryological significance of the malformation, and expresses the opinion that the occurrence of a patent pericardium is one aspect of a general condition. He explains these cases “ by supposing that in the early development of the embryo, some slight injury occurred to the general ccelom, which resulted in a lack of development of the pleuro-pericardial membrane.”
Faper, Virchow’s Archiv, Bd. Ixxiv.
Epstein, Miinchen. med. Wehnschr., 1910, Bd. x. S. 522.
Perna, Anat. Anz., Jena, 1910, Bd. xxxv. S. 522.
Piaut, Frankfurt Zischr. f. Path., 1913, Bd. xii. S. 141.
Keira, Journ. Anat. and Physiol., London, 1907, vol. xli. (3rd Ser. vol. ii.) 6. Powe.t, Trans. Path. Soc. London, vol. xx. p. 99.
Verst, Miinchen. med. Wehnschr., 1909, Nr. 57, 8. 2664.
M‘Garry, Anatomical Record, 1914, vol. viii. p. 43.