Talk:Paper - A case of unusual congenital malformation (1936)

A CASE OF UNUSUAL CONGENITAL MALFORMATION

By Pror. M. N. DE, M.B., M.R.C.P. (Lonpon) anpD H. K. DUTTA, M.B.

From the Department of Pathology, Medical College, Calcutta

Caszs of congenital abnormalities associated with maldevelopment of different organs simultaneously in one and the same individual are infrequent. The following case may be of sufficient interest to justify its publication.

Fig. 1.

The case was that of a still-born foetus apparently about 34-36 weeks old. The general development was below the average. There was marked scoliosis on the right side, the trunk and the lower extremities almost meeting each other at right angles. This bend was occupied on the right side of the abdomen by a bulging produced by the displaced liver and coils of intestine. The upper part of the body presented no peculiarities except that the nipples were absent on the chest wall. In the lower part, however, many abnormalities were seen, both in front and behind. 440 M. N. De and H. K. Dutta

The outstanding feature was the complete absence of the ventral abdominal wall resulting in the non-closure of the umbilicus and thereby producing a condition of exomphalos. Commencing from the lower part of the epigastric region and extending downwards to the pubis, there was a large inverted ‘“‘racket”’-shaped bare area, the lateral boundaries of which were formed by thickened cord-like folds of peritoneum containing the hypogastric vessels.

Fig. 2.

The abdominal wall on the right side of this gap, extending upwards to the right anterior axillary line, consisted of a thin layer of peritoneum only, the skin and musculature stopping abruptly along this line. The peritoneal covering dipped down at this part to be reflected backwards towards the posterior abdominal wall. On further examination, the floor of the racket-shaped area was found to consist of an upper markedly corrugated part and a lower smooth surface. The coiled intestine when traced caudally was found to terminate abruptly in this corrugated area without apparent further differentiation into large gut and rectum. Histological examination revealed the fact that the coils consisted Unusual Congenital Malformation 44]

of small intestine, while the corrugated area represented the wall of the large gut. There was no external opening of the proctodaeum, the position of the anus being indicated by a dimple in the anal region.

The liver was displaced laterally and forwards and was attached to the posterior abdominal wall by 4 pedicle containing its blood vessels.

Evidence of external genitalia was entirely lacking. There were two curious finger-like papillae, one on either side, on the medial aspect of the inguinal folds and situated at the extreme lower end of the mammary lines. At first sight each of them looked like a penis. But, on careful examination, these were found to be outgrowths from the skin only, thus probably representing ectopic and abnormal nipples. In a ridge-like body with a bulbous extremity, arising from the smooth area at the lower part of the ‘“‘racket”’ and projecting beyond its rounded distal end, rudiments of the prostate gland were found.

The ureters were very tortuous, considerably dilated, and their orifices were found buried in the smooth area at the floor of the ‘“‘racket’’, one on either side of the prostatic ridge. This area, therefore, corresponded to the trigonum vesicae.

In the lumbo-sacral region of the back there was a swelling, about the size of a hen’s egg, which was found to be a meningocele due to spina bifida of the lower lumbar vertebrae. Both feet showed marked talipes equinovarus. No abnormalities were found in any other organ.

MAIN FEATURES

1. The deficiency of the anterior abdominal wall was associated with concomitant defects of exomphalos, huge ectopia vesicae and arrested development of the external genitalia and urethra.

2. The intestine was not obviously differentiated—there being no apparent large intestine. The cloaca was not separated into dorsal and ventral chambers and the anus was imperforate.

8. Spina bifida.

4. There were two remarkable structures which from their situation are suggested to have been abnormally placed nipples.