Recently we had the opportunity of examining a young man with bilateral atrophy of the testes, whose case history may serve to point out the dangers

Transcription

1 From the 2. Medical Clinic, University of Hamburg, Germany (Professor Dr. A. Jores) BILATERAL TESTICULAR ATROPHY AS A RESULT OF SCROTAL HEMATOMA IN THE NEWBORN By Henryk Nowakowski Recently we had the opportunity of examining a young man with bilateral atrophy of the testes, whose case history may serve to point out the dangers of testicular trauma in the newborn. A survey of the extensive literature on male sterility shows, that one must consider not only cryptorchidism but also traumatic injury of the testes as one of the causes of subsequent infertility. CASE RECORD The patient, a 22 years old male (Cl. Fe.), was sent to us because of his abnormal physical and sexual development. There were no abnormalities in the family history. Both parents were quite normal, but his youngest sister suffered from bilateral disorder of the hip joints. The patient himself had had the following diseases: scarlet fever, diphtheria, pertussis, mumps (without orchitis) and swelling of the hilus glands at the age of 3 years. His physical development was normal up to the 14th year. From this time he grew rapidly, axillary and pubic hair developed and the penis was normal for his age. Erections and pollutions occurred but the testicles remained quite small. There was no change in the voice. Fig. 1 shows the clinical condition at the first examination. The patient looks some what infantile, height is 185 cm., the span of his arms 192 cm. and weight 68.9 kg. The abnormally increased length of the upper and low extremities is striking. The pelvis is wide and of a feminine type. The penis is quite normally developed, as is the scrotum, but the testicles scarcely larger than a pea. The axillary and pubic hair is scanty and there is an absence of hair on the face. A prostate could not be palpated. The examination of the seminal fluid showed azoospermia and no fructose could be found in the seminal plasma. The findings indicate a severe tubular and hormonal insufficiency of both testes (Nowakowski, 1954). 1. The investigation was supported by a grant of the Deutsche Forschungsgemeinschaft.

2 Fig. 1. Pat. Cl. Fe., 22 years old with bilateral testicular atrophy, azoospermia and high excretion of gonadotrophins. Eunuchoid symptoms of slight degree. The histological pattern of the atrophie testis is shown in Fig. 2. There are some tubules which contain only Sertoli-cells and no germ-cells and in the intertubular tissue an excessive hyperplasia of the Leydig-cells can be seen. The histological picture is typical of a severe primary atrophy of the testis. In view of the severe nature of the morphological changes prognosis with respect to fertility must be considered poor. The gonadotrophin excretion in the urine amounted to 480 mouse-units in 24 hours (method of Klinefelter, Albright 8e Griswold, 1943). The daily output of 17-keto steroids was about to mg. and of corticoids 1.99 mg. (Heard-Sobel-method). With the exception of a slight decrease in basal metabolism of -12% the internal examination revealed no abnormalities. X-ray examination showed that the epiphyseal fissures of all bones of the extremities were present. On the basis of these clinical findings a diagnosis of bilateral primary testicular atrophy with tubular and hormonal insufficiency of both testes, i. e. of Klinefelter's syndrome (KS) was made. Up to this point, the case presents no unusual features. But the following fact is of particular importance: The mother of the patient told us that during the birth of the boy, by a breech presentation, an enormous hematoma of the

3 Fig. 2. (see Fig. 1). The tubuli concorti contain only Excessive proliferation of Leydig-cells. Typical picture of primary testicular atrophy. 125 X, Azan stain. Histological findings Sertoli-cells. in testis of Cl. Fe. genital region developed. As a result there was a urinary retention of several days' duration. Fortunately we were able to procure the hospital records of 1932, the year of the patient's birth, which confirmed the mother's statement. In view of the unusual dis turbance a photograph of the child was made at the time. Fig. 3 shows that there was a hematoma of the scrotum the size of a fist and an edematous swelling of the penis. In the right inguinal region at the root of the scro- Fig. 3. (a + b). Picture of genital organs of patient Cl. Fe. at birth ( ). in breech position. Extensive hematoma of penis and scrotum. Ulcération of right inguinal region, due to a bulla.

4 - turn there was a nonfistulating ulcération of an about the size of a penny due to a bulla which had broken. The testicles were very hard but not swollen. few days. When the With conservative treatment the hematoma disappeared in a child was dismissed from the clinic the mother's attention was drawn to the probability that there had been a lesion of both testes and that infertility might result. These clinical observation warrant a number of important conclusions. There is no doubt as to the causal relationship between the scrotal hematoma, which occurred at birth, and the subsequent atrophy of the testes. At birth the genital lesions appeared so severe, that testicular damage confirmed later was - suspected at that time. When one slops to consider, that 3 /o of all births are breech presentations, it follows, that this abnormal position represents a par ticular danger for the male gonads and may lead to testicular atrophy and infertility. This is a fact familiar to pathologists (Simmonds, 1910, Schultz, 1913, Oberndorfer, 1931) though clinical monographies and handbooks make no mention of this (e. g. Naujoks, 1934). The most frequent cause of damage intra partum is hemorrhage of the testes, which in exceptional cases may involve the entire organ. Hemorrhages of this kind are seen not only in breech presentations, however, but also under normal conditions. Postmortem examinations performed by Simmonds (1910) have shown, that in l/a of all newborn males, who died at birth there were testicular hemorrhages of varying degree. But even in boys days or weeks after birth a residuum of hemorrhage could be found at autopsy. In most cases, these hemorrhages are reabsorbed without extensive cicatriation. According to Obern dorfer, the testes in exceptional cases may be destroyed and show a picture of severe fibrotic degeneration. The clinician should consider the significance of these findings especially in regard to the etiology of primary testicular atrophy in adults. Since there is a causal relation between birth trauma and testicular damage established, it seemed of interest to conduct catamnestic studies on abnormal birth in other cases of KS, and in patients with bilateral testicular atrophy of unknown etiology. This was done in 14 cases at our clinic (6 with typical KS, 4 with primary (hypergonadotrophic) testicular atrophy of unknown etiology. 4 cases with fibrosis of testes), whose mothers were still alive and could be questioned about the birth. No abnormalities were reported, especially as there were no breech presentations in this material. Whether our case represents a rare complication or is indicative of a pathological relationship deserving more careful attention will have to be established by further systematic studies. SUMMARY Report of a 22 years old man with bilateral testicular atrophy, azoospermia and a high excretion of gonadotrophins in the urine with the clinical condition

5 of Klinefelter's syndrome and eunuchoid symptoms. It is shown, that the gonadal lesion was the result of a severe trauma intra partum due to breech position followed by an extensive scrotal hematoma. REFERENCES Klinefelter, H. E., Albright, F. 8c Griswold, G. F.: J. Clin. Endocrinol. 3, 529, Naujoks, H.: Die Geburtsverletzungen des Kindes. Encke-Verlag, Stuttgart, Nowakowski, H.: Störungen der Keimdrüsenfunktion beim Manne, in: Die Sexualität des Menschen. Ed. by H. Giese, Encke-Verlag, Stuttgart, Oberndorfer, S.: Die inneren männlichen Geschlechtsorgane, in: Hdbch. spez. path. Anat. u. Histologie VI/3, Springer-Verlag, Simmonds, M.: Unnas Festschrift 1, 524, Schultze, W. H.: Hdbch. allg. Path. u. path. Anat. d. Kindesalters. Ed. by H. Brüning and E. Schwalbe, Verlag J. F. Bergmann, 1913.