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Pregnancy and uterine malformations
Pregnancy and uterine malformations A report
of two
THEODORE Eureka,
unusual
W.
cases
LORING,
M.D.
California
Two cases of pregnancy associated with congenital uterine abnormalities are presented. Complications are much greater in the abnormal uterus of dual Miillerian origin (bicornis, septate, etc.) compared to the uterus of single Miillerian origin (didelphys, unicornis, etc.). Abortion, prematurity, breech and transverse presentations, and retained placentas are more common in the malformed uterus. Fertility does not seem to be impaired.
THE P ~0 BLE M o F pregnancy and the congenitally abnormal uterus has existed almost since the time of Eve or, at least, since the time of her daughters. In 1699, Mauriceau reported a case of pregnancy in the rudimentary horn of a uterus bicornis. For many years, the congenital uterine abnormalities have been considered mere anatomic curiosities and assumed to be quite rare. In reality, the incidence of abnormal uterine development is much greater than had been formerly assumed. Hay1 has reported the incidence of all uterine abnormalities to be as high as 12 per cent. The abnormality of the uterus didelphys bicollis, however, is considered to be quite rare and the incidence is not readily determined from the literature. Four cases in 114,243 consecutive obstetric admissions (1 in 28,560) have been reported from the New York Lying-In Hospital while Philpott and ROSS,* in 1954, reported 41 cases in 39,190 admissions (1 in 954) and Zabriskie,3 in 1962, reported 11 cases in 28,539 admissions (1 in 2,683).
Presented by Thirty-ninth Pacific Coast Gvnecoloeical Springs, &itish October 3-7,
Probably the true incidence is somewhere between these extremes. A review of the literature reveals many cases of pregnancies occurring in alternate sides of a uterus didelphys bicollis but only 6 cases of pregnancies occurring simultaneously on each side have been reported.4-g Curiously enough, different gestational ages have been reported in several cases, supporting the concept of superfetation as suggested by Findley.lO Most likely there have been many more cases of twin pregnancies occurring in a uterus didelphys which have never been reported such as the case I am now belatedly reporting. Sterility, abortion, premature labor, complications of labor and delivery, and postpartum complications are frequently associated with congenital abnormalities of the uterus. Strassmanll has suggested that congenital abnormalities of the female reproductive system are among the most frequently overlooked causes of gynecologic and obstetric problems. We are all familiar with the development of the normal female genital tract by the fusion of the Miillerian ducts to form the uterus and the upper vagina. This fusion occurs between the sixth and tenth weeks after conception. Incomplete fusion of the Miillerian system will produce abnormalities of the uterus; the extent of the anomaly is
invitation at the Annual Meeting of the Obstetrical and Societv. Harrison Hot Columbia, Canada, 1972.
Reprint requests: Theodore W. Loring, M.D., 2607 Harris St., Eureka, California 95501.
505
506
Loring
Table I. Jarcho
classification
Uterus simplex (normal) 1. 2. 3. 4. 5. 6. 7.
Uterus Uterus Uterus Uterus Uterus Uterus Uterus
didelphys bicollis (septate vagina) duplex bicollis (vagina simplex) bicornis unicollis (vagina simplex) septus (complete) subseptus (partial) arcuatus (concave fundus) unicornis
Table II. Semmons’ Group 1
2 \
classification Classification
/
Functional uteri of single (uterus bicornis bicollis, uterus bicornis unicornis tary horn)
Mi.illerian origin uterus unicornis, with rudimen-
Functional uteri of dual Mi.illerian origin, with varying degrees of failure of fusion or absorption of the medial septa (uterus bicornis unicollis, uterus septus and subseptus, uterus arcuatus)
directly related to the degree of failure of fusion that occurs. As a result, there has existed confusion in terminology and many classifications have been proposed. The classification as proposed by Jarcho,” based on an anatomic description, is probably the one most accepted and used by clinicians (Table I). A more recent classification by Semmons’” is based on physiologic factors as well as anatomic factors. This excellent and extensive article reviews in detail the over-all problems of 56 personal cases and 500 reported cases of genital tract anomalies. He divides the anomalies into two groups (Table II ) . The following reports illustrate a case of a uterus bicornis unicollis corrected by operation with 4 subsequent pregnancies and the the other case is that of a twin pregnancy occurring in a uterus didelphys bicollis. Case
reports
Case 1. Mrs.
B. T., a white woman, aged 30 years, was first seen in December, 1953, complaining of the failure to carry a pregnancy to term. Her past history revealed that her first pregnancy occurred in 1948. At about 12 weeks of gestation, a laparotomy was performed because of pelvic pain and diagnosis of an ectopic pregnancy. The uterus was found to be abnormal in
shape and was described as being “heart shaped.” At about 18 weeks, she spontaneously aborted twins. During the next 6 years, she had 7 firsttrimester abortions, the last in 1954. A curettage, on February 24, 1954, showed a cavity suggestive of a uterus bicornis unicollis. This \cas later confirmed by hysterosalpingography. On May 2 4, 1954, a Strassman procedure was performed. The left uterine cavity was larger than the right; postoperative hysterosalpingography however, showed a fairly normal uterine cavity. She became pregnant again late in 1955, and was scheduled for delivery by cesarean section but developed contractions at 38 weeks, so an immediate cesarean section was performed and a normal 5 pound, 10 ounce male infant was delivered. Exploration of the uterine cavity with a normal-appearing myometrial wall at the site of the unification. The postpartum cfjurse was uneventful. She again became pregnant in 1957, and had a normal prenatal course, with delivery at 38 weeks of a normal 5 pound, 13 ounce male infant. Tubal ligation was advised but this was rejected because of religious reasons. Another pregnancy occurred in 1959, and was carried to term without incident, with delivery of another male infant, 5 pounds, 6 ounces. The fourth cesarean section was performed in 1961, after premature labor at 35 rceeks. A 4, pound, 10 ounce female infant was delivered and, at this time, at her request, tubal ligation was done. The uterine wall, at the time of the last section, appeared essentially as at the first. Case 2. Mrs. B. G., a 19-year-old white woman, was first seen on June 2, 1966. The last menstrual period was February 20, 1966; the general physical examination was not remarkable, except for the pelvic finding of a uterus didelphys bicollis. The right uterus was enlarged and suggestive of an early pregnancy. Pelvic measurements were within normal limits as were all iaboratory studies. The past history was not remarkable. Menses began at age 13 years; there were no menstrual problems and she was not aware of the congenital abnormality but did complain of dyspareunia on occasions. The prenatal course was uneventful until she developed vaginal bleeding between the twelfth and fourteenth weeks. This seemed to be coming from the left cervix and responded to progestogen therapy. On December 1, 1966, at 4.0 weeks’ gestation, she went into spontaneous labor. After
Volume 116 Number
4
about
6 hours
Pregnancy
of labor,
without
significant
pro-
gress, x-ray pelvimetry was done which showed a large fetus in a frank breech presentation. A low transverse cesarean section was done on the right uterus with delivery of a 10 pound, 2% ounce male infant. Exploration of the pelvis revealed the typical findings of a uterus didelphys bicollis, with no other abnormalities. The left uterus was enlarged to the size of a 4 months’ pregnancy. The postpartum course was not remarkable. On August -4, 1967, she was again seen with a pregnancy in the right uterus. The last menstrual period was May 10, 1567, the expected date of confinement was February 17, 1968. The pregnancy progressed normally but by about the twenty-fourth week it was apparent that both uteri were pregnant. At 36 weeks, an anteroposterior supine x-ray examination of the abdomen confirmed the diagnosis of twins. The fetus on the right was larger than that on the left and was in a transverse presentation. On January 26, 1968, at 38 weeks, she developed contractions so an imtnediate cesarean section was performed under spinal anesthesia, the ahdomen being entered through a midline incision. To accommodate both pregnant uteri in the pelvis, the right uterus had rotated 90° to the left and was completely posterior beneath the left uterus. The vessels of the ovarian and broad ligaments were markedly engorged, with some vessels, especially on the right, measuring 2 to 3 cm. in diameter. The left uterus blocked any approach to the right uterus so the bladder reflection on the left was freed from the lower segment; the uterus was entered through a transverse incision and a 6
and
uterine
malformations
507
pound female infant was extracted. Bleeding from the uterine incision was controlled by Pennington clamps. The left uterus failed to contract down but after it was emptied it could be displaced laterally. The torsion of the right uterus could then be corrected, the bladder peritoneum reflected, and the uterus entered, and a 6 pound, 11 ounce male infant was delivered. Both infants cried immediately and were normal in all respects. By this time the left placenta had failed to separate. One cubic centimeter of oxytocin was injected into the right uterine myometrium and then both sides contracted. The placentas separated and were delivered at about the same time. The closure of the uterine incisions was accomplished without difficulty. Blood loss during the procedure was estimated at 700 to 800 cc. and was replaced by 1,000 C.C. of whole blood during and after operation. The postoperative and postpartum courses were not remarkable. She was examined occasionally during the next 2 years for minor gynecologic complaints. On July 24, 1970, she was referred by the Mental Health Clinic psychiatrist and requested a therapeutic abortion and sterilization. During the previous year she had been ingesting drugs, including lysergic acid diethylamide, and had developed psychiatric problems requiring hospitalization. Pelvic examination confirmed an eighteen to twenty week pregnancy in the right uterus. On July 29, 1970, the right uterus was emptied by hysterotomy and bilateral segments removed from both tubes. Her postoperative course was again uneventful.
REFERENCES
1. Hay, D.: J. Obstet. Gynaecol. Br. Commonw. 68: 361, 1961. 2. Philpott, N. W., and Ross, J. E.: AM. J. OBSTET. GYNECOL. 68: 285, 1954. 3. Zabriskie, J. R.: West. J. Surg. 70: 293, 1962. 4. Rowlett, W. M.: J. Fla. Med. Assoc. 12: 5, 1925. 5. Brody, S.: AM. J. OBSTET. GYNECOL. 67: 161, 1954. 6. Colaco, L.: J. Obstet. Gynaecol. Br. Emp. 56: 1018, 1949.
Discussion DR. ton.
DONALD M. MCINTYRE, Dr. Loring has reported
Seattle, to us
Washingthe wide
7. 8. 9. 10. 11. 12. 13.
Dorgan, L. T., and Clarke, 0. E.: AM. J. OBSTET. GYNEC:OL. 72: 663, 1956. Sandoval, Z.: Ginecol. Obstet. Mex. 22: 1967. Moncure, P. St. L.: Va. Med. Mon. 66: 593, 1939. Findley, P.: AM. J. OBSTET. GYNECOL. 12: 318, 1926. Strassman, E. 0.: Fertil. Steril. 17: 165, 1966. Jarcho, J.: Am. J. Surg. 71: 106, 1946. Semmons, J. P.: Obstet. Gynecol. 19: 328, 1962.
variations hicollis, Perhaps
of the incidence of uterus didelphys ranging from 1 in 28,560 to 1 in 954. an explanation could he that different
508
Loring
institutions may have obstetric and gynecologic problems referred more often, thus changing the population from which they report. For example, Jones had an interest in the problem of uterine anomalies and their impact on obstetric complications. He had many pregnant patients with uterine anomalies referred; thus his incidence would naturally be much higher than that of other obstetric services. Dr. Loring mentioned 2 very excellent papers by Jarcho and Semmons. Both of these physicians had an unusually wide experience in personally dealing with the problem of gynecologic and obstetric complications of uterine anomalies and have written excellent papers. Jones stated that in no patient was there a fetal pelvic disproportion caused by opposite-side blockage of the birth canal by the nonpregnant uterus. Jones antedated Semmons in his physiologic classification of uterine anomalies and believed that obstetric complications of labor are related to the degree of the Miillerian fusion failure. He also felt that breech presentation in the uterus didelphys bicollis was a strong indication for cesarean section unless labor was progressing very satisfactorily. He affirmed that the uterus and cervix developed from one Miillerian duct are much less effective in labor and often lead to inertia, hemorrhage, and, rarely, uterine rupture. Dr. Loring’s first case, where he performed a Strassman procedure, was well handled. If the patient had consulted Dr. Loring earlier, there may have been less fetal waste. When does metroplasty become indicated? Use of salpingogram in the nonpregnant state and/or intrauterine examination at the time of a late abortion or delivery may lead to earlier evaluation and possibly need for surgical treatment. However, not all patients require surgical treatment. I have cared for a woman in her tenth pregnancy with a uterus bicornus unicollis. All 10 of her pregnancies resulted in term-size infants. There were no abortions. Another patient’s first pregnancy was a previable premature baby. With the next 2 pregnancies, there were successive increases in size and maturity of the infants. They were premature but easily viable, healthy infants. It boils down to the fact that some people definitely need metroplasty, and some may not. Each situation must be carefully studied. Dr. Loring’s second patient’s obstetric management was extremely well handled. I believe
June 15, 1973 Am. J. Obstet. Gynecol.
that he is correct when he says that there are probably a number of unreported twin pregnancies with a uterus didelphys; however, I personally have never seen one. I should like to pose a question regarding the type of uterine incision in cesarean section in a patient with a common cervix and 2 uteri. Would he recommend a classical cesarean section instead of a lower uterine transverse incision, with the thought that a later pregnancy might occur in the opposite side and a cesarean section might not he necessary? In therapeutic and voluntary abortions, there are complications from uterine anomalies. In one patient, with a complete septum, perforation occurred in the midline during dilatation. The left uterus was entered later and suction evacuation was followed by curettage. It was revealed that them was no trophoblastic tissue, merely decidua. During the same operations, the right uterus was found to contain the early pregnancy which was removed. In this patient, separate uteri could not be palpated by bimanual examination. I believe, but cannot prove, that there are rare patients in early pregnancy with uteri which may be felt to be in an anterior position and normal as to expected size when measured by uterine sound but which have a sacculation posteriorly which neither the rectal finger nor the abdominal hand can palpate. The pregnancy seems to be located in this high sacculation of the uterus, perhaps an explanation for reports of patients who have had early curettage without removal of the pregnancy. I congratulate Dr. Loring on his good presentation, and the excellent management of the 2 patients presented. DR. CHARLES F. LANGMADE, Pasadena, California. We have a series of 14 cases of vaginal agenesis, and I think 2 of them are worthy of mention here at this time. The first patient was born with no vagina and no rectum and, on a subsequent pull through operation to restore her rectal continuity, a pelvic abscess developed. At age 19, we reconstructed the vagina and, on exploration, she had dense pelvic adhesions, the right tube was completely destroyed, and the left tube was also completely destroyed but about 2 cm. remained and we left that. The uterus was bicomuate. She subsequently conceived and about 3 months ago was delivered of a healthy baby. The second patient was seen at the time she was having an appendectomy at the age of about 12. The general surgeon had not done a
Volume 116 Number 4
pelvic exmaination. She had a hemoperitoneum and was menstruating into the peritoneal cavity. A perineal sinus was made through which she menstruated for about 7 years and then vaginal reconstruction was done. At the time of operation, the tubes were almost completely destroyed; she conceived but, because of severe cardiac complications, the pregnancy was terminated. I am sure we would not consider these two patients with their congenital malformations to ever be able to conceive. We all learned long ago never to tell a girl she can’t conceive but I think these 2 patients sort of carry it to the “nth” degree. DR. AUSTIN W. LEA, San Rafael, California. Dr. Loring’s paper and Dr. McIntyre’s comments were of great value in understanding pregnancy in this anomaly. Dr. Loring referred to some of the complications. I would like to point out one pitfall that those of us less astute than Dr. Loring might run into. A youngster who skates for the Ice Follies had a pregnancy in the right horn of the uterus which she carried to term; it was well verified that it was term, although the male child weighed only 3 pounds, 12 ounces. A few years later, she conceived in the left horn. This time the uterus grew quite rapidly and she was delivered of a female child at term weighing 6 pounds, 14 ounces. Heavy postpartum bleeding required replacement. She unexpectedly conceived a few years later. She did not request abortion but opted for term tubal ligation. The uterus again grew rapidly and she was delivered about 3 weeks prior to term of another male infant from the right horn weighing 4 pounds, 8 ounces. This is not to intimate that male infants come from the right and female infants from the left. In order to avoid another bleeding problem, immediate manual removal of the placenta was done. As it was swept off and being removed, a bulge from the left horn was felt. This gave quite a cremasteric reflex but, of course, as this bag contained another female child which had its own blood supply, an incidental extraction was done. DR. JESSE A. RUST, San Diego, California. Two years ago, we had some difficulty with cesarean section, near term, with a patient who had previously had a Strassman operation. Because of intrauterine synechia, it was difficult to get the baby out and also to remove the placenta. Recently, we encountered a second case; this patient had a didelphic uterus. The junction of the 2 uteri was just above the external OS. This patient had lost 3 babies at 24 to 26 weeks. We
Pregnancy
and
uterine
malformations
509
took care of her with the third and found the placenta almost totally infarcted. We operated upon her 4 months after delivery and, with synechia in mind, put in a Dalkon shield. At about 4 months postoperatively, she has a bicomuate uterus and perhaps an incompetent internal cervical OS. The question I have regarding this is, in reviewing the literature, did Dr. Loring run into any postoperative complications resulting from intrauterine synechia? DR. WALTER S. KEIFER, Seattle, Washington. Dr. Lea brought up the pitfalls. I have found those pitfalls in a patient with a uterus didelphys. She was studied before pregnancy. She could become pregnant on the right side, but the left was only a rudimentary horn, a very natural and convenient contraceptive. We delivered 2 children for her. At the third delivery, the vaginal septum disappeared. She visited her lawyer and has been irate ever since. DR. RICHARD N. BOLTON, Portland, Oregon. This paper reminds me of a patient who has plagued me off and on for the last 8 years. She aborted her first 3 pregnancies, and a subsequent hysterosalpingogram demonstrated a uterine septum which was corrected by a Strassmann metroplasty. She had been informed that this operation would necessitate delivery by cesarean section. However, she went into premature labor and arrived at the hospital dilated to 6 cm. She subsequently was delivered vaginally. Exploration of the uterus at this time revealed a strong scar. This patient has now been delivered of a total of 3 babies vaginally, all of them somewhat premature, and I do believe that she has an incompetent cervix. There was another similar patient of my late associate, Dr. Duncan R. Neilson, who had a uterine septum removed by the same operation and returned to her family physician for subsequent care. Not clearly understanding that she was to be delivered by cesarean section, she was allowed to go into spontaneous labor and ultimately was delivered of 4 babies vaginally with no difficulty. It would seem that at least occasionally this scar maintains itself in a very solid and stable state. By the way, I might mention that there are approximately 12 wives in our group whose first names are Betty. It don’t believe that any of these women have given clinical evidence of a uterine septum, and I would feel, on the basis of this rather short series, that the first name of
510
Loring
Betty can be excluded as a causative factor. DR. LORING (Closing). Synechiae were described by Dr. Rust; how you avoid them I do not know. I was fortunate in my series of one not to have any. It can be a problem. Simmonds described the fetal wastage in his Group 1 as 35 per cent and his Group 2 as 49.3 per cent which illustrates a definite clinical difference between the 2 groups. He also described a very high incidence in breech presentation, approsimately 35 per cent. There is one point Dr. Lea mentioned as to astuteness; I would argue with him but he always wins the arguments.
In reviewing the literature on the uterus didelphys, the right horn predominates in conception over the left horn, approximately .1 to one. Sometime in the future one of us might find out whether these people were right handed or left handed. With regard to the vaginal septum, 1 did offer surgical correction to the couple after the first cesarean, again, with the thoughts that she might get pregnant on the left side and I might allow her to labor. However, her husband said, “No, doctor, I am very, very satisfied with lvhat she’s got. Twice as much as anyone else.”
of two
THEODORE Eureka,
unusual
W.
cases
LORING,
M.D.
California
Two cases of pregnancy associated with congenital uterine abnormalities are presented. Complications are much greater in the abnormal uterus of dual Miillerian origin (bicornis, septate, etc.) compared to the uterus of single Miillerian origin (didelphys, unicornis, etc.). Abortion, prematurity, breech and transverse presentations, and retained placentas are more common in the malformed uterus. Fertility does not seem to be impaired.
THE P ~0 BLE M o F pregnancy and the congenitally abnormal uterus has existed almost since the time of Eve or, at least, since the time of her daughters. In 1699, Mauriceau reported a case of pregnancy in the rudimentary horn of a uterus bicornis. For many years, the congenital uterine abnormalities have been considered mere anatomic curiosities and assumed to be quite rare. In reality, the incidence of abnormal uterine development is much greater than had been formerly assumed. Hay1 has reported the incidence of all uterine abnormalities to be as high as 12 per cent. The abnormality of the uterus didelphys bicollis, however, is considered to be quite rare and the incidence is not readily determined from the literature. Four cases in 114,243 consecutive obstetric admissions (1 in 28,560) have been reported from the New York Lying-In Hospital while Philpott and ROSS,* in 1954, reported 41 cases in 39,190 admissions (1 in 954) and Zabriskie,3 in 1962, reported 11 cases in 28,539 admissions (1 in 2,683).
Presented by Thirty-ninth Pacific Coast Gvnecoloeical Springs, &itish October 3-7,
Probably the true incidence is somewhere between these extremes. A review of the literature reveals many cases of pregnancies occurring in alternate sides of a uterus didelphys bicollis but only 6 cases of pregnancies occurring simultaneously on each side have been reported.4-g Curiously enough, different gestational ages have been reported in several cases, supporting the concept of superfetation as suggested by Findley.lO Most likely there have been many more cases of twin pregnancies occurring in a uterus didelphys which have never been reported such as the case I am now belatedly reporting. Sterility, abortion, premature labor, complications of labor and delivery, and postpartum complications are frequently associated with congenital abnormalities of the uterus. Strassmanll has suggested that congenital abnormalities of the female reproductive system are among the most frequently overlooked causes of gynecologic and obstetric problems. We are all familiar with the development of the normal female genital tract by the fusion of the Miillerian ducts to form the uterus and the upper vagina. This fusion occurs between the sixth and tenth weeks after conception. Incomplete fusion of the Miillerian system will produce abnormalities of the uterus; the extent of the anomaly is
invitation at the Annual Meeting of the Obstetrical and Societv. Harrison Hot Columbia, Canada, 1972.
Reprint requests: Theodore W. Loring, M.D., 2607 Harris St., Eureka, California 95501.
505
506
Loring
Table I. Jarcho
classification
Uterus simplex (normal) 1. 2. 3. 4. 5. 6. 7.
Uterus Uterus Uterus Uterus Uterus Uterus Uterus
didelphys bicollis (septate vagina) duplex bicollis (vagina simplex) bicornis unicollis (vagina simplex) septus (complete) subseptus (partial) arcuatus (concave fundus) unicornis
Table II. Semmons’ Group 1
2 \
classification Classification
/
Functional uteri of single (uterus bicornis bicollis, uterus bicornis unicornis tary horn)
Mi.illerian origin uterus unicornis, with rudimen-
Functional uteri of dual Mi.illerian origin, with varying degrees of failure of fusion or absorption of the medial septa (uterus bicornis unicollis, uterus septus and subseptus, uterus arcuatus)
directly related to the degree of failure of fusion that occurs. As a result, there has existed confusion in terminology and many classifications have been proposed. The classification as proposed by Jarcho,” based on an anatomic description, is probably the one most accepted and used by clinicians (Table I). A more recent classification by Semmons’” is based on physiologic factors as well as anatomic factors. This excellent and extensive article reviews in detail the over-all problems of 56 personal cases and 500 reported cases of genital tract anomalies. He divides the anomalies into two groups (Table II ) . The following reports illustrate a case of a uterus bicornis unicollis corrected by operation with 4 subsequent pregnancies and the the other case is that of a twin pregnancy occurring in a uterus didelphys bicollis. Case
reports
Case 1. Mrs.
B. T., a white woman, aged 30 years, was first seen in December, 1953, complaining of the failure to carry a pregnancy to term. Her past history revealed that her first pregnancy occurred in 1948. At about 12 weeks of gestation, a laparotomy was performed because of pelvic pain and diagnosis of an ectopic pregnancy. The uterus was found to be abnormal in
shape and was described as being “heart shaped.” At about 18 weeks, she spontaneously aborted twins. During the next 6 years, she had 7 firsttrimester abortions, the last in 1954. A curettage, on February 24, 1954, showed a cavity suggestive of a uterus bicornis unicollis. This \cas later confirmed by hysterosalpingography. On May 2 4, 1954, a Strassman procedure was performed. The left uterine cavity was larger than the right; postoperative hysterosalpingography however, showed a fairly normal uterine cavity. She became pregnant again late in 1955, and was scheduled for delivery by cesarean section but developed contractions at 38 weeks, so an immediate cesarean section was performed and a normal 5 pound, 10 ounce male infant was delivered. Exploration of the uterine cavity with a normal-appearing myometrial wall at the site of the unification. The postpartum cfjurse was uneventful. She again became pregnant in 1957, and had a normal prenatal course, with delivery at 38 weeks of a normal 5 pound, 13 ounce male infant. Tubal ligation was advised but this was rejected because of religious reasons. Another pregnancy occurred in 1959, and was carried to term without incident, with delivery of another male infant, 5 pounds, 6 ounces. The fourth cesarean section was performed in 1961, after premature labor at 35 rceeks. A 4, pound, 10 ounce female infant was delivered and, at this time, at her request, tubal ligation was done. The uterine wall, at the time of the last section, appeared essentially as at the first. Case 2. Mrs. B. G., a 19-year-old white woman, was first seen on June 2, 1966. The last menstrual period was February 20, 1966; the general physical examination was not remarkable, except for the pelvic finding of a uterus didelphys bicollis. The right uterus was enlarged and suggestive of an early pregnancy. Pelvic measurements were within normal limits as were all iaboratory studies. The past history was not remarkable. Menses began at age 13 years; there were no menstrual problems and she was not aware of the congenital abnormality but did complain of dyspareunia on occasions. The prenatal course was uneventful until she developed vaginal bleeding between the twelfth and fourteenth weeks. This seemed to be coming from the left cervix and responded to progestogen therapy. On December 1, 1966, at 4.0 weeks’ gestation, she went into spontaneous labor. After
Volume 116 Number
4
about
6 hours
Pregnancy
of labor,
without
significant
pro-
gress, x-ray pelvimetry was done which showed a large fetus in a frank breech presentation. A low transverse cesarean section was done on the right uterus with delivery of a 10 pound, 2% ounce male infant. Exploration of the pelvis revealed the typical findings of a uterus didelphys bicollis, with no other abnormalities. The left uterus was enlarged to the size of a 4 months’ pregnancy. The postpartum course was not remarkable. On August -4, 1967, she was again seen with a pregnancy in the right uterus. The last menstrual period was May 10, 1567, the expected date of confinement was February 17, 1968. The pregnancy progressed normally but by about the twenty-fourth week it was apparent that both uteri were pregnant. At 36 weeks, an anteroposterior supine x-ray examination of the abdomen confirmed the diagnosis of twins. The fetus on the right was larger than that on the left and was in a transverse presentation. On January 26, 1968, at 38 weeks, she developed contractions so an imtnediate cesarean section was performed under spinal anesthesia, the ahdomen being entered through a midline incision. To accommodate both pregnant uteri in the pelvis, the right uterus had rotated 90° to the left and was completely posterior beneath the left uterus. The vessels of the ovarian and broad ligaments were markedly engorged, with some vessels, especially on the right, measuring 2 to 3 cm. in diameter. The left uterus blocked any approach to the right uterus so the bladder reflection on the left was freed from the lower segment; the uterus was entered through a transverse incision and a 6
and
uterine
malformations
507
pound female infant was extracted. Bleeding from the uterine incision was controlled by Pennington clamps. The left uterus failed to contract down but after it was emptied it could be displaced laterally. The torsion of the right uterus could then be corrected, the bladder peritoneum reflected, and the uterus entered, and a 6 pound, 11 ounce male infant was delivered. Both infants cried immediately and were normal in all respects. By this time the left placenta had failed to separate. One cubic centimeter of oxytocin was injected into the right uterine myometrium and then both sides contracted. The placentas separated and were delivered at about the same time. The closure of the uterine incisions was accomplished without difficulty. Blood loss during the procedure was estimated at 700 to 800 cc. and was replaced by 1,000 C.C. of whole blood during and after operation. The postoperative and postpartum courses were not remarkable. She was examined occasionally during the next 2 years for minor gynecologic complaints. On July 24, 1970, she was referred by the Mental Health Clinic psychiatrist and requested a therapeutic abortion and sterilization. During the previous year she had been ingesting drugs, including lysergic acid diethylamide, and had developed psychiatric problems requiring hospitalization. Pelvic examination confirmed an eighteen to twenty week pregnancy in the right uterus. On July 29, 1970, the right uterus was emptied by hysterotomy and bilateral segments removed from both tubes. Her postoperative course was again uneventful.
REFERENCES
1. Hay, D.: J. Obstet. Gynaecol. Br. Commonw. 68: 361, 1961. 2. Philpott, N. W., and Ross, J. E.: AM. J. OBSTET. GYNECOL. 68: 285, 1954. 3. Zabriskie, J. R.: West. J. Surg. 70: 293, 1962. 4. Rowlett, W. M.: J. Fla. Med. Assoc. 12: 5, 1925. 5. Brody, S.: AM. J. OBSTET. GYNECOL. 67: 161, 1954. 6. Colaco, L.: J. Obstet. Gynaecol. Br. Emp. 56: 1018, 1949.
Discussion DR. ton.
DONALD M. MCINTYRE, Dr. Loring has reported
Seattle, to us
Washingthe wide
7. 8. 9. 10. 11. 12. 13.
Dorgan, L. T., and Clarke, 0. E.: AM. J. OBSTET. GYNEC:OL. 72: 663, 1956. Sandoval, Z.: Ginecol. Obstet. Mex. 22: 1967. Moncure, P. St. L.: Va. Med. Mon. 66: 593, 1939. Findley, P.: AM. J. OBSTET. GYNECOL. 12: 318, 1926. Strassman, E. 0.: Fertil. Steril. 17: 165, 1966. Jarcho, J.: Am. J. Surg. 71: 106, 1946. Semmons, J. P.: Obstet. Gynecol. 19: 328, 1962.
variations hicollis, Perhaps
of the incidence of uterus didelphys ranging from 1 in 28,560 to 1 in 954. an explanation could he that different
508
Loring
institutions may have obstetric and gynecologic problems referred more often, thus changing the population from which they report. For example, Jones had an interest in the problem of uterine anomalies and their impact on obstetric complications. He had many pregnant patients with uterine anomalies referred; thus his incidence would naturally be much higher than that of other obstetric services. Dr. Loring mentioned 2 very excellent papers by Jarcho and Semmons. Both of these physicians had an unusually wide experience in personally dealing with the problem of gynecologic and obstetric complications of uterine anomalies and have written excellent papers. Jones stated that in no patient was there a fetal pelvic disproportion caused by opposite-side blockage of the birth canal by the nonpregnant uterus. Jones antedated Semmons in his physiologic classification of uterine anomalies and believed that obstetric complications of labor are related to the degree of the Miillerian fusion failure. He also felt that breech presentation in the uterus didelphys bicollis was a strong indication for cesarean section unless labor was progressing very satisfactorily. He affirmed that the uterus and cervix developed from one Miillerian duct are much less effective in labor and often lead to inertia, hemorrhage, and, rarely, uterine rupture. Dr. Loring’s first case, where he performed a Strassman procedure, was well handled. If the patient had consulted Dr. Loring earlier, there may have been less fetal waste. When does metroplasty become indicated? Use of salpingogram in the nonpregnant state and/or intrauterine examination at the time of a late abortion or delivery may lead to earlier evaluation and possibly need for surgical treatment. However, not all patients require surgical treatment. I have cared for a woman in her tenth pregnancy with a uterus bicornus unicollis. All 10 of her pregnancies resulted in term-size infants. There were no abortions. Another patient’s first pregnancy was a previable premature baby. With the next 2 pregnancies, there were successive increases in size and maturity of the infants. They were premature but easily viable, healthy infants. It boils down to the fact that some people definitely need metroplasty, and some may not. Each situation must be carefully studied. Dr. Loring’s second patient’s obstetric management was extremely well handled. I believe
June 15, 1973 Am. J. Obstet. Gynecol.
that he is correct when he says that there are probably a number of unreported twin pregnancies with a uterus didelphys; however, I personally have never seen one. I should like to pose a question regarding the type of uterine incision in cesarean section in a patient with a common cervix and 2 uteri. Would he recommend a classical cesarean section instead of a lower uterine transverse incision, with the thought that a later pregnancy might occur in the opposite side and a cesarean section might not he necessary? In therapeutic and voluntary abortions, there are complications from uterine anomalies. In one patient, with a complete septum, perforation occurred in the midline during dilatation. The left uterus was entered later and suction evacuation was followed by curettage. It was revealed that them was no trophoblastic tissue, merely decidua. During the same operations, the right uterus was found to contain the early pregnancy which was removed. In this patient, separate uteri could not be palpated by bimanual examination. I believe, but cannot prove, that there are rare patients in early pregnancy with uteri which may be felt to be in an anterior position and normal as to expected size when measured by uterine sound but which have a sacculation posteriorly which neither the rectal finger nor the abdominal hand can palpate. The pregnancy seems to be located in this high sacculation of the uterus, perhaps an explanation for reports of patients who have had early curettage without removal of the pregnancy. I congratulate Dr. Loring on his good presentation, and the excellent management of the 2 patients presented. DR. CHARLES F. LANGMADE, Pasadena, California. We have a series of 14 cases of vaginal agenesis, and I think 2 of them are worthy of mention here at this time. The first patient was born with no vagina and no rectum and, on a subsequent pull through operation to restore her rectal continuity, a pelvic abscess developed. At age 19, we reconstructed the vagina and, on exploration, she had dense pelvic adhesions, the right tube was completely destroyed, and the left tube was also completely destroyed but about 2 cm. remained and we left that. The uterus was bicomuate. She subsequently conceived and about 3 months ago was delivered of a healthy baby. The second patient was seen at the time she was having an appendectomy at the age of about 12. The general surgeon had not done a
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pelvic exmaination. She had a hemoperitoneum and was menstruating into the peritoneal cavity. A perineal sinus was made through which she menstruated for about 7 years and then vaginal reconstruction was done. At the time of operation, the tubes were almost completely destroyed; she conceived but, because of severe cardiac complications, the pregnancy was terminated. I am sure we would not consider these two patients with their congenital malformations to ever be able to conceive. We all learned long ago never to tell a girl she can’t conceive but I think these 2 patients sort of carry it to the “nth” degree. DR. AUSTIN W. LEA, San Rafael, California. Dr. Loring’s paper and Dr. McIntyre’s comments were of great value in understanding pregnancy in this anomaly. Dr. Loring referred to some of the complications. I would like to point out one pitfall that those of us less astute than Dr. Loring might run into. A youngster who skates for the Ice Follies had a pregnancy in the right horn of the uterus which she carried to term; it was well verified that it was term, although the male child weighed only 3 pounds, 12 ounces. A few years later, she conceived in the left horn. This time the uterus grew quite rapidly and she was delivered of a female child at term weighing 6 pounds, 14 ounces. Heavy postpartum bleeding required replacement. She unexpectedly conceived a few years later. She did not request abortion but opted for term tubal ligation. The uterus again grew rapidly and she was delivered about 3 weeks prior to term of another male infant from the right horn weighing 4 pounds, 8 ounces. This is not to intimate that male infants come from the right and female infants from the left. In order to avoid another bleeding problem, immediate manual removal of the placenta was done. As it was swept off and being removed, a bulge from the left horn was felt. This gave quite a cremasteric reflex but, of course, as this bag contained another female child which had its own blood supply, an incidental extraction was done. DR. JESSE A. RUST, San Diego, California. Two years ago, we had some difficulty with cesarean section, near term, with a patient who had previously had a Strassman operation. Because of intrauterine synechia, it was difficult to get the baby out and also to remove the placenta. Recently, we encountered a second case; this patient had a didelphic uterus. The junction of the 2 uteri was just above the external OS. This patient had lost 3 babies at 24 to 26 weeks. We
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took care of her with the third and found the placenta almost totally infarcted. We operated upon her 4 months after delivery and, with synechia in mind, put in a Dalkon shield. At about 4 months postoperatively, she has a bicomuate uterus and perhaps an incompetent internal cervical OS. The question I have regarding this is, in reviewing the literature, did Dr. Loring run into any postoperative complications resulting from intrauterine synechia? DR. WALTER S. KEIFER, Seattle, Washington. Dr. Lea brought up the pitfalls. I have found those pitfalls in a patient with a uterus didelphys. She was studied before pregnancy. She could become pregnant on the right side, but the left was only a rudimentary horn, a very natural and convenient contraceptive. We delivered 2 children for her. At the third delivery, the vaginal septum disappeared. She visited her lawyer and has been irate ever since. DR. RICHARD N. BOLTON, Portland, Oregon. This paper reminds me of a patient who has plagued me off and on for the last 8 years. She aborted her first 3 pregnancies, and a subsequent hysterosalpingogram demonstrated a uterine septum which was corrected by a Strassmann metroplasty. She had been informed that this operation would necessitate delivery by cesarean section. However, she went into premature labor and arrived at the hospital dilated to 6 cm. She subsequently was delivered vaginally. Exploration of the uterus at this time revealed a strong scar. This patient has now been delivered of a total of 3 babies vaginally, all of them somewhat premature, and I do believe that she has an incompetent cervix. There was another similar patient of my late associate, Dr. Duncan R. Neilson, who had a uterine septum removed by the same operation and returned to her family physician for subsequent care. Not clearly understanding that she was to be delivered by cesarean section, she was allowed to go into spontaneous labor and ultimately was delivered of 4 babies vaginally with no difficulty. It would seem that at least occasionally this scar maintains itself in a very solid and stable state. By the way, I might mention that there are approximately 12 wives in our group whose first names are Betty. It don’t believe that any of these women have given clinical evidence of a uterine septum, and I would feel, on the basis of this rather short series, that the first name of
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Betty can be excluded as a causative factor. DR. LORING (Closing). Synechiae were described by Dr. Rust; how you avoid them I do not know. I was fortunate in my series of one not to have any. It can be a problem. Simmonds described the fetal wastage in his Group 1 as 35 per cent and his Group 2 as 49.3 per cent which illustrates a definite clinical difference between the 2 groups. He also described a very high incidence in breech presentation, approsimately 35 per cent. There is one point Dr. Lea mentioned as to astuteness; I would argue with him but he always wins the arguments.
In reviewing the literature on the uterus didelphys, the right horn predominates in conception over the left horn, approximately .1 to one. Sometime in the future one of us might find out whether these people were right handed or left handed. With regard to the vaginal septum, 1 did offer surgical correction to the couple after the first cesarean, again, with the thoughts that she might get pregnant on the left side and I might allow her to labor. However, her husband said, “No, doctor, I am very, very satisfied with lvhat she’s got. Twice as much as anyone else.”