CASE REPORT
Pseudotumors of the umbilical cord and fetal membranes INGOCLAUSEN AND STENGROVETHOMSEN From the Department of Obstetrics and Gynecology, Odense University Hospital, Odense, Denmark
Acta Obstet Gynecol Scand 1992; 71: 148-1.50 Antenatally diagnosed pseudotumors, i.e. non-neoplastic tumors, of the umbilical cord aqd fetal membranes may when scanned by ultrasound have an appearance leading to misdiagnosis. In the present cases. a hematoma in the fetal membranes was interpreted as a chorioangioma, and a cystic mass inside the umbilical cord caused by degeneration of Wharton’s jelly was regarded as an omphalomesenteric or allantoic cyst. The two cases are presented. Color Doppler evaluation seems advisable. Key words: pregnancy complications; umbilical cord; hematoma; cyst; pseudotumor Submitted July 15, 1991 Accepted October 14, 1991
Tumors of the umbilical cord or fetal membranes are uncommon findings that can be discovered prenatally by ultasound scanning. The nature of such tumors cannot always be determined accurately before delivery. Although they can be regarded as benign, they may represent a great hazard to the fetus. No malignant tumor of the two structures has ever been published (1). We describe 2 cases of so-called pseudotumors, i.e. non-neoplastic tumors, of the umbilical cord and fetal membranes.
Case reports Case I
A 25-year-old primipara was admitted because of vaginal bleeding in the 28th week of pregnancy. She experienced no pain and the uterus was relaxed and not tender. A normal CTG was recorded. A t the initial ultrasound scanning, there were no signs of fetal defects, intra-uterine growth retardation o r hydrops, and a normal anterior wall placenta was described. O n account of continuous hemorrhage and irritability of the uterus, a new ultrasound examination was performed 6 h later. Behind the internal Acta Obstet Gynecol Scand 71 (1992)
cervical 0s an echo-dense, massive tumor measuring 8 X 9 cm was seen (Fig. 1). There was no demonstrable connection to the fetus o r umbilical cord. After a week the process displayed small echo-free areas of a supposedly vascular nature and, therefore, it was thought to be a chorioangioma and not a fibroma of the uterine wall. Color Doppler imaging was not undertaken. Apart from vaginal spotting, the pregnancy took an uncomplicated course. The tumor did not change its appearance on repeated ultrasound scanning, and fetal growth was normal. A healthy male infant weighing 2,500 g was born at cesarean section in week 35 of pregnancy. The placenta was normal. Located to the fetal membranes, a clearly demarcated reddish-brown jelly-like tumor was found and microscopically shown t o be a coagulum. The postoperative course was uneventful. Case
I1
A 25-year-old primipara was examined by ultrasound scanning in week 22 of her hitherto uncomplicated pregnancy. A cystic mass with a diameter of 3 cm inside the umbilical cord and surrounded by thickened Wharton’s jelly was noted 3 cm from the
Pseudotumors of pregnancy
149
Fig. 1. The solid pseudotumor (coagulum) in case I. p : placenta; c: coagulum; uc: umbilical cord.
Fig. 2. The umbilical cord pseudocyst in case 11. p s : pseudocyst; uc: umbilical cord; uf: amniotic fluid.
fetal pole (Fig. 2). Color Doppler examination revealed normal flow in the vein as well as in the two arteries, but no flow inside the cyst. No fetal abnormalities were demonstrated. The cyst was tentatively of omphalomesenteric or allantoic origin. Further fetal development was completely satisfactory, and the size of the cyst remained stable. A healthy male infant weighing 2,580 g was born spontaneously in week 37. The cyst burst during the delivery. Wharton’s jelly surrounding the cyst was macroscopically edematous and the vessels widely separated. Histologically, there was no epithelial lining of the cyst wall, which was extremely edematous. The lesion was diagnosed as representing a local degeneration of Wharton’s jelly.
in differentiating between tumors with a good and a poor prognosis. Serial ultrasonography seems advisable in any case. True cysts in the umbilical cord are remnants of the omphalomesenteric duct or the allantois. They are invariably located near the fetal end of the cord. In contrast to pseudocysts, true cysts are lined with columnar or flattened epithelium. It is believed that pseudocysts are caused by localized mucoid degeneration of Wharton’s jelly (4). T h e etiology remains unknown but, in recent years, a few reports have suggested an association with trisomy 18 ( 5 , 6). Thus, karyotyping might be indicated, but was not done in our case. The lesions are easily demonstrated by ultrasound, and by means of color Doppler imaging, any vascularity can be ruled out (3); The purpose of Wharton’s jelly is to act as a supportive structure for the umbilical cord vessels which lack adventitia. Should degeneration of the jelly occur, it must be regarded as a potentially dangerous anomaly, in contrast to true cysts. It was hypothesized that the fetal circulation could be compromised because of constriction of the umbilical vessels in the area of degeneration (4, 7). It was later demonstrated that fetal aortic blood flow was reduced in the presence of a pseudocyst near the cord (8). This subject needs further investigation.
Discussion Solid tumors of the fetal membranes separate from the placenta are often recognized as biplacentas o r degenerated products of a coexisting twin. The vanishing twin often signals its presence by vaginal bleeding in the first trimester of pregnancy, sometimes giving rise to an intra-uterine hematoma. However, this event is unlikely in late pregnancy. In the case of solid tumors inside the uterus, there are two types of true neoplasm to bear in mind: hemangiomas and teratomas (1, 2). Hemangiomas are most often located in the placenta (chorioangiomas) and rarely in the umbilical cord. Their location to the fetal membranes has never been described. This also applies to the teratomas which are even more rare. These tumors usually do not interfere with the fetal circulation, but fetal growth retardation, heart insufficiency and death can be the consequence Of a richly vascularized tumor. The true nature of our hematoma could probably have been revealed by color Doppler imaging (3) which may also be helpful 11’
References 1. Clausen I. Umbilical cord anomalies and antenatal fetal deaths. Obstet Gynecol Surv 1989; 44: 841-5. 2, Yavner DL, Redline RW, Angiomyxoma of the umbilical cord with massive cystic degeneration of Wharton’s jelly, Arch path01 Lab Med 1989; 113: 935-7, 3. Jauniaux E, Campbell s, Vyas s. The use of color Doppler imaging for prenatal diagnosis of umbilical Acta Obstet Gynecol Scand 71 (1992)
150
4.
5. 6. 7.
I . Clausen and S. Grove Thomsen
cord anomalies: report of three cases. Am J Obstet Gynecol 1989; 161: 1195-7. Bergman P, Lundin P, Malmstrom T. Mucoid degeneration of Wharton’s jelly. An umbilical cord anomaly threatening fetal life. Acta Ohstet Gynecol Scand 1961; 40: 372-8. Jauniaux E, Donner C, Thomas C, Francotte J , Rodesch F, Avni FE. Umbilical cord pseudocyst in trisomy 18. Prenat Diag 1988; 8: 557-63. Constantine G , Anderson J, Fowlie A. Umbilical cord pseudocyst in trisomy 18. J Obstet Gynaecol 1990; 11: 274-5. Labarrere C, Sebastiani M, Siminovich M, Torassa E,
Acla
Obsrer Gynecol Scand 71 (1992)
Althabe 0. Absence of Wharton’s jelly around the urnbilical arteries: an unusual cause of perinatal mortality. Placenta 1985; 6: 555-9. 8. Kirkinen P, Jouppila P. Intrauterine membranous cyst: a report of antenatal diagnosis and obstetric aspects in two cases. Obstet Gynecol 1986; 67(Suppl): 26s-30s.
Address for correspondence: Ingo Clausen, M.D. Araliavej 60 DK-8462 Harlev J Denmark
Pseudotumors of the umbilical cord and fetal membranes INGOCLAUSEN AND STENGROVETHOMSEN From the Department of Obstetrics and Gynecology, Odense University Hospital, Odense, Denmark
Acta Obstet Gynecol Scand 1992; 71: 148-1.50 Antenatally diagnosed pseudotumors, i.e. non-neoplastic tumors, of the umbilical cord aqd fetal membranes may when scanned by ultrasound have an appearance leading to misdiagnosis. In the present cases. a hematoma in the fetal membranes was interpreted as a chorioangioma, and a cystic mass inside the umbilical cord caused by degeneration of Wharton’s jelly was regarded as an omphalomesenteric or allantoic cyst. The two cases are presented. Color Doppler evaluation seems advisable. Key words: pregnancy complications; umbilical cord; hematoma; cyst; pseudotumor Submitted July 15, 1991 Accepted October 14, 1991
Tumors of the umbilical cord or fetal membranes are uncommon findings that can be discovered prenatally by ultasound scanning. The nature of such tumors cannot always be determined accurately before delivery. Although they can be regarded as benign, they may represent a great hazard to the fetus. No malignant tumor of the two structures has ever been published (1). We describe 2 cases of so-called pseudotumors, i.e. non-neoplastic tumors, of the umbilical cord and fetal membranes.
Case reports Case I
A 25-year-old primipara was admitted because of vaginal bleeding in the 28th week of pregnancy. She experienced no pain and the uterus was relaxed and not tender. A normal CTG was recorded. A t the initial ultrasound scanning, there were no signs of fetal defects, intra-uterine growth retardation o r hydrops, and a normal anterior wall placenta was described. O n account of continuous hemorrhage and irritability of the uterus, a new ultrasound examination was performed 6 h later. Behind the internal Acta Obstet Gynecol Scand 71 (1992)
cervical 0s an echo-dense, massive tumor measuring 8 X 9 cm was seen (Fig. 1). There was no demonstrable connection to the fetus o r umbilical cord. After a week the process displayed small echo-free areas of a supposedly vascular nature and, therefore, it was thought to be a chorioangioma and not a fibroma of the uterine wall. Color Doppler imaging was not undertaken. Apart from vaginal spotting, the pregnancy took an uncomplicated course. The tumor did not change its appearance on repeated ultrasound scanning, and fetal growth was normal. A healthy male infant weighing 2,500 g was born at cesarean section in week 35 of pregnancy. The placenta was normal. Located to the fetal membranes, a clearly demarcated reddish-brown jelly-like tumor was found and microscopically shown t o be a coagulum. The postoperative course was uneventful. Case
I1
A 25-year-old primipara was examined by ultrasound scanning in week 22 of her hitherto uncomplicated pregnancy. A cystic mass with a diameter of 3 cm inside the umbilical cord and surrounded by thickened Wharton’s jelly was noted 3 cm from the
Pseudotumors of pregnancy
149
Fig. 1. The solid pseudotumor (coagulum) in case I. p : placenta; c: coagulum; uc: umbilical cord.
Fig. 2. The umbilical cord pseudocyst in case 11. p s : pseudocyst; uc: umbilical cord; uf: amniotic fluid.
fetal pole (Fig. 2). Color Doppler examination revealed normal flow in the vein as well as in the two arteries, but no flow inside the cyst. No fetal abnormalities were demonstrated. The cyst was tentatively of omphalomesenteric or allantoic origin. Further fetal development was completely satisfactory, and the size of the cyst remained stable. A healthy male infant weighing 2,580 g was born spontaneously in week 37. The cyst burst during the delivery. Wharton’s jelly surrounding the cyst was macroscopically edematous and the vessels widely separated. Histologically, there was no epithelial lining of the cyst wall, which was extremely edematous. The lesion was diagnosed as representing a local degeneration of Wharton’s jelly.
in differentiating between tumors with a good and a poor prognosis. Serial ultrasonography seems advisable in any case. True cysts in the umbilical cord are remnants of the omphalomesenteric duct or the allantois. They are invariably located near the fetal end of the cord. In contrast to pseudocysts, true cysts are lined with columnar or flattened epithelium. It is believed that pseudocysts are caused by localized mucoid degeneration of Wharton’s jelly (4). T h e etiology remains unknown but, in recent years, a few reports have suggested an association with trisomy 18 ( 5 , 6). Thus, karyotyping might be indicated, but was not done in our case. The lesions are easily demonstrated by ultrasound, and by means of color Doppler imaging, any vascularity can be ruled out (3); The purpose of Wharton’s jelly is to act as a supportive structure for the umbilical cord vessels which lack adventitia. Should degeneration of the jelly occur, it must be regarded as a potentially dangerous anomaly, in contrast to true cysts. It was hypothesized that the fetal circulation could be compromised because of constriction of the umbilical vessels in the area of degeneration (4, 7). It was later demonstrated that fetal aortic blood flow was reduced in the presence of a pseudocyst near the cord (8). This subject needs further investigation.
Discussion Solid tumors of the fetal membranes separate from the placenta are often recognized as biplacentas o r degenerated products of a coexisting twin. The vanishing twin often signals its presence by vaginal bleeding in the first trimester of pregnancy, sometimes giving rise to an intra-uterine hematoma. However, this event is unlikely in late pregnancy. In the case of solid tumors inside the uterus, there are two types of true neoplasm to bear in mind: hemangiomas and teratomas (1, 2). Hemangiomas are most often located in the placenta (chorioangiomas) and rarely in the umbilical cord. Their location to the fetal membranes has never been described. This also applies to the teratomas which are even more rare. These tumors usually do not interfere with the fetal circulation, but fetal growth retardation, heart insufficiency and death can be the consequence Of a richly vascularized tumor. The true nature of our hematoma could probably have been revealed by color Doppler imaging (3) which may also be helpful 11’
References 1. Clausen I. Umbilical cord anomalies and antenatal fetal deaths. Obstet Gynecol Surv 1989; 44: 841-5. 2, Yavner DL, Redline RW, Angiomyxoma of the umbilical cord with massive cystic degeneration of Wharton’s jelly, Arch path01 Lab Med 1989; 113: 935-7, 3. Jauniaux E, Campbell s, Vyas s. The use of color Doppler imaging for prenatal diagnosis of umbilical Acta Obstet Gynecol Scand 71 (1992)
150
4.
5. 6. 7.
I . Clausen and S. Grove Thomsen
cord anomalies: report of three cases. Am J Obstet Gynecol 1989; 161: 1195-7. Bergman P, Lundin P, Malmstrom T. Mucoid degeneration of Wharton’s jelly. An umbilical cord anomaly threatening fetal life. Acta Ohstet Gynecol Scand 1961; 40: 372-8. Jauniaux E, Donner C, Thomas C, Francotte J , Rodesch F, Avni FE. Umbilical cord pseudocyst in trisomy 18. Prenat Diag 1988; 8: 557-63. Constantine G , Anderson J, Fowlie A. Umbilical cord pseudocyst in trisomy 18. J Obstet Gynaecol 1990; 11: 274-5. Labarrere C, Sebastiani M, Siminovich M, Torassa E,
Acla
Obsrer Gynecol Scand 71 (1992)
Althabe 0. Absence of Wharton’s jelly around the urnbilical arteries: an unusual cause of perinatal mortality. Placenta 1985; 6: 555-9. 8. Kirkinen P, Jouppila P. Intrauterine membranous cyst: a report of antenatal diagnosis and obstetric aspects in two cases. Obstet Gynecol 1986; 67(Suppl): 26s-30s.
Address for correspondence: Ingo Clausen, M.D. Araliavej 60 DK-8462 Harlev J Denmark
