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Sacral parasite conjoined twin
There is some evidence to support the use of prophylactic -blockade in nonhypertensive patients with Marfan’s syndrome to slow the rate of aortic dilatation; however, its prophylactic use in patients without Marfan’s syndrome has not been as convincing.5 Rossiter et al8 have recommended antepartum surveillance with echocardiography every 6 – 8 weeks during pregnancy and then 4 – 8 weeks after delivery to determine the need for intervention. Surgical intervention of an aortic aneurysm in a pregnant woman would necessitate cardiopulmonary bypass during the pregnancy that may compromise fetal status. There is scant literature as to recommendations for mode of delivery. The review by Williams et al2 discussed an additional series of 14 pregnant women with aortic dissection that had good outcomes with cesarean delivery or pregnancy termination followed by repair of the aneurysm. They then concluded that aortic disruptions during pregnancy were best managed by cesarean delivery and immediate repair of the lesions. However, all of the patients they reported were symptomatic from their disease or had evidence of fetal distress. The case report by Locufier et al4 in 1989 described a woman who was found incidentally to have a descending thoracic aneurysm in the postpartum period after a vaginal delivery, but did not make any recommendations as to a preferred mode of delivery. Therefore, in our case, because there was a paucity of information specifically for pregnant patients with asymptomatic aortic aneurysms that were deemed at low risk for spontaneous rupture by standard surgical criteria, we saw no reason to prohibit the patient from attempting vaginal delivery after informed consent. Shortening of the second stage of labor by forceps delivery to prevent excessive Valsalva maneuvers and increased intrathoracic pressures was also considered prudent. Because 40% of aortic aneurysms are asymptomatic and are found only incidentally during diagnostic imaging studies for other indications, most aneurysms detected during pregnancy will be chance findings. Al-
Sacral Parasite Conjoined Twin Szu-Yuan Chou, MD, So-Jung Liang, MD, Chih-Fen Wu, MD, and Chun-Sen Hsu, MD Department of Obstetrics and Gynecology, Taipei Medical University–Municipal Wan Fan Hospital, Taipei, Taiwan Address reprint requests to: Chun-Sen Hsu, MD, Department of Obstetrics and Gynecology, Taipei Medical University–Municipal Wan Fan Hospital, 111, Hsin-Long Road Sec. 3, Taipei 116, Taiwan; E-mail: [email protected].
938
though allowing normal labor and vaginal delivery in an asymptomatic woman with a thoracic aortic aneurysm is unsettling, it can be safely performed after taking into account the etiology of the aneurysm, the diameter of the lesion, the patient’s blood pressure, and surgical risks to both mother and fetus. A multidisciplinary team approach to management in a tertiary care center can optimize both maternal and fetal/neonatal outcomes. REFERENCES 1. Sparacia G, Midiri M, Finazzo M, Bartolotta T. Aortic aneurysm. RadPa Teaching File. Available at: http://www. unipa.it/⬃radpa/p2/aneurysm.html. Accessed April 1, 2001. 2. Williams GM, Gott VL, Brawley RK, Schauble JF, Labs JD. Aortic disease associated with pregnancy. J Vasc Surg 1988;8: 470 –5. 3. Risken MJ, Nadel ES, Brown DF. Back pain in a pregnant patient. J Emerg Med 2001;20:403– 6. 4. Locufier JL, Bosschaerts T, Barthel J, Delwarte D, Barroy JP. Aneurysm of the decending thoracic aorta in a young woman. J Cardiovasc Surg 1989;30:499 –502. 5. Fuster V, Andrews P. Medical treatment of the aorta. Cardiol Clin 1999;17:697–715. 6. Coady MA, Rizzo JA, Elefteriades JA. Developing surgical intervention criteria for thoracic aortic aneurysms. Cardiol Clin 1999;17:827–39. 7. Foster JH, Bolasny BL, Gobbel WG Jr, Scott HW Jr. Comparative study of elective resection and expectant treatment of abdominal aortic aneurysm. Surg Gynecol Obstet 1969;129:1–9. 8. Rossiter JP, Repke JT, Morales AJ, Murphy EA, Pyeritz RE. A prospective longitudinal evaluation of pregnancy in the Marfan’s syndrome. Am J Obstet Gynecol 1995;173: 1599 – 606.
Received May 10, 2001. Received in revised form July 20, 2001. Accepted July 26, 2001.
BACKGROUND: A variant of conjoined twins is one in which one twin is incomplete. CASE: A female infant was born vaginally at 40 weeks’ gestation to a healthy primipara. No important abnormalities were noted during prenatal examinations. The infant was fully developed in all external aspects except for a parasitic body conjoined with her sacrococcygeal region. Separated by operation 2 weeks after birth, the parasite contained lower limbs, adipose tissue, muscles, and a bowel sac. Over 4 years of observation, no abnormalities have been found since the operation.
VOL. 98, NO. 5, PART 2, NOVEMBER 2001 © 2001 by The American College of Obstetricians and Gynecologists. Published by Elsevier Science Inc.
0029-7844/01/$20.00 PII S0029-7844(01)01482-X
CONCLUSION: Obstetricians should be aware of the existence of a parasite twin during prenatal examinations and of the importance of the differential diagnosis of parasite and teratoma, a neoplasm with malignant potential. (Obstet Gynecol 2001;98:938 – 40. © 2001 by the American College of Obstetricians and Gynecologists.)
In humans, sacral parasite conjoined twins are rare anomalies consisting of an incomplete twin (parasite) attached to the fully developed body of the cotwin (infant) in the sacrococcygeal region. The rarity of this anomaly is still not reported in the literature, but the incidence of conjoined twins stillborn or born alive is about one per 50,000 births.1,2 CASE A female infant weighing 3500 g was born vaginally at 40 weeks’ gestation to a healthy 30-year-old primipara. The woman had no family history of congenital anomalies and had taken no medication during her pregnancy. Prenatal care had been given at the clinic of Taipei Medical University–affiliated Wan Fan Hospital from 30 weeks’ gestation on; before then, she had attended the prenatal clinic at a local university hospital. During routine prenatal examinations, the woman had no abnormal findings noted (ultrasonographic findings, findings relating to uterine size or weight gain, or results of serum screening); the mother had ␣-thalassemia and the father -thalassemia. The placenta was single and normal. The Apgar scores of the infant were 9 at 1 minute and 10 at 5 minutes. At birth, the infant was noted to have a parasitic twin conjoined with her sacrococcygeal region. She was referred to our pediatric department to be assessed for surgical correction. The infant was fully developed in all external aspects except for a parasitic body conjoined with her sacrococcygeal region (Figure 1). The conjoined structure was completely covered with skin that was continuous with that of the infant. Magnetic resonance imaging of the pelvis revealed a conjoined twin that did not communicate with the spinal cord or related structures. The healthy body had bilobular fluid-collecting spaces with blind ends at precoccygeal regions, and a sinus tract (or fluid sac) was suspected. A long bone and unknown soft tissue in the parasitic body of the twin were also present. Radiologic examination of the whole spine showed no definite bony abnormalities. One soft tissue mass with partial bone structure (including half a pelvis, a femur, a fibula, a tibia, and partial foot bones) was noted at the sacrococcygeal area. In the radiographic report, suspected teratoma or parasitic twin was noted. A fistula
VOL. 98, NO. 5, PART 2, NOVEMBER 2001
Figure 1. Structure conjoined to the autosite. Chou. Sacral Parasite Conjoined Twin. Obstet Gynecol 2001.
was suspected at the junction between the parasitic body and the sacrum. During fistulographic assessment, contrast medium pooled in the pocket between the parasitic body and the infant, but no fistula tract was detected. Single-contrast barium enema revealed a normal course of large bowel without fistulas. Two weeks after birth, the infant underwent surgery in the prone position. An incision was made where the parasitic body was attached to the infant. Cystic structures were connected to the infant coccyx; the whole parasitic body was excised, along with part of the infant’s coccyx. The wound was sutured by the close primary and a drain was placed. One tissue fragment obtained for pathologic examination was 10 ⫻ 10 ⫻ 5-cm. Skin covered the parasitic body, and the whole mass was irregularly shaped. Two deformed lower limbs were attached to the main mass. Poorly formed toes also were identified. After dissection of the main mass, bone of bilateral lower limbs and some pelvic bone were seen. The mass was composed mainly of adipose tissue, admixed with some disarrayed muscle bundles. A bowel sac also was found in the adipose tissue. Microscopically, the specimen included lower limbs, adipose tissue, muscles, and a bowel sac, which was covered by colonic mucosa and partly by squamous epithelium.
Chou et al
Sacral Parasite Conjoined Twin
939
determined that the infection was not related to the previous existence of the sacral parasite conjoined twin. Distinguishing a parasite from a teratoma is a clinical challenge. A parasite may be diagnosed as a teratoma, especially in the case of sacral parasite, which sometimes appears as a tumor-like mass. The distinction between parasite and teratoma is clinically important because a teratoma is a neoplasm with malignant potential.5 We can distinguish these two abnormalities only by gross findings: a parasite has the structure of a part of the body, such as part of a limb, a finger, a toe, a nail, or intestine, in its external appearance, whereas teratoma appears as a simple tumorlike mass even if it has such structures within.5 Figure 2. Infant’s perineal area 4 years after surgical removal of the parasitic body. Chou. Sacral Parasite Conjoined Twin. Obstet Gynecol 2001.
COMMENT The reported incidence of conjoined twins is about one per 50,000 births,1 but the frequency of sacral parasite has not yet been reported. A higher incidence of female infants in all types of conjoined twins was noted.3 The reason for this higher incidence is unclear. Prenatal diagnosis of sacral parasite conjoined twins is difficult because of the absence of ossified elements in connections, changing fetal positions, and the rarity of these abnormalities. Gastrointestinal and cardiovascular abnormalities are encountered frequently in conjoined twins.4 We have observed this child for about 4 years (Figure 2), and she has no abnormal gastrointestinal or cardiovascular findings. Recently, the child was admitted to our pediatric inpatient ward because of an episode of urinary tract infection. It was
Mesenchymal Stem Villous Hyperplasia of the Placenta and Fetal Growth Restriction Yoshimitsu Kuwabara, MD, Yoshio Shima, MD, Tsutomu Araki, MD, and Sumio Shin, MD Departments of Obstetrics and Gynecology and Premature and Neonatal Medicine, Japanese Red Cross Katsushika Maternity Hospital, Tokyo, Japan; and Department of Obstetrics and Gynecology, Nippon Medical School, Tokyo, Japan
Address reprint requests to: Yoshimitsu Kuwabara, MD, Department of Obstetrics and Gynecology, Nippon Medical School, 1-1-5, Sendagi, Bunkyou-ku, Tokyo 113-8602, Japan; E-mail: kuwa_obgy@hotmail. com.
940
REFERENCES 1. Freeman HL. Conjoined thoracopagus twins: Case report. Am J Obstet Gynecol 1964;84:1904 –9. 2. Hoyle RM, Thomas CG Jr. Twenty-three-year follow-up of separated ischiopagus tetrapus conjoined twins. Ann Surg 1989;210:673–9. 3. Lu T, Lee KH. Obstetric management of conjoined twins. J Obstet Gynaecol Br Commonw 1967;74:757– 62. 4. Harper RG, Kenigsberg K, Sia CG, Horn D, Stern D, Bongiovi V. Xiphopagus conjoined twins: A 300-year review of the obstetric, morphopathologic, neonatal, and surgical parameters. Am J Obstet Gynecol 1980;137: 617–29. 5. Tokunaga S, Ikeda T, Matsuo T, Maeda H, Kurosaki N, Shimoda H. A case of sacral parasite. Congen Anom 1986; 26:321–30. Received November 27, 2000. Received in revised form March 14, 2001. Accepted June 7, 2001.
BACKGROUND: Mesenchymal stem villous hyperplasia of the placenta is a rare placental anomaly characterized by placental vascular malformation and the appearance of a partial mole. CASE: A multiparous woman presented with fetal growth restriction (FGR) at 35 weeks’ gestation. Ultrasonographic examination showed multiple anechoic lesions on the placental surface. Cesarean delivery was performed at 37 weeks’ gestation, and a healthy 1536-g female was delivered. The placenta had aneurysmal dilatations of the chorionic vessels. Unlike other cases, it also showed subamniotic hemorrhage and had no features of a partial mole. Histologic examination established the diagnosis of mesenchymal stem villous hyperplasia of the placenta with severe thrombosis. CONCLUSION: Fetal growth restriction can be caused by severe thrombosis in this placental anomaly. (Obstet Gy-
VOL. 98, NO. 5, PART 2, NOVEMBER 2001 © 2001 by The American College of Obstetricians and Gynecologists. Published by Elsevier Science Inc.
0029-7844/01/$20.00 PII S0029-7844(01)01512-5
Sacral Parasite Conjoined Twin Szu-Yuan Chou, MD, So-Jung Liang, MD, Chih-Fen Wu, MD, and Chun-Sen Hsu, MD Department of Obstetrics and Gynecology, Taipei Medical University–Municipal Wan Fan Hospital, Taipei, Taiwan Address reprint requests to: Chun-Sen Hsu, MD, Department of Obstetrics and Gynecology, Taipei Medical University–Municipal Wan Fan Hospital, 111, Hsin-Long Road Sec. 3, Taipei 116, Taiwan; E-mail: [email protected].
938
though allowing normal labor and vaginal delivery in an asymptomatic woman with a thoracic aortic aneurysm is unsettling, it can be safely performed after taking into account the etiology of the aneurysm, the diameter of the lesion, the patient’s blood pressure, and surgical risks to both mother and fetus. A multidisciplinary team approach to management in a tertiary care center can optimize both maternal and fetal/neonatal outcomes. REFERENCES 1. Sparacia G, Midiri M, Finazzo M, Bartolotta T. Aortic aneurysm. RadPa Teaching File. Available at: http://www. unipa.it/⬃radpa/p2/aneurysm.html. Accessed April 1, 2001. 2. Williams GM, Gott VL, Brawley RK, Schauble JF, Labs JD. Aortic disease associated with pregnancy. J Vasc Surg 1988;8: 470 –5. 3. Risken MJ, Nadel ES, Brown DF. Back pain in a pregnant patient. J Emerg Med 2001;20:403– 6. 4. Locufier JL, Bosschaerts T, Barthel J, Delwarte D, Barroy JP. Aneurysm of the decending thoracic aorta in a young woman. J Cardiovasc Surg 1989;30:499 –502. 5. Fuster V, Andrews P. Medical treatment of the aorta. Cardiol Clin 1999;17:697–715. 6. Coady MA, Rizzo JA, Elefteriades JA. Developing surgical intervention criteria for thoracic aortic aneurysms. Cardiol Clin 1999;17:827–39. 7. Foster JH, Bolasny BL, Gobbel WG Jr, Scott HW Jr. Comparative study of elective resection and expectant treatment of abdominal aortic aneurysm. Surg Gynecol Obstet 1969;129:1–9. 8. Rossiter JP, Repke JT, Morales AJ, Murphy EA, Pyeritz RE. A prospective longitudinal evaluation of pregnancy in the Marfan’s syndrome. Am J Obstet Gynecol 1995;173: 1599 – 606.
Received May 10, 2001. Received in revised form July 20, 2001. Accepted July 26, 2001.
BACKGROUND: A variant of conjoined twins is one in which one twin is incomplete. CASE: A female infant was born vaginally at 40 weeks’ gestation to a healthy primipara. No important abnormalities were noted during prenatal examinations. The infant was fully developed in all external aspects except for a parasitic body conjoined with her sacrococcygeal region. Separated by operation 2 weeks after birth, the parasite contained lower limbs, adipose tissue, muscles, and a bowel sac. Over 4 years of observation, no abnormalities have been found since the operation.
VOL. 98, NO. 5, PART 2, NOVEMBER 2001 © 2001 by The American College of Obstetricians and Gynecologists. Published by Elsevier Science Inc.
0029-7844/01/$20.00 PII S0029-7844(01)01482-X
CONCLUSION: Obstetricians should be aware of the existence of a parasite twin during prenatal examinations and of the importance of the differential diagnosis of parasite and teratoma, a neoplasm with malignant potential. (Obstet Gynecol 2001;98:938 – 40. © 2001 by the American College of Obstetricians and Gynecologists.)
In humans, sacral parasite conjoined twins are rare anomalies consisting of an incomplete twin (parasite) attached to the fully developed body of the cotwin (infant) in the sacrococcygeal region. The rarity of this anomaly is still not reported in the literature, but the incidence of conjoined twins stillborn or born alive is about one per 50,000 births.1,2 CASE A female infant weighing 3500 g was born vaginally at 40 weeks’ gestation to a healthy 30-year-old primipara. The woman had no family history of congenital anomalies and had taken no medication during her pregnancy. Prenatal care had been given at the clinic of Taipei Medical University–affiliated Wan Fan Hospital from 30 weeks’ gestation on; before then, she had attended the prenatal clinic at a local university hospital. During routine prenatal examinations, the woman had no abnormal findings noted (ultrasonographic findings, findings relating to uterine size or weight gain, or results of serum screening); the mother had ␣-thalassemia and the father -thalassemia. The placenta was single and normal. The Apgar scores of the infant were 9 at 1 minute and 10 at 5 minutes. At birth, the infant was noted to have a parasitic twin conjoined with her sacrococcygeal region. She was referred to our pediatric department to be assessed for surgical correction. The infant was fully developed in all external aspects except for a parasitic body conjoined with her sacrococcygeal region (Figure 1). The conjoined structure was completely covered with skin that was continuous with that of the infant. Magnetic resonance imaging of the pelvis revealed a conjoined twin that did not communicate with the spinal cord or related structures. The healthy body had bilobular fluid-collecting spaces with blind ends at precoccygeal regions, and a sinus tract (or fluid sac) was suspected. A long bone and unknown soft tissue in the parasitic body of the twin were also present. Radiologic examination of the whole spine showed no definite bony abnormalities. One soft tissue mass with partial bone structure (including half a pelvis, a femur, a fibula, a tibia, and partial foot bones) was noted at the sacrococcygeal area. In the radiographic report, suspected teratoma or parasitic twin was noted. A fistula
VOL. 98, NO. 5, PART 2, NOVEMBER 2001
Figure 1. Structure conjoined to the autosite. Chou. Sacral Parasite Conjoined Twin. Obstet Gynecol 2001.
was suspected at the junction between the parasitic body and the sacrum. During fistulographic assessment, contrast medium pooled in the pocket between the parasitic body and the infant, but no fistula tract was detected. Single-contrast barium enema revealed a normal course of large bowel without fistulas. Two weeks after birth, the infant underwent surgery in the prone position. An incision was made where the parasitic body was attached to the infant. Cystic structures were connected to the infant coccyx; the whole parasitic body was excised, along with part of the infant’s coccyx. The wound was sutured by the close primary and a drain was placed. One tissue fragment obtained for pathologic examination was 10 ⫻ 10 ⫻ 5-cm. Skin covered the parasitic body, and the whole mass was irregularly shaped. Two deformed lower limbs were attached to the main mass. Poorly formed toes also were identified. After dissection of the main mass, bone of bilateral lower limbs and some pelvic bone were seen. The mass was composed mainly of adipose tissue, admixed with some disarrayed muscle bundles. A bowel sac also was found in the adipose tissue. Microscopically, the specimen included lower limbs, adipose tissue, muscles, and a bowel sac, which was covered by colonic mucosa and partly by squamous epithelium.
Chou et al
Sacral Parasite Conjoined Twin
939
determined that the infection was not related to the previous existence of the sacral parasite conjoined twin. Distinguishing a parasite from a teratoma is a clinical challenge. A parasite may be diagnosed as a teratoma, especially in the case of sacral parasite, which sometimes appears as a tumor-like mass. The distinction between parasite and teratoma is clinically important because a teratoma is a neoplasm with malignant potential.5 We can distinguish these two abnormalities only by gross findings: a parasite has the structure of a part of the body, such as part of a limb, a finger, a toe, a nail, or intestine, in its external appearance, whereas teratoma appears as a simple tumorlike mass even if it has such structures within.5 Figure 2. Infant’s perineal area 4 years after surgical removal of the parasitic body. Chou. Sacral Parasite Conjoined Twin. Obstet Gynecol 2001.
COMMENT The reported incidence of conjoined twins is about one per 50,000 births,1 but the frequency of sacral parasite has not yet been reported. A higher incidence of female infants in all types of conjoined twins was noted.3 The reason for this higher incidence is unclear. Prenatal diagnosis of sacral parasite conjoined twins is difficult because of the absence of ossified elements in connections, changing fetal positions, and the rarity of these abnormalities. Gastrointestinal and cardiovascular abnormalities are encountered frequently in conjoined twins.4 We have observed this child for about 4 years (Figure 2), and she has no abnormal gastrointestinal or cardiovascular findings. Recently, the child was admitted to our pediatric inpatient ward because of an episode of urinary tract infection. It was
Mesenchymal Stem Villous Hyperplasia of the Placenta and Fetal Growth Restriction Yoshimitsu Kuwabara, MD, Yoshio Shima, MD, Tsutomu Araki, MD, and Sumio Shin, MD Departments of Obstetrics and Gynecology and Premature and Neonatal Medicine, Japanese Red Cross Katsushika Maternity Hospital, Tokyo, Japan; and Department of Obstetrics and Gynecology, Nippon Medical School, Tokyo, Japan
Address reprint requests to: Yoshimitsu Kuwabara, MD, Department of Obstetrics and Gynecology, Nippon Medical School, 1-1-5, Sendagi, Bunkyou-ku, Tokyo 113-8602, Japan; E-mail: kuwa_obgy@hotmail. com.
940
REFERENCES 1. Freeman HL. Conjoined thoracopagus twins: Case report. Am J Obstet Gynecol 1964;84:1904 –9. 2. Hoyle RM, Thomas CG Jr. Twenty-three-year follow-up of separated ischiopagus tetrapus conjoined twins. Ann Surg 1989;210:673–9. 3. Lu T, Lee KH. Obstetric management of conjoined twins. J Obstet Gynaecol Br Commonw 1967;74:757– 62. 4. Harper RG, Kenigsberg K, Sia CG, Horn D, Stern D, Bongiovi V. Xiphopagus conjoined twins: A 300-year review of the obstetric, morphopathologic, neonatal, and surgical parameters. Am J Obstet Gynecol 1980;137: 617–29. 5. Tokunaga S, Ikeda T, Matsuo T, Maeda H, Kurosaki N, Shimoda H. A case of sacral parasite. Congen Anom 1986; 26:321–30. Received November 27, 2000. Received in revised form March 14, 2001. Accepted June 7, 2001.
BACKGROUND: Mesenchymal stem villous hyperplasia of the placenta is a rare placental anomaly characterized by placental vascular malformation and the appearance of a partial mole. CASE: A multiparous woman presented with fetal growth restriction (FGR) at 35 weeks’ gestation. Ultrasonographic examination showed multiple anechoic lesions on the placental surface. Cesarean delivery was performed at 37 weeks’ gestation, and a healthy 1536-g female was delivered. The placenta had aneurysmal dilatations of the chorionic vessels. Unlike other cases, it also showed subamniotic hemorrhage and had no features of a partial mole. Histologic examination established the diagnosis of mesenchymal stem villous hyperplasia of the placenta with severe thrombosis. CONCLUSION: Fetal growth restriction can be caused by severe thrombosis in this placental anomaly. (Obstet Gy-
VOL. 98, NO. 5, PART 2, NOVEMBER 2001 © 2001 by The American College of Obstetricians and Gynecologists. Published by Elsevier Science Inc.
0029-7844/01/$20.00 PII S0029-7844(01)01512-5