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Case report: In-utero aspiration of sacrococcygeal cyst
ClinicalRadiology (1991) 44, 119-120
Case Report: In-utero Aspiration of Sacrococcygeal Cyst M. J. W E S T O N
and H. ANDREWS
Department of Radiodiagnosis, Bristol Royal Infirmary, Bristol Pre-natal aspiration, under ultrasound control, was performed on a sacrococcygeal cyst measuring 19 cm in diameter in a 38 week fetus to allow normal vaginal delivery of a live baby girl. This is the first report of such intervention leading to normal delivery. Weston, M.J. & A n d r e w s , H . (1991). Sacrococcygeal Cyst
Clinical Radiology 44, 119-120. C a s e R e p o r t : I n - u t e r o A s p i r a t i o n o f
S k i n - c o v e r e d s a c r o c o c c y g e a l m a s s e s in t h e n e o n a t e are o f varied origin, t h e c o m m o n e s t b e i n g t e r a t o m a s , l i p o m a s and m e n i n g o c o e l e s ( L e m i r e a n d B e c k w i t h , 1982). T h e y may g r o w to a n e n o r m o u s size a n d o b s t r u c t l a b o u r , w i t h c o n s e q u e n t i n c r e a s e in p e r i n a t a l m o r t a l i t y ( G r o s s et al., 1987). T h e r e are n o k n o w n risk f a c t o r s f o r s a c r o c o c c y g e a l cysts, a n d t h u s e~/rly d i a g n o s i s is o n l y p o s s i b l e w h e n routine o b s t e t r i c u l t r a s o u n d is p e r f o r m e d . O t h e r w i s e t h e c o m m o n e s t p r e s e n t a t i o n is ' l a r g e f o r d a t e s ' . F r o m t h e literature it is a p p a r e n t t h a t few s a c r o c o c c y g e a l t e r a t o m a s d i a g n o s e d p r i o r to 30 w e e k s g e s t a t i o n s u r v i v e ( K u h l m a n n et al., 1987). W e r e p o r t a c a s e in w h i c h the early d i a g n o s i s and the successful i n t r a u t e r i n e a s p i r a t i o n o f a s a c r o c o c c y geal cyst a l l o w e d a n o r m a l t r a n s v a g i n a t d e l i v e r y o f a live baby girl.
polyhydramnios or of fetal hydrops. At 38 weeks gestation it measured 19 cm in diameter (Fig. 3). In order to allow a vaginal delivery, percutaneous aspiration of the cyst was undertaken at 38 weeks gestation, prior to induction of labour. A 20 gauge spinal needle was advanced into the cyst under ultrasound control, using aseptic technique. The puncture site was chosen to avoid the anterior lying placenta. Two aspirations were performed on consecutive days. The first yielded 21 of clear, straw coloured fluid and the second 750 ml, resulting in almost complete collapse of the cyst. There was no evidence of any complications.
CASE REPORT A 22-year-old, caucasian, G2 P1, presented for a routine ultrasound examination at 18 weeks, at which time a large sacrococcygeal cystic structure was demonstrated. A 20 weeks gestation a detailed anomaly ultrasound scan revealed that this cystic structure was unilocular and arising from the anterior surface of the coccyx (Fig. 1). It measured 6 cm in diameter and was thin-walled with no internal echoes. There was no evidence of any other fetal abnormality and ultrasound estimation of gestational age corresponded with dates. A diagnosis of a cystic sacrococcygeal teratoma was made. Monthly follow-up ultrasound scans demonstrated normal fetal growth. The sacrococcygeal cyst remained thin-walled and unilocular but showed a gradual increase in size (Fig. 2). There was no evidence of
Fig. 2 - Ultrasound scan showing increasing size of sacrococcygeal cyst.
Fig. 1 -Ultrasound scan of fetus at 20 weeks gestation showing a perineal cystic lesion (arrow). Correspondence to: Dr M. J. Weston, Department of Radiodiagnosis, Bristol Royal Infirmary, Marlborough Street, Bristol BS2 8HW.
Fig. 3 Ultrasound scan at 38 weeks gestation showing the cyst to measure 19 cm in diameter.
120
CLINICAL RADIOLOGY
Fig. 4 - Post-natal picture showing a large empty sac arising from the sacrum. Labour was induced with artificial rupture of membranes and Prostin pessary immediately after the second aspiration. The first stage of labour lasted 80 min and the second 8 min. A baby girl weighing 3.77 kg, with an immediate Apgar score of nine was delivered. A large collapsed skincovered cystic sac overlaying the sacrum was identified (Fig. 4). Two days later the cyst was removed at operation. A plane of dissection was found with difficulty and active bleeding resulted in the loss of 260 ml of blood. On histology the lesion resembled a meningomyelocoele, the cyst being lined with patchy layers of columnar epithelium resembling choroid plexus, neuroglial tissue, nerves and collagen. It could be also described as a monodermal teratoma (i.e. unilateral differentiation of tissues derived from one of the germ cell layers). No communication with the spinal cord was shown. Radiographs of the spine showed no significant bony abnormality. The baby's post=operative course was uneventful. There was normal tone and movement in the limbs and an ultrasound scan of the brain was normal. She was discharged home at 18 days of age. DISCUSSION S a c r o c o c c y g e a l t u m o u r s are rare with a frequency o f only one o r two p e r 40 000 live births with the m a j o r i t y occurring in females. S a c r o c o c c y g e a l t e r a t o m a c a n be complicated by non-immune hydrops and placentomegaly, a n d these cases c a r r y a p o o r prognosis. Sacrococcygeal t e r a t o m a s have a n 18% a s s o c i a t e d a n o m a l y rate ( K u h l m a n n et al., 1987) with s p i n a bifida, meningocoele, v a g i n a l stenosis, t r a c h e o - o e s o p h a g e a l fistula, ventricular septal defect a n d m a x i l l o f a c i a l a n o m a l i e s . P r e - n a t a l diagnosis is beneficial in a v o i d i n g the u n e x p e c t e d development of obstructed labour. N e o n a t a l d e a t h has followed r u p t u r e o f sacrococeygeal t e r a t o m a s d u r i n g vaginal delivery ( G u i g i a r o et al., 1977). I f the diagnosis is m a d e a n t e n a t a l l y , the literature a d v o cates elective C a e s a r e a n section ( G r o s s et al., 1987; Sepulveda, 1989). W h e n the d i a g n o s i s o f sacrococcygeal t u m o u r is m a d e d u r i n g o b s t r u c t e d v a g i n a l l a b o u r , others have surgically d e c o m p r e s s e d the t u m o u r t r a n s a b d o m i nally, at e m e r g e n c y o p e r a t i o n , in o r d e r to allow vaginal delivery (Tanaree, 1982; E d w a r d s , 1983). D r a i n a g e p e r vaginum, given the a p p r o p r i a t e fetal p r e s e n t a t i o n , has
also been p e r f o r m e d as an e m e r g e n c y (Weiss et al., 1976). U l t r a s o u n d shows the m a j o r i t y o f s a c r o c o c c y g e a l teratom a s to have a large solid c o m p o n e n t , t h o u g h p r e d o m i n antly cystic lesions m a y also occur, a n d it is in the latter lesions t h a t p l a n n e d i n t r a u t e r i n e i n t e r v e n t i o n b y aspir a t i o n m a y allow a n o r m a l vaginal delivery. M i n t z et al. (1983) r e p o r t e d p e r c u t a n e o u s a s p i r a t i o n i m m e d i a t e l y p r i o r to elective C a e s a r i a n section, b u t we believe o u r s is the first r e p o r t o f n o r m a l vaginal delivery following such aspiration. A definite histological diagnosis m a y be difficult ( L e m i r e a n d Beckwith, 1982). H i s t o l o g i c a l l y this lesion has the characteristics o f a m y e l o m e n i n g o c o e t e . This raises the possibility t h a t there will be t e t h e r i n g o f the spinal c o r d o r an increased risk o f meningitiS later in life. In the adult, p e r e u t a n e o u s a s p i r a t i o n o f an a n t e r i o r sacral m e n i n g o c o e l e carries a high risk o f meningitis ( K o f i n a s et al., 1987). Clinically a n d surgically, however, the cyst b e h a v e d as a m o n o d e r m a l t e r a t o m a . N o o t h e r neurological defect has been f o u n d a n d no c o m m u n i c a t i o n to the spinal c o r d was shown; the lack o f a clear p l a n e of cleavage a n d the v a s c u l a r i t y o f the lesion at surgery also f a v o u r the diagnosis o f t e r a t o m a (Smith et al., 1961). W e c o n c l u d e t h a t i m m e d i a t e l y p r e - l a b o u r , percutaneous a s p i r a t i o n is o f g r e a t value in the t r e a t m e n t o f large sacrococcygea! cysts which will a l m o s t certainly cause obstructed labour.
REFERENCES
Edwards, WR (1983). A fetal sacrococcygeal tumor obstructing labor after attempted home confinement. Obstetrics and Gynecology, 61, 19. Gross, SJ, Benzie, R J, Sermer, M, Skidmore, MB & Wilson, SR (1987). Sacrococcygeal teratoma: Prenatal diagnosis and rfianagement. American Journal of Obstetrics and Gynecology, 156, 393-396. Guigiaro, A, Boario, U, Francesco, G & Freni, G (1977). Three cases of intrapartum rupture of sacrococcygeal teratoma (in Italian). Minerva Pediatrica, 29, 1517. Kofinas, AD, Hatjis, CG, Ernest, JM & Parkar, RL (1987). Anterior sacral meningocele in pregnancy. Obstetrics and Gynecology, 69, 441 444. Kuhlmann, RS, Warsof, SL, Levy, DL, Flake, AJ & Harrison, MR (1987). Fetal sacrococcygeal teratoma. Fetal Therapy, 2, 95 100. Lemire, RJ & Beckwith, JB (1982). Pathogenesis of congenital tumors and malformations of the sacrococcygeal region. Teratology, 25, 201 213. Mintz, MC, Mennuti, M & Fishman, M (1983). Prenatal aspiration of sacrococcygeal teratoma. American Journal of Roentgenology, 141, 367 368. Sepulveda, WH (1989). Prenatal sonographic diagnosis of congenital sacrococcygeal teratoma and management. Journal of Perinatal Medicine, 17, 93-97. Smith, B, Passars, E & Clatworthy, HW (1961). The vascular anatomy of sacrococcygeal teratomas: its significance in surgical management. Surgery, 49, 534. Tanaree, P (1982). Delivery obstructed by sacrococcygeal teratoma. American Journal of Obstetrics and Gynaecology, 142, 239. Weiss, DB, Wajntraub, G, Abulafia, Y & Schiller, M (1976). Vaginal surgical intervention for a sacro-coccygeal teratoma obstructing labour. Acta Obstetricia et Gynecologica Scandinavica, 55, 183.
Case Report: In-utero Aspiration of Sacrococcygeal Cyst M. J. W E S T O N
and H. ANDREWS
Department of Radiodiagnosis, Bristol Royal Infirmary, Bristol Pre-natal aspiration, under ultrasound control, was performed on a sacrococcygeal cyst measuring 19 cm in diameter in a 38 week fetus to allow normal vaginal delivery of a live baby girl. This is the first report of such intervention leading to normal delivery. Weston, M.J. & A n d r e w s , H . (1991). Sacrococcygeal Cyst
Clinical Radiology 44, 119-120. C a s e R e p o r t : I n - u t e r o A s p i r a t i o n o f
S k i n - c o v e r e d s a c r o c o c c y g e a l m a s s e s in t h e n e o n a t e are o f varied origin, t h e c o m m o n e s t b e i n g t e r a t o m a s , l i p o m a s and m e n i n g o c o e l e s ( L e m i r e a n d B e c k w i t h , 1982). T h e y may g r o w to a n e n o r m o u s size a n d o b s t r u c t l a b o u r , w i t h c o n s e q u e n t i n c r e a s e in p e r i n a t a l m o r t a l i t y ( G r o s s et al., 1987). T h e r e are n o k n o w n risk f a c t o r s f o r s a c r o c o c c y g e a l cysts, a n d t h u s e~/rly d i a g n o s i s is o n l y p o s s i b l e w h e n routine o b s t e t r i c u l t r a s o u n d is p e r f o r m e d . O t h e r w i s e t h e c o m m o n e s t p r e s e n t a t i o n is ' l a r g e f o r d a t e s ' . F r o m t h e literature it is a p p a r e n t t h a t few s a c r o c o c c y g e a l t e r a t o m a s d i a g n o s e d p r i o r to 30 w e e k s g e s t a t i o n s u r v i v e ( K u h l m a n n et al., 1987). W e r e p o r t a c a s e in w h i c h the early d i a g n o s i s and the successful i n t r a u t e r i n e a s p i r a t i o n o f a s a c r o c o c c y geal cyst a l l o w e d a n o r m a l t r a n s v a g i n a t d e l i v e r y o f a live baby girl.
polyhydramnios or of fetal hydrops. At 38 weeks gestation it measured 19 cm in diameter (Fig. 3). In order to allow a vaginal delivery, percutaneous aspiration of the cyst was undertaken at 38 weeks gestation, prior to induction of labour. A 20 gauge spinal needle was advanced into the cyst under ultrasound control, using aseptic technique. The puncture site was chosen to avoid the anterior lying placenta. Two aspirations were performed on consecutive days. The first yielded 21 of clear, straw coloured fluid and the second 750 ml, resulting in almost complete collapse of the cyst. There was no evidence of any complications.
CASE REPORT A 22-year-old, caucasian, G2 P1, presented for a routine ultrasound examination at 18 weeks, at which time a large sacrococcygeal cystic structure was demonstrated. A 20 weeks gestation a detailed anomaly ultrasound scan revealed that this cystic structure was unilocular and arising from the anterior surface of the coccyx (Fig. 1). It measured 6 cm in diameter and was thin-walled with no internal echoes. There was no evidence of any other fetal abnormality and ultrasound estimation of gestational age corresponded with dates. A diagnosis of a cystic sacrococcygeal teratoma was made. Monthly follow-up ultrasound scans demonstrated normal fetal growth. The sacrococcygeal cyst remained thin-walled and unilocular but showed a gradual increase in size (Fig. 2). There was no evidence of
Fig. 2 - Ultrasound scan showing increasing size of sacrococcygeal cyst.
Fig. 1 -Ultrasound scan of fetus at 20 weeks gestation showing a perineal cystic lesion (arrow). Correspondence to: Dr M. J. Weston, Department of Radiodiagnosis, Bristol Royal Infirmary, Marlborough Street, Bristol BS2 8HW.
Fig. 3 Ultrasound scan at 38 weeks gestation showing the cyst to measure 19 cm in diameter.
120
CLINICAL RADIOLOGY
Fig. 4 - Post-natal picture showing a large empty sac arising from the sacrum. Labour was induced with artificial rupture of membranes and Prostin pessary immediately after the second aspiration. The first stage of labour lasted 80 min and the second 8 min. A baby girl weighing 3.77 kg, with an immediate Apgar score of nine was delivered. A large collapsed skincovered cystic sac overlaying the sacrum was identified (Fig. 4). Two days later the cyst was removed at operation. A plane of dissection was found with difficulty and active bleeding resulted in the loss of 260 ml of blood. On histology the lesion resembled a meningomyelocoele, the cyst being lined with patchy layers of columnar epithelium resembling choroid plexus, neuroglial tissue, nerves and collagen. It could be also described as a monodermal teratoma (i.e. unilateral differentiation of tissues derived from one of the germ cell layers). No communication with the spinal cord was shown. Radiographs of the spine showed no significant bony abnormality. The baby's post=operative course was uneventful. There was normal tone and movement in the limbs and an ultrasound scan of the brain was normal. She was discharged home at 18 days of age. DISCUSSION S a c r o c o c c y g e a l t u m o u r s are rare with a frequency o f only one o r two p e r 40 000 live births with the m a j o r i t y occurring in females. S a c r o c o c c y g e a l t e r a t o m a c a n be complicated by non-immune hydrops and placentomegaly, a n d these cases c a r r y a p o o r prognosis. Sacrococcygeal t e r a t o m a s have a n 18% a s s o c i a t e d a n o m a l y rate ( K u h l m a n n et al., 1987) with s p i n a bifida, meningocoele, v a g i n a l stenosis, t r a c h e o - o e s o p h a g e a l fistula, ventricular septal defect a n d m a x i l l o f a c i a l a n o m a l i e s . P r e - n a t a l diagnosis is beneficial in a v o i d i n g the u n e x p e c t e d development of obstructed labour. N e o n a t a l d e a t h has followed r u p t u r e o f sacrococeygeal t e r a t o m a s d u r i n g vaginal delivery ( G u i g i a r o et al., 1977). I f the diagnosis is m a d e a n t e n a t a l l y , the literature a d v o cates elective C a e s a r e a n section ( G r o s s et al., 1987; Sepulveda, 1989). W h e n the d i a g n o s i s o f sacrococcygeal t u m o u r is m a d e d u r i n g o b s t r u c t e d v a g i n a l l a b o u r , others have surgically d e c o m p r e s s e d the t u m o u r t r a n s a b d o m i nally, at e m e r g e n c y o p e r a t i o n , in o r d e r to allow vaginal delivery (Tanaree, 1982; E d w a r d s , 1983). D r a i n a g e p e r vaginum, given the a p p r o p r i a t e fetal p r e s e n t a t i o n , has
also been p e r f o r m e d as an e m e r g e n c y (Weiss et al., 1976). U l t r a s o u n d shows the m a j o r i t y o f s a c r o c o c c y g e a l teratom a s to have a large solid c o m p o n e n t , t h o u g h p r e d o m i n antly cystic lesions m a y also occur, a n d it is in the latter lesions t h a t p l a n n e d i n t r a u t e r i n e i n t e r v e n t i o n b y aspir a t i o n m a y allow a n o r m a l vaginal delivery. M i n t z et al. (1983) r e p o r t e d p e r c u t a n e o u s a s p i r a t i o n i m m e d i a t e l y p r i o r to elective C a e s a r i a n section, b u t we believe o u r s is the first r e p o r t o f n o r m a l vaginal delivery following such aspiration. A definite histological diagnosis m a y be difficult ( L e m i r e a n d Beckwith, 1982). H i s t o l o g i c a l l y this lesion has the characteristics o f a m y e l o m e n i n g o c o e t e . This raises the possibility t h a t there will be t e t h e r i n g o f the spinal c o r d o r an increased risk o f meningitiS later in life. In the adult, p e r e u t a n e o u s a s p i r a t i o n o f an a n t e r i o r sacral m e n i n g o c o e l e carries a high risk o f meningitis ( K o f i n a s et al., 1987). Clinically a n d surgically, however, the cyst b e h a v e d as a m o n o d e r m a l t e r a t o m a . N o o t h e r neurological defect has been f o u n d a n d no c o m m u n i c a t i o n to the spinal c o r d was shown; the lack o f a clear p l a n e of cleavage a n d the v a s c u l a r i t y o f the lesion at surgery also f a v o u r the diagnosis o f t e r a t o m a (Smith et al., 1961). W e c o n c l u d e t h a t i m m e d i a t e l y p r e - l a b o u r , percutaneous a s p i r a t i o n is o f g r e a t value in the t r e a t m e n t o f large sacrococcygea! cysts which will a l m o s t certainly cause obstructed labour.
REFERENCES
Edwards, WR (1983). A fetal sacrococcygeal tumor obstructing labor after attempted home confinement. Obstetrics and Gynecology, 61, 19. Gross, SJ, Benzie, R J, Sermer, M, Skidmore, MB & Wilson, SR (1987). Sacrococcygeal teratoma: Prenatal diagnosis and rfianagement. American Journal of Obstetrics and Gynecology, 156, 393-396. Guigiaro, A, Boario, U, Francesco, G & Freni, G (1977). Three cases of intrapartum rupture of sacrococcygeal teratoma (in Italian). Minerva Pediatrica, 29, 1517. Kofinas, AD, Hatjis, CG, Ernest, JM & Parkar, RL (1987). Anterior sacral meningocele in pregnancy. Obstetrics and Gynecology, 69, 441 444. Kuhlmann, RS, Warsof, SL, Levy, DL, Flake, AJ & Harrison, MR (1987). Fetal sacrococcygeal teratoma. Fetal Therapy, 2, 95 100. Lemire, RJ & Beckwith, JB (1982). Pathogenesis of congenital tumors and malformations of the sacrococcygeal region. Teratology, 25, 201 213. Mintz, MC, Mennuti, M & Fishman, M (1983). Prenatal aspiration of sacrococcygeal teratoma. American Journal of Roentgenology, 141, 367 368. Sepulveda, WH (1989). Prenatal sonographic diagnosis of congenital sacrococcygeal teratoma and management. Journal of Perinatal Medicine, 17, 93-97. Smith, B, Passars, E & Clatworthy, HW (1961). The vascular anatomy of sacrococcygeal teratomas: its significance in surgical management. Surgery, 49, 534. Tanaree, P (1982). Delivery obstructed by sacrococcygeal teratoma. American Journal of Obstetrics and Gynaecology, 142, 239. Weiss, DB, Wajntraub, G, Abulafia, Y & Schiller, M (1976). Vaginal surgical intervention for a sacro-coccygeal teratoma obstructing labour. Acta Obstetricia et Gynecologica Scandinavica, 55, 183.