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Tailgut cyst in a neonate
Tailgut Cyst in a Neonate By Jung-Tak Oh, Suk Woo Son, Myung Joon Kim, Lucia Kim, Hoguen Kim, and Eui Ho Hwang Seoul, Korea
Tailgut cyst is a rare lesion of developmental origin located in the retrorectal space, which usually presents as a multilocular cystic mass. It is usually found in adults, and neonatal cases are extremely rare. The authors report a tailgut cyst in a neonate that was found by prenatal ultra-
sonogram, which was like a teratoma in gross appearance. J Pediatr Surg 35:1833-1835. Copyright © 2000 by W.B. Saunders Company.
T
by the 35-mm stage (56 days gestational age). The anus is formed cephalad to the tail. Because the primitive gut extends into the tail beyond the point at which the anus develops, it is called the tailgut. Remnants of the tailgut may result in congenital cysts in this region, or the neuroenteric cord may be the source.1 It is a multilocular mass located between the rectum, sacrum, and coccyx. The size of mass is several centimeters in diameter and it usually is confined to the retrorectal space.1 These findings are the reason that tailgut cysts are usually found in adults despite their congenital origin. It is predominant in females. Hjermstad and Helwig1 reported a 3.1:1 female to male ratio. The reason for such a female predilection is unclear, but similar female predilection is present in sacrococcygeal teratoma of children.10 Typical symptoms of tailgut cysts are closely linked to their site. Many adult patients complain of discomfort while sitting or of persistent pain after a fall on their buttocks. Other symptoms described have included urinary retention, dysuria, changes in caliber of stool, and rectal bleeding.5,7,8 Because of the location, the tailgut cyst almost always can be palpated by rectal examination. Additional investigation should include barium enema, endosonography, computed tomography (CT) and magnetic resonance imaging (MRI).6
AILGUT CYST is a rare congenital disorder located in the retrorectal space and usually presents as a multilocular cystic mass. It is believed to arise from a remnant of embryonic tailgut, and neonatal cases are extremely rare. We report a tailgut cyst in a neonate that could be removed completely. CASE REPORT A 3.1-kg baby girl was born at 39 weeks’ gestation by cesarean section delivery. At 36 weeks’ gestation, prenatal ultrasonography was performed, and a large sacrococcygeal mass was found. After birth, the patient was healthy except for a round cystic mass on the sacrococcygeal area (Fig 1). Its diameter was 9 cm at the largest point. Barium enema results showed extrinsic mass of sacrococcygeal area and no correlation with the rectum and sigmoid colon. Magnetic resonance imaging (MRI) was carried out and showed a large well-demarcated cystic mass with a few small septate cysts in the presacral and sacrococcygeal region. There was no solid component within the cystic mass (Fig 2). At the age of 4 days, under the impression of a cystic sacrococcygeal teratoma, the patient underwent complete surgical excision including removal of the coccyx. Operative findings were compatible with MRI findings. The muscles surrounding the lesion were thin, but they were dissected easily from the mass. Microscopic finding showed a multilocular cyst lined by ciliated columnar epithelium (Fig 3). Those findings were compatible with a tailgut cyst. The patient was discharged on the seventh postoperative day. The patient has been doing well 1 year after treatment (Fig 4).
DISCUSSION
Tailgut cyst is a rare congenital disorder originating from the embryonic tailgut. It usually is found in adults, and neonatal cases are extremely rare. In the Englishlanguage literature, Hjermstad and Helwig1 reported 53 cases of tailgut cysts, but only 2 of their cases were younger than 10 years of age. Other reports about tailgut cysts have small numbers, and we could not find a neonatal tailgut cyst similar to our case.2-9 Early in development, the embryo possesses a true tail. This is maximally developed around the 8-mm stage (35 days gestational age) and usually completely regresses
INDEX WORDS: Tailgut cyst, neonate.
From the Departments of Pediatric Surgery, Radiology, and Pathology, Yonsei University College of Medicine, Seoul, Korea. Address reprint requests to Eui Ho Hwang, MD, Department of Pediatric Surgery, Yonsei University College of Medicine, CPO Box 8044, Seoul, Korea. Copyright © 2000 by W.B. Saunders Company 0022-3468/00/3512-0033$03.00/0 doi:10.1053/jpsu.2000.19281
Journal of Pediatric Surgery, Vol 35, No 12 (December), 2000: pp 1833-1835
1833
1834
OH ET AL
Fig 1. Photograph shows huge sacrococcygeal mass. Fig 3. Microscopic pathologic finding shows a multilocular cyst lined by ciliated columnar epithelium and squamous epithelium.
Barium enema is useful to initially show the lesion, and transrectal sonography can show the cystic nature of the mass. For evaluation of a retrorectal mass, MRI may have both disadvantages and advantages over CT. MRI is limited in detecting calcification in lesions, particularly when it would be helpful in excluding dermoid cyst or teratoma. However, MRI can reliably diagnose fatty tumors using fat suppression technique, and, with sagittal scans, the relationship of the mass to the surrounding rectum and bony structure can be assessed easily.6,11 Microscopically, the lining of the cysts ranged from a single layer of epithelium without crypts or villi to a
Fig 2. T1-weighted sagittal MR image (TR/TE:500/11) with fat suppression shows a large well-demarcated cystic mass with a few small septate cysts in the presacral and sacrococcygeal region.
wide range of epithelial types ordinarily found in the adult and fetal gastrointestinal tract. The cyst may have areas of smooth muscle, but it does not possess a myenteric plexus or serosa. Those are important features that make differential diagnoses from teratoma, dermoid cyst, enteric cyst and other retrorectal lesions.1 In our case, lining epithelia were squamous epithelium and ciliated columnar epithelium. Other findings were compatible with criteria of tailgut cyst. Hjermstad and Helwig1 reported that squamous epithelium, present in 75% of the cysts, was the most common type, and 50% of the cysts were lined with ciliated columnar epithelium. Complete surgical excision is the treatment of choice for all tailgut cysts. If complete excision is not undertaken, remnants of the lesion can become infected or malignant.1,4,9 In our experience, neonatal tailgut cyst can be excised more easily than adult tailgut cyst because of rare inflammatory change.
Fig 4. Gross appearance of sacrococcygeal area 1 year after resection.
TAILGUT CYST
1835
REFERENCES 1. Hjermstad BM, Helwig EB: Tailgut cysts. Report of 53 cases. Am J Clin Pathol 89:139-147, 1988 2. Caropreso PR, Wengert PA Jr, Milford HE: Tailgut cyst—A rare retrorectal tumor. Dis Colon Rectum 18:597-600, 1975 3. Hutton KAR, Benson EA: Case report: Tailgut cyst—Assessment with transrectal ultrasound. Clin Radiol 45:288-289, 1992 4. Fujitaka T, Nakayama H, Fukuda S, et al: A tailgut cyst found accompanying rectal cancer: Report of a case. Surgery Today (Jpn J Surg) 25:65-67, 1995 5. Levert LM, Rooyen WV, Bergen HAVD: Cysts of the tailgut. Eur J Surg 162:149-152, 1996 6. Kim MJ, Kim WH, Kim NK, et al: Tailgut cyst: Multilocular
cystic appearance on MRI. J Comput Assist Tomogr 21:731-732, 1997 7. Pyo DJ: Tailgut cyst (retrorectal hamartoma): Case report and review. Mt Sinai J Med 57:249-252, 1990 8. Uhlig BE, Johnson RL: Presacral tumors and cysts in adults. Dis Colon Rectum 18:581-596, 1975 9. Maruyama A, Murabayashi K, Hayashi M, et al: Adenocarcinoma arising in a tailgut cyst: Report of a case. Surg Today 28:1319-1322, 1998 10. Woolley MM: Teratomas, in Ashcraft KW, Holder TM (eds): Pediatric Surgery. Philadelphia, PA, Saunders, 1993, pp 849-855 11. Liessi G, Cesari S, Pavanello M, et al: Tailgut cysts: CT and MR findings. Abdom Imag 20:256-258, 1995
Tailgut cyst is a rare lesion of developmental origin located in the retrorectal space, which usually presents as a multilocular cystic mass. It is usually found in adults, and neonatal cases are extremely rare. The authors report a tailgut cyst in a neonate that was found by prenatal ultra-
sonogram, which was like a teratoma in gross appearance. J Pediatr Surg 35:1833-1835. Copyright © 2000 by W.B. Saunders Company.
T
by the 35-mm stage (56 days gestational age). The anus is formed cephalad to the tail. Because the primitive gut extends into the tail beyond the point at which the anus develops, it is called the tailgut. Remnants of the tailgut may result in congenital cysts in this region, or the neuroenteric cord may be the source.1 It is a multilocular mass located between the rectum, sacrum, and coccyx. The size of mass is several centimeters in diameter and it usually is confined to the retrorectal space.1 These findings are the reason that tailgut cysts are usually found in adults despite their congenital origin. It is predominant in females. Hjermstad and Helwig1 reported a 3.1:1 female to male ratio. The reason for such a female predilection is unclear, but similar female predilection is present in sacrococcygeal teratoma of children.10 Typical symptoms of tailgut cysts are closely linked to their site. Many adult patients complain of discomfort while sitting or of persistent pain after a fall on their buttocks. Other symptoms described have included urinary retention, dysuria, changes in caliber of stool, and rectal bleeding.5,7,8 Because of the location, the tailgut cyst almost always can be palpated by rectal examination. Additional investigation should include barium enema, endosonography, computed tomography (CT) and magnetic resonance imaging (MRI).6
AILGUT CYST is a rare congenital disorder located in the retrorectal space and usually presents as a multilocular cystic mass. It is believed to arise from a remnant of embryonic tailgut, and neonatal cases are extremely rare. We report a tailgut cyst in a neonate that could be removed completely. CASE REPORT A 3.1-kg baby girl was born at 39 weeks’ gestation by cesarean section delivery. At 36 weeks’ gestation, prenatal ultrasonography was performed, and a large sacrococcygeal mass was found. After birth, the patient was healthy except for a round cystic mass on the sacrococcygeal area (Fig 1). Its diameter was 9 cm at the largest point. Barium enema results showed extrinsic mass of sacrococcygeal area and no correlation with the rectum and sigmoid colon. Magnetic resonance imaging (MRI) was carried out and showed a large well-demarcated cystic mass with a few small septate cysts in the presacral and sacrococcygeal region. There was no solid component within the cystic mass (Fig 2). At the age of 4 days, under the impression of a cystic sacrococcygeal teratoma, the patient underwent complete surgical excision including removal of the coccyx. Operative findings were compatible with MRI findings. The muscles surrounding the lesion were thin, but they were dissected easily from the mass. Microscopic finding showed a multilocular cyst lined by ciliated columnar epithelium (Fig 3). Those findings were compatible with a tailgut cyst. The patient was discharged on the seventh postoperative day. The patient has been doing well 1 year after treatment (Fig 4).
DISCUSSION
Tailgut cyst is a rare congenital disorder originating from the embryonic tailgut. It usually is found in adults, and neonatal cases are extremely rare. In the Englishlanguage literature, Hjermstad and Helwig1 reported 53 cases of tailgut cysts, but only 2 of their cases were younger than 10 years of age. Other reports about tailgut cysts have small numbers, and we could not find a neonatal tailgut cyst similar to our case.2-9 Early in development, the embryo possesses a true tail. This is maximally developed around the 8-mm stage (35 days gestational age) and usually completely regresses
INDEX WORDS: Tailgut cyst, neonate.
From the Departments of Pediatric Surgery, Radiology, and Pathology, Yonsei University College of Medicine, Seoul, Korea. Address reprint requests to Eui Ho Hwang, MD, Department of Pediatric Surgery, Yonsei University College of Medicine, CPO Box 8044, Seoul, Korea. Copyright © 2000 by W.B. Saunders Company 0022-3468/00/3512-0033$03.00/0 doi:10.1053/jpsu.2000.19281
Journal of Pediatric Surgery, Vol 35, No 12 (December), 2000: pp 1833-1835
1833
1834
OH ET AL
Fig 1. Photograph shows huge sacrococcygeal mass. Fig 3. Microscopic pathologic finding shows a multilocular cyst lined by ciliated columnar epithelium and squamous epithelium.
Barium enema is useful to initially show the lesion, and transrectal sonography can show the cystic nature of the mass. For evaluation of a retrorectal mass, MRI may have both disadvantages and advantages over CT. MRI is limited in detecting calcification in lesions, particularly when it would be helpful in excluding dermoid cyst or teratoma. However, MRI can reliably diagnose fatty tumors using fat suppression technique, and, with sagittal scans, the relationship of the mass to the surrounding rectum and bony structure can be assessed easily.6,11 Microscopically, the lining of the cysts ranged from a single layer of epithelium without crypts or villi to a
Fig 2. T1-weighted sagittal MR image (TR/TE:500/11) with fat suppression shows a large well-demarcated cystic mass with a few small septate cysts in the presacral and sacrococcygeal region.
wide range of epithelial types ordinarily found in the adult and fetal gastrointestinal tract. The cyst may have areas of smooth muscle, but it does not possess a myenteric plexus or serosa. Those are important features that make differential diagnoses from teratoma, dermoid cyst, enteric cyst and other retrorectal lesions.1 In our case, lining epithelia were squamous epithelium and ciliated columnar epithelium. Other findings were compatible with criteria of tailgut cyst. Hjermstad and Helwig1 reported that squamous epithelium, present in 75% of the cysts, was the most common type, and 50% of the cysts were lined with ciliated columnar epithelium. Complete surgical excision is the treatment of choice for all tailgut cysts. If complete excision is not undertaken, remnants of the lesion can become infected or malignant.1,4,9 In our experience, neonatal tailgut cyst can be excised more easily than adult tailgut cyst because of rare inflammatory change.
Fig 4. Gross appearance of sacrococcygeal area 1 year after resection.
TAILGUT CYST
1835
REFERENCES 1. Hjermstad BM, Helwig EB: Tailgut cysts. Report of 53 cases. Am J Clin Pathol 89:139-147, 1988 2. Caropreso PR, Wengert PA Jr, Milford HE: Tailgut cyst—A rare retrorectal tumor. Dis Colon Rectum 18:597-600, 1975 3. Hutton KAR, Benson EA: Case report: Tailgut cyst—Assessment with transrectal ultrasound. Clin Radiol 45:288-289, 1992 4. Fujitaka T, Nakayama H, Fukuda S, et al: A tailgut cyst found accompanying rectal cancer: Report of a case. Surgery Today (Jpn J Surg) 25:65-67, 1995 5. Levert LM, Rooyen WV, Bergen HAVD: Cysts of the tailgut. Eur J Surg 162:149-152, 1996 6. Kim MJ, Kim WH, Kim NK, et al: Tailgut cyst: Multilocular
cystic appearance on MRI. J Comput Assist Tomogr 21:731-732, 1997 7. Pyo DJ: Tailgut cyst (retrorectal hamartoma): Case report and review. Mt Sinai J Med 57:249-252, 1990 8. Uhlig BE, Johnson RL: Presacral tumors and cysts in adults. Dis Colon Rectum 18:581-596, 1975 9. Maruyama A, Murabayashi K, Hayashi M, et al: Adenocarcinoma arising in a tailgut cyst: Report of a case. Surg Today 28:1319-1322, 1998 10. Woolley MM: Teratomas, in Ashcraft KW, Holder TM (eds): Pediatric Surgery. Philadelphia, PA, Saunders, 1993, pp 849-855 11. Liessi G, Cesari S, Pavanello M, et al: Tailgut cysts: CT and MR findings. Abdom Imag 20:256-258, 1995