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| {{Sacrococcygeal teratoma}} | | {{Sacrococcygeal teratoma}} |
| {{CMG}} | | {{CMG}}{{AE}}{{MGS}}{{sab}} |
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| | {{SK}} Fetiform Teratoma, Fetus in fetu |
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| ==[[Sacrococcygeal teratoma overview|Overview]]== | | ==[[Sacrococcygeal teratoma overview|Overview]]== |
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| ==[[Sacrococcygeal teratoma natural history, complications, and prognosis|Natural history, Complications, and Prognosis]]== | | ==[[Sacrococcygeal teratoma historical perspective|Historical Perspective]]== |
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| ==Diagnosis==
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| ==[[Sacrococcygeal teratoma history and symptoms|History & Symptoms]]==
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| ==[[Sacrococcygeal teratoma physical examination|Physical Examination]]==
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| ==Lab tests==
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| ==[[Sacrococcygeal teratoma echocardiography or ultrasound|Echocardiography or Ultrasound]]==
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| During [[prenatal]] [[ultrasound]], an SCT having an external component may appear as a fluid-filled cyst or a solid mass sticking out from the fetus's buttocks. Fetal SCTs that are entirely internal may be undetected if they are small; detection (or at least suspicion) is possible when the fetal bladder is seen in an abnormal position, due to the SCT pushing other organs out of place.
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| Other tumors can occur in the sacrococcygeal and/or presacral regions<ref name="pmid6366733">{{cite journal
| | ==[[Sacrococcygeal teratoma classification|Classification]]== |
| | author = Bale PM
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| | title = Sacrococcygeal developmental abnormalities and tumors in children.
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| | journal = Perspectives in pediatric pathology
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| | volume = 8
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| | issue = 1
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| | pages = 9-56
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| | year = 1984
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| | pmid = 6366733
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| | doi =
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| | issn =
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| }}</ref> and hence must be ruled out to obtain a [[differential diagnosis]]. These include [[ependymoma|extraspinal ependymoma]]<ref name="pmid10726703">{{cite journal
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| | author = Aktuğ T, Hakgüder G, Sarioğlu S, Akgür FM, Olguner M, Pabuçcuoğlu U
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| | title = Sacrococcygeal extraspinal ependymomas: the role of coccygectomy.
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| | journal = J. Pediatr. Surg.
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| | volume = 35
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| | issue = 3
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| | pages = 515-8
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| | year = 2000
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| | pmid = 10726703
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| | doi =
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| | issn =
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| }}</ref>, [[neuroblastoma]] and [[rhabdomyosarcoma]]. Smaller SCTs with an external component, seen in prenatal ultrasounds or at birth, often are mistaken for [[spina bifida]].
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| ==Treatment== | | ==[[Sacrococcygeal teratoma pathophysiology|Pathophysiology]]== |
| The preferred first treatment for SCT is complete surgical removal (ie, complete resection). The preferred approach to a small SCT is through the [[perineum]]; a large SCT may require an additional approach through the [[abdomen]]. Resection should include the coccyx and may also include portions of the [[sacrum]]. The surgery should include reattachment of the small muscles and ligaments formerly attached to the coccyx, in effect reconstructing the posterior perineum.
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| SCTs are classified morphologically according to how much of them is outside or inside the body:
| | ==[[Sacrococcygeal teratoma causes|Causes]]== |
| *Altman Type I — entirely external, sometimes attached to the body only by a narrow '''stalk'''
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| *Altman Type II
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| *Altman Type III
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| *Altman Type IV — entirely internal; this is a '''presacral teratoma'''
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| The Altman Type has a significant influence on management, surgical approach, and complications of SCT. Serial ultrasound and MRI monitoring of SCTs in fetuses [[in utero]] has demonstrated that the Altman Type can change over time.
| | ==[[Sacrococcygeal teratoma differential diagnosis|Differentiating Sacrococcygeal teratoma from other Diseases]]== |
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| Regardless of location in the body, [[teratoma]]s are classified according to a [[cancer staging]] system. This indicates whether [[chemotherapy]] or [[radiation therapy]] may be needed.
| | ==[[Sacrococcygeal teratoma epidemiology and demographics|Epidemiology and Demographics]]== |
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| ==Management of fetal SCTs== | | ==[[Sacrococcygeal teratoma risk factors|Risk Factors]]== |
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| Management of most fetal SCTs involves [[watch and wait]], also known as [[expectant management]]. An often used [[decision tree]] is as follows:
| | ==[[Sacrococcygeal teratoma screening|Screening]]== |
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| *Perform detailed ultrasound exam including fetal [[echocardiogram]] and [[Doppler]] [[Data-flow analysis|flow analysis]]
| | ==[[Sacrococcygeal teratoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| **If fetal high output failure, placentomegaly, or [[hydrops]]
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| ***If fetus not mature, perform [[pregnancy termination]] or [[fetal intervention]]
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| ***Else fetus mature, perform emergency [[Cesarean section]]
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| **Else no emergent problems, perform serial [[non-stress test]]s and ultrasound [[biophysical profile]]s and plan delivery
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| ***If emergent problems develop, return to top of decision tree
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| ***Else if SCT over 5–10cm or [[polyhydramnios]], perform early (37 weeks gestation) [[Caesarean delivery on maternal request|elective Cesarean]] section
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| ***Else SCT small and no complications, permit term [[spontaneous vaginal delivery]]
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| Emergent problems include maternal [[mirror syndrome]], [[polyhydramnios]], and [[preterm labor]]. Poor management decisions, including interventions that are either premature or delayed, can have dire consequences.<ref name="pmid11965727">{{cite journal
| | ==Diagnosis== |
| | author = Mazneĭkova V, Dimitrova V | | [[Sacrococcygeal teratoma history and symptoms|History & Symptoms]] | [[Sacrococcygeal teratoma physical examination|Physical Examination]] | [[Sacrococcygeal teratoma laboratory tests|Laboratory Tests]] | [[Sacrococcygeal teratoma pelvis x ray|Pelvis X Ray]] | [[Sacrococcygeal teratoma CT|CT]] | [[Sacrococcygeal teratoma MRI|MRI]] | [[Sacrococcygeal teratoma echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Sacrococcygeal teratoma other imaging findings|Other Imaging Findings]] | [[Sacrococcygeal teratoma other diagnostic studies|Other Diagnostic Studies]] |
| | title = [Prenatal ultrasonographic diagnosis of four cases of sacrococcygeal teratoma]
| | ==Treatment== |
| | language = Bulgarian | | [[Sacrococcygeal teratoma medical therapy|Medical Therapy]] | [[Sacrococcygeal teratoma surgery|Surgery]] | [[Sacrococcygeal teratoma primary prevention|Primary Prevention]] | [[Sacrococcygeal teratoma secondary prevention|Secondary Prevention]] | [[Sacrococcygeal teratoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Sacrococcygeal teratoma future or investigational therapies|Future or Investigational Therapies]] |
| | journal = Akusherstvo i ginekologii͡a
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| | volume = 38
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| | issue = 1
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| | pages = 64-9
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| | year = 1999
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| | pmid = 11965727
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| | doi =
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| | issn =
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| }}</ref><ref name="pmid17525561">{{cite journal
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| | author = Sheil AT, Collins KA
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| | title = Fatal birth trauma due to an undiagnosed abdominal teratoma: case report and review of the literature
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| | journal = The American journal of forensic medicine and pathology : official publication of the National Association of Medical Examiners
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| | volume = 28
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| | issue = 2
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| | pages = 121-7
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| | year = 2007
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| | pmid = 17525561
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| | doi = 10.1097/01.paf.0000257373.91126.0d
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| | issn =
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| }}</ref>
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| In many cases, a fetus with a small SCT (under 5 or 10 cm) may be delivered vaginally.<ref name="pmid12475573">{{cite journal
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| | author = Anteby EY, Yagel S
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| | title = Route of delivery of fetuses with structural anomalies
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| | journal = Eur. J. Obstet. Gynecol. Reprod. Biol.
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| | volume = 106
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| | issue = 1
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| | pages = 5-9
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| | year = 2003
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| | pmid = 12475573
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| | doi =
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| | issn =
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| }}</ref><ref name="pmid11336088">{{cite journal
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| | author = Ruangtrakool R, Nitipon A, Laohapensang M, ''et al''
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| | title = Sacrococcygeal teratoma: 25 year experience
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| | journal = Journal of the Medical Association of Thailand = Chotmaihet thangphaet | |
| | volume = 84 | |
| | issue = 2
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| | pages = 265-73
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| | year = 2001
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| | pmid = 11336088
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| | doi =
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| | issn =
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| }}</ref><ref name="pmid8458172">{{cite journal
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| | author = McCurdy CM, Seeds JW
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| | title = Route of delivery of infants with congenital anomalies
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| | journal = Clinics in perinatology
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| | volume = 20
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| | issue = 1
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| | pages = 81-106
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| | year = 1993
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| | pmid = 8458172
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| | doi =
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| | issn =
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| }}</ref><ref name="pmid2205995">{{cite journal
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| | author = Kainer F, Winter R, Hofmann HM, Karpf EF
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| | title = [Sacrococcygeal teratoma. Prenatal diagnosis and prognosis]
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| | language = German | |
| | journal = Zentralblatt für Gynäkologie
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| | volume = 112
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| | issue = 10
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| | pages = 609-16
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| | year = 1990
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| | pmid = 2205995
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| | doi =
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| | issn =
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| }}</ref>
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| ==Complications==
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| Maternal complications of '''pregnancy''' may include [[mirror syndrome]].<ref name="pmid17465292">{{cite journal
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| | author = Finamore PS, Kontopoulos E, Price M, Giannina G, Smulian JC
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| | title = Mirror syndrome associated with sacrococcygeal teratoma: a case report
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| | journal = The Journal of reproductive medicine | |
| | volume = 52
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| | issue = 3
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| | pages = 225-7
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| | year = 2007
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| | pmid = 17465292
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| | doi =
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| | issn =
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| }}</ref> Maternal complications of '''delivery''' may include a [[Cesarean section]] or, alternatively, a vaginal delivery with mechanical [[dystocia]].<ref name="pmid17469451">{{cite journal
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| | author = Nalbanski B, Markov D, Brankov O | |
| | title = [Sacrococcygeal teratoma--a case report and literature review]
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| | language = Bulgarian
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| | journal = Akusherstvo i ginekologii͡a
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| | volume = 46
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| | issue = 2
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| | pages = 41-5
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| | year = 2007
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| | pmid = 17469451
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| | doi =
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| | issn =
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| }}</ref>
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| Complications of the mass effect of a teratoma in general are addressed on the [[teratoma]] page. Complications of the mass effect of a large SCT may include [[hip dysplasia]], [[bowel obstruction]], [[urinary obstruction]], [[hydronephrosis]] and [[hydrops fetalis]]. Even a small SCT can produce complications of mass effect, if it is presacral (Altman Type IV).<ref name="pmid15750911">{{cite journal
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| | author = Galili O, Mogilner J
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| | title = Type IV sacrococcygeal teratoma causing urinary retention: a rare presentation.
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| | journal = J. Pediatr. Surg.
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| | volume = 40
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| | issue = 2
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| | pages = E18-20 | |
| | year = 2005
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| | pmid = 15750911
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| | doi = 10.1016/j.jpedsurg.2004.10.003
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| | issn = | |
| }}</ref> In the fetus, severe hydronephrosis may contribute to inadequate lung development. Also in the fetus and newborn, the [[anus]] may be [[imperforate anus|imperforate]].
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| Later complications of the mass effect and/or surgery may include [[neurogenic bladder]], other forms of [[urinary incontinence]], [[fecal incontinence]], and other chronic problems resulting from accidental damage to or sacrifice of nerves and muscles within the pelvis.<ref name="pmid3439358">{{cite journal
| | ==Case Studies== |
| | author = Engelskirchen R, Holschneider AM, Rhein R, Hecker WC, Höpner F
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| | title = [Sacral teratomas in childhood. An analysis of long-term results in 87 children]
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| | language = German
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| | journal = Zeitschrift für Kinderchirurgie : organ der Deutschen, der Schweizerischen und der Osterreichischen Gesellschaft für Kinderchirurgie = Surgery in infancy and childhood
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| | volume = 42
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| | issue = 6
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| | pages = 358-61
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| | year = 1987
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| | pmid = 3439358
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| | doi =
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| | issn =
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| }}</ref> [[coccygectomy|Removal of the coccyx]] may include additional complications. In one review of 25 patients,<ref>[http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Retrieve&db=PubMed&list_uids= 16373161&dopt=AbstractPlus PubMed]</ref> however, the most frequent complication was an unsatisfactory appearance of the surgical scar.
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| ===Late effects===
| | [[Sacrococcygeal teratoma case study one|Case #1]] |
| Pelvic scarring may necessitate that [[Pregnancy|pregnant women]] who are SCT survivors deliver their babies by [Cesarean section].{{Fact|date=June 2007}}
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| Complications of not removing the coccyx may include both recurrence of the teratoma<ref name="Lahdenne+1993">{{cite journal
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| | author = Lahdenne P, Heikinheimo M, Nikkanen V, Klemi P, Siimes MA, Rapola J
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| | title = Neonatal benign sacrococcygeal teratoma may recur in adulthood and give rise to malignancy.
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| | journal = Cancer
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| | volume = 72
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| | issue = 12
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| | pages = 3727-31
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| | year = 1993
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| | pmid = 8252490
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| | doi =
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| }} Synopsis: 45 survivors of infant SCT were followed up. Two reported recurrent benign teratoma and one reported metastatic adenocarcinoma originating from the residual coccyx. They were aged 21-43 at diagnosis.</ref> and [[Metastasis|metastatic cancer]].<ref name="Lahdenne+1993"/><ref name="Lack+1993">{{cite journal
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| | author = Lack EE, Glaun RS, Hefter LG, Seneca RP, Steigman C, Athari F
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| | title = Late occurrence of malignancy following resection of a histologically mature sacrococcygeal teratoma. Report of a case and literature review.
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| | journal = Arch. Pathol. Lab. Med.
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| | volume = 117
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| | issue = 7
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| | pages = 724-8
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| | year = 1993
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| | pmid = 8323438
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| | doi =
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| }} Synopsis: A 40 year old man has widely metastatic [[adenocarcinoma]] arising from the residual coccyx remaining after surgical removal of an apparently benign SCT at age 2 months.</ref> Late malignancies usually involve incomplete excision of the coccyx and are [[adenocarcinoma]].
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| ==See also== | | ==See also== |
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| {{Pathology|state=collapsed}} | | {{Pathology|state=collapsed}} |
| {{Joints of torso|state=collapsed}} | | {{Joints of torso|state=collapsed}} |
| {{SIB}}
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| [[Category:Congenital disorders]] | | [[Category:Congenital disorders]] |
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