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| | {{Infobox medical condition |
| | | name = Caudal duplication |
| | | synonyms = Caudal duplication syndrome, Dipygus |
| | | field = [[Pediatrics]], [[Genetics]] |
| | | symptoms = Duplication of [[pelvic organs]], [[spinal cord]], [[lower limbs]] |
| | | complications = [[Urinary tract infections]], [[bowel obstruction]], [[spinal cord abnormalities]] |
| | | onset = [[Congenital]] |
| | | duration = [[Lifelong]] |
| | | causes = [[Genetic mutation]], [[developmental anomaly]] |
| | | risks = [[Family history]], [[genetic predisposition]] |
| | | diagnosis = [[Prenatal ultrasound]], [[MRI]], [[CT scan]] |
| | | differential = [[Sirenomelia]], [[spina bifida]], [[sacral agenesis]] |
| | | treatment = [[Surgical intervention]], [[multidisciplinary care]] |
| | | prognosis = Varies depending on severity and associated anomalies |
| | | frequency = Extremely rare |
| | }} |
| | {{Short description|A rare congenital disorder}} |
| {{Infobox medical condition | | {{Infobox medical condition |
| |name = | | | name = Split notochord syndrome |
| |synonym = Split notochord syndrome<ref>{{cite web |last1=RESERVED |first1=INSERM US14-- ALL RIGHTS |title=Orphanet: Caudal duplication |url=https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=1756 |website=www.orpha.net |accessdate=27 October 2019 |language=en}}</ref>
| | | specialty = [[Pediatrics]], [[Neurosurgery]], [[Medical genetics]] |
| |image =
| | | symptoms = [[Spinal dysraphism]], [[gastrointestinal tract duplication]], [[neural tube defects]], [[hydrocephalus]], [[meningocele]], [[myelomeningocele]] |
| |image_size =
| | | complications = Neurological deficits, bowel or bladder dysfunction, infections, developmental delays |
| |alt =
| | | onset = Congenital (present at birth) |
| |caption =
| | | duration = Lifelong |
| |pronounce =
| | | types = Varies in presentation depending on extent of spinal and gastrointestinal involvement |
| |specialty = <!--from Wikidata; can be overwritten--> | | | causes = Congenital malformation due to abnormal embryonic development of the [[notochord]] and [[endoderm]] |
| |symptoms = | |
| |complications = | |
| |onset = | |
| |duration = | |
| |types = | |
| |causes = | |
| }} | | }} |
| '''Caudal duplication''', (or caudal duplication syndrome) is a rare [[Birth defect|congenital disorder]] in which various structures of the caudal region, [[Cloaca (embryology)|embryonic cloaca]], and [[neural tube]] exhibit a spectrum of abnormalities such as duplication and malformations.<ref name=":3">{{cite journal| vauthors = Dominguez R, Rott J, Castillo M, Pittaluga RR, Corriere JN |date= October 1993 |title=Caudal Duplication Syndrome|journal=Archives of Pediatrics & Adolescent Medicine|volume=147|issue=10|pages=1048–52|doi=10.1001/archpedi.1993.02160340034009 |pmid= 8213674 }}</ref> The exact causes of the condition is unknown,<ref name=":3" /> though there are several theories implicating abnormal embryological development as a cause for the condition.<ref name=":4" /> Diagnosis is often made during prenatal development of the second trimester through anomaly scans or immediately after birth.<ref name=":6" /> However, rare cases of adulthood diagnosis has also been observed. Treatment is often required to correct such abnormalities according to the range of symptoms present,<ref name=":6" /> whilst treatment options vary from conservative expectant management to resection of caudal tissue to restore normal function or appearance.<ref name=":7">{{cite journal | vauthors = Molinaro F, Cerchia E, Bulotta AL, Angotti R, Di Maggio G, Bianchi A, Messina M |title=Caudal 'duplication' or 'split' syndrome: Is there a misnomer?|issue=10|pages=351–356|journal=Journal of Pediatric Surgery Case Reports|volume=1|doi=10.1016/j.epsc.2013.09.007|date=October 2013|doi-access=free}}</ref> As a rare congenital disorder, the prevalence at birth is less than 1 per 100,000 <ref name=":4">{{cite journal | vauthors = Sur A, Sardar SK, Paria A | title = Caudal duplication syndrome | journal = Journal of Clinical Neonatology | volume = 2 | issue = 2 | pages = 101–2 | date = April 2013 | pmid = 24049755 | pmc = 3775131 | doi = 10.4103/2249-4847.116412 }}</ref> with less than 100 cases reported worldwide.<ref name=":5">{{cite journal| vauthors = Goil P, Bannerjee A |date=2015-04-10|title=A Rare Presentation of Caudal Duplication Syndrome in an Adult with No Functional Impairment|journal=Journal of Case Reports|volume=4|issue=2|pages=324–327|doi=10.17659/01.2014.0081|doi-access=free}}</ref> | | '''Caudal duplication''' is a rare congenital disorder characterized by the duplication of structures in the caudal region of the body. This condition is also known as caudal duplication syndrome or dipygus. It involves the duplication of various anatomical structures, which may include the [[spinal cord]], [[gastrointestinal tract]], [[urogenital system]], and [[lower limbs]]. |
| | | ==Presentation== |
| The term "caudal duplication syndrome" has been coined since 1993<ref name=":3" /> to describe caudal abnormalities and conditions. However, there has been recent debate into the appropriateness of the term being "caudal split syndrome" instead of caudal duplication due to the "splitting" nature of the abnormalities, rather than "duplication".<ref name=":7" />
| | Individuals with caudal duplication may present with a wide range of anatomical duplications. The severity and extent of duplication can vary significantly among affected individuals. Common features include: |
| | | * Duplication of the [[spinal cord]] and [[vertebrae]], leading to a condition known as [[diastematomyelia]]. |
| == Signs and symptoms ==
| | * Duplication of the [[gastrointestinal tract]], which may involve the [[colon]] and [[rectum]]. |
| The condition’s symptoms vary greatly due to the diverse spectrum of gastrointestinal (GI), urogenital (genitourinary, GU), spinal, and limb anomalies.<ref name=":0">{{cite journal | vauthors = Swaika S, Basu S, Bhadra RC, Sarkar R, Maitra SK | title = Caudal duplication syndrome-report of a case and review of literature | journal = The Indian Journal of Surgery | volume = 75 | issue = Suppl 1 | pages = 484–7 | date = June 2013 | pmid = 24426655 | pmc = 3693374 | doi = 10.1007/s12262-013-0838-z }}</ref> Common examples include anorectal malformation, and duplication of the external genitalia,<ref name=":0" /> while other symptoms include incomplete duplication of the lower spine, spinal cord, ([[diastematomyelia]]) <ref name=":1">{{cite journal | vauthors = Taneja AK, Zaffani G, Amato-Filho AC, Queiroz L, Zanardi V, Menezes-Netto JR | title = Caudal duplication syndrome | journal = Arquivos de Neuro-Psiquiatria | volume = 67 | issue = 3A | pages = 695–6 | date = September 2009 | pmid = 19722052 | doi = 10.1590/S0004-282X2009000400023 | doi-access = free }}</ref> partial fusion of complete duplication of the uterus, vagina, colon, and bladder.<ref>{{OMIM|607864|Caudal Duplication Anomaly}}</ref>
| | * Duplication of the [[urogenital system]], including the presence of two [[bladders]] or [[urethras]]. |
| | | * Duplication of the [[lower limbs]], which can result in the presence of extra [[legs]] or [[feet]]. |
| Malformations of the spine have indicated to cause varying levels of neurological impairment.<ref name=":1" /> Although the level of neurological impairment is dependent upon the severity and type of spinal abnormality, most reported cases of spinal cord duplication exhibit severe neurological impairment,<ref name=":1" /> though cases of mild or absent neurological impairment has also been observed.<ref name=":1" />
| | ==Etiology== |
| | | The exact cause of caudal duplication is not well understood. It is believed to result from an abnormality during the early stages of [[embryonic development]]. One hypothesis suggests that the condition arises from an incomplete splitting of the [[caudal eminence]], a structure involved in the formation of the lower body during embryogenesis. |
| Though patients often present a diverse variety of symptoms, a case was observed in which a female adult with duplication of the colon, rectum, anus, urinary bladder, urethra, uterus, cervix, vagina, and external genitalia exhibited no detrimental effects.<ref name=":6">{{cite journal | vauthors = Samuk I, Levitt M, Dlugy E, Kravarusic D, Ben-Meir D, Rajz G, Konen O, Freud E | display-authors = 6 | title = Caudal Duplication Syndrome: the Vital Role of a Multidisciplinary Approach and Staged Correction | journal = European Journal of Pediatric Surgery Reports | volume = 4 | issue = 1 | pages = 001–005 | date = December 2016 | pmid = 28018799 | pmc = 5177553 | doi = 10.1055/s-0035-1570370 }}</ref> This suggests that rarely seen cases of complete duplication of the urogenital and gastrointestinal tract are often [[asymptomatic]].<ref name=":2">{{cite journal | vauthors = Shah KR, Joshi A | title = Complete genitourinary and colonic duplication: a rare presentation in an adult patient | journal = Journal of Ultrasound in Medicine | volume = 25 | issue = 3 | pages = 407–11 | date = March 2006 | pmid = 16495506 | doi = 10.7863/jum.2006.25.3.407 }}</ref>
| | ==Diagnosis== |
| | | Diagnosis of caudal duplication is typically made through a combination of [[clinical examination]] and [[imaging studies]]. [[Ultrasound]], [[MRI]], and [[CT scans]] are commonly used to assess the extent of duplication and to plan for potential surgical interventions. |
| As patients often present a multitude of differing symptoms within the caudal region, each patient exhibits a unique, characterised symptom which may or may not be cosmetically or physiologically detrimental to the individual.<ref name=":3" />
| | ==Management== |
| {| class="wikitable"
| | The management of caudal duplication is complex and often requires a multidisciplinary approach. Treatment is tailored to the specific needs of the individual and may involve: |
| |+
| | * Surgical correction of anatomical duplications to improve function and appearance. |
| Summary of Caudal Duplication Anomalies
| | * Management of associated complications, such as [[urinary tract infections]] or [[bowel obstruction]]. |
| !Classification<ref name=":3" />
| | * Long-term follow-up with specialists in [[pediatrics]], [[orthopedics]], [[urology]], and [[gastroenterology]]. |
| !Anomalies and salient clinical features<ref name=":3" />
| | ==Prognosis== |
| |-
| | The prognosis for individuals with caudal duplication varies depending on the severity of the condition and the presence of associated anomalies. With appropriate medical and surgical management, many individuals can lead functional lives, although they may require ongoing medical care. |
| |Gastrointestinal tract anomalies
| | ==Related pages== |
| |
| | * [[Congenital disorder]] |
| * Duplication of [[Colon (organ)|colon]], [[cecum]], [[Human anus|ani]], [[Appendix (anatomy)|appendix]], and [[ileum]] | | * [[Diastematomyelia]] |
| *[[Agenesis]], [[atresia]], [[cyst]], [[hernia]]tion, [[omphalocele]], [[imperforate anus]], and [[Anal fistula|rectal fistula]]
| | * [[Embryonic development]] |
| *[[Meckel's diverticulum|Meckel’s diverticulum]], [[intestinal malrotation]], and [[situs inversus]] | | * [[Spinal cord]] |
| | | {{stub}} |
| |-
| |
| |Genitourinary tract anomalies
| |
| |
| |
| * Duplication of external and internal genitalia, [[vagina]], [[cervix]], [[Urinary bladder|bladder]], [[urethra]], [[uterus]], [[ureter]], [[scrotum]], and [[Testicle|testis]] | |
| * Bilateral exstrophy of [[Bladder exstrophy|bladder]], [[cryptorchidism]], malrotation, agenesis of kidney, rectourethral fistula, urinary disturbances including incontinence and retention
| |
| |-
| |
| |Spinal anomalies (includes lower limb anomalies)
| |
| |
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| * Duplication of lumbar and thoracic vertebrae, sacrum, coccyx, illium, and spinal cord | |
| * Hemivertebra, sacral agenesis, [[Spina bifida|myelomeningocele]], diplomyelia, [[spina bifida]], and [[diastematomyelia]]<ref name=":1" />
| |
| * Third leg from [[meningocele]]
| |
| |}
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| == Causes == | |
| The exact cause of the condition is unknown.<ref name=":3" /> Although various theories indicate incomplete separation of monozygotic twins as an etiological factor,<ref name=":4" /> abnormal adherence between the [[ectoderm]] and [[endoderm]] during [[gastrulation]],<ref name=":0" /> polytopic primary developmental field defects,<ref>{{cite journal | vauthors = Kroes HY, Takahashi M, Zijlstra RJ, Baert JA, Kooi KA, Hofstra RM, van Essen AJ | title = Two cases of the caudal duplication anomaly including a discordant monozygotic twin | journal = American Journal of Medical Genetics | volume = 112 | issue = 4 | pages = 390–3 | date = November 2002 | pmid = 12376942 | doi = 10.1002/ajmg.10594 }}</ref> [[Somatic mutation|somatic]] and [[Germline mutation|germ line mutations]] in [[Developmental biology|developmental genes]],<ref name=":4" /> and damage to the caudal cell mass and posterior gut have also been linked to cause structural anomalies in the caudal region.<ref name=":0" /> | |
| | |
| It is speculated that the condition is related to the [[Hox gene|HOX gene]],<ref name=":4" /> namely HOX10 and HOX11. Normally coding for the mammalian appendicular and axial skeleton, misexpression of the genetic factors could lead to abnormal proliferation of the caudal [[mesenchyme]].<ref name=":4" />
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| Embryology is suggested to have an intimate association with the development of caudal duplication syndrome.<ref name=":3" /> At day 15 after fertilisation, the [[notochord]] grows from the [[primitive knot]], in which it invaginates and forms the notochord canal within.<ref name=":3" /> Progressively, on day 20, the ventral wall of the notochord dissolves, while communications are formed between the amniotic and yolk sac.<ref name=":3" /> One such connection is the Kovalevsky’s canal. From the 23rd to 25th day of gestation, the spinal cord develops except for its distal-most aspect where the notochord and neural tube are joined to form the caudal cell mass.<ref name=":3" /> The canal of Kovalevsky crosses the caudal cell mass, while endoderm located anteriorly to the cell mass develops into the [[hindgut]], various insults towards the cell mass and hindgut during the stage of development may lead to the development of caudal anomalies, one of which is caudal duplication syndrome.<ref name=":3" />
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| The incomplete regression of Kovalevsky’s canal may also lead to formations of fibrous bands joining the hindgut to the [[spinal canal]],<ref name=":7" /> possibly leading to the onset of diastemetaomyelia.<ref name=":6" /> These bands may divide the notochord, developing into duplications of the lower spine and spinal cord, the adjacent mesoderm is also divided, resulting in duplicates of GI and GU tracts.<ref name=":6" /> Subsequently, the duplications can also lead to the presence of a range of anomalies including [[Fistula|dorsal enteric fistulas]], enteric cysts, [[spina bifida]], malformed or duplicated colon, bladder, sacrum, and lower spinal cord.<ref name=":3" /><ref name=":9" />
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| Moreover, a midline pelvic mass defect during gestation could be an obstacle to caudal migration of paramesonephric structures ([[Paramesonephric duct|Müllerian duct]]), which could lead to duplication of the genital tract.<ref name=":3" /> Whilst failures of migration or fusion of those structures is one of the more prevalent embryological theories for duplication of lower genitourinary organs such as the bladder.<ref name=":3" /> Intestinal duplications extending into the rectum or anus is often rare.<ref name=":3" /><ref name=":5" /> However, if the caudal cell mass is divided early, duplications of the distal bowel may still occur.<ref name=":3" />
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| In gastrointestinal abnormalities, a mechanism known as “caudal twinning”<ref name=":4" /> is proposed in which during the 23th to 25th day of gestation, the intestinal tract is filled by rapid proliferation of endothelial cells, as the gut increases in size, vacuoles appear within the cell masses to constitute a single lumen.<ref name=":3" /><ref name=":6" /> However, in abnormal cases where a vacuole is pinched off, a second lumen is created.<ref name=":6" /> The second lumen is then proposed to magnify in size in proportion to the growth of the colon, effectively duplicating all caudal structures distal from the point of separation.<ref name=":3" /><ref name=":2" />
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| == Diagnosis ==
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| The condition can often be seen as malformations that can be diagnosed by a prenatal anomaly scan in the second trimester, while progressively detailed examinations can be conducted after the first day of life of the baby.<ref name=":4" /> If an abnormality is detected early on, psychological and surgical preparation may be required to resort to a [[Caesarean section|cesarean section]] to prevent obstructed labour, in which medical paediatric and surgical care soon follows after delivery.<ref name=":10">{{cite journal | vauthors = Hu T, Browning T, Bishop K | title = Caudal duplication syndrome: imaging evaluation of a rare entity in an adult patient | journal = Radiology Case Reports | volume = 11 | issue = 1 | pages = 11–5 | date = March 2016 | pmid = 26973727 | pmc = 4769617 | doi = 10.1016/j.radcr.2015.12.001 }}</ref><ref>{{cite journal | vauthors = Hindryckx A, De Catte L | title = Prenatal diagnosis of congenital renal and urinary tract malformations | journal = Facts, Views & Vision in ObGyn | volume = 3 | issue = 3 | pages = 165–74 | date = 2011 | pmid = 24753862 | pmc = 3991456 }}</ref><ref name=":12" />
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| Diagnosis during adulthood is extremely rare<ref name=":3" /> in cases where abnormalities are [[asymptomatic]] or are not visible upon physical inspection upon prenatal or birth inspections.<ref name=":5" /> Similarly to paediatric and prenatal diagnosis, an adulthood diagnosis can be made through various imaging modalities<ref name=":6" /> such as computed tomography (CT) scans to explicitly define the range of symptoms present in caudal duplication.<ref name=":10" />
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| == Treatment ==
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| The rare, complex syndrome includes a wide spectrum of malformations ranging from partial or isolated to complete duplication of caudal organs in GI, GU, and neural systems.<ref name=":10" /> The syndrome may cause functional impairments such as an [[imperforate anus]] and hernia which may lead to death due to shock and organ failure and require prompt surgical intervention,<ref>{{cite journal | vauthors = Acer T, Ötgün İ, Sağnak Akıllı M, Gürbüz EE, Güney LH, Hiçsönmez A | title = A newborn with caudal duplication and duplex imperforate anus | journal = Journal of Pediatric Surgery | volume = 48 | issue = 5 | pages = E37-43 | date = May 2013 | pmid = 23701807 | doi = 10.1016/j.jpedsurg.2013.03.068 }}</ref> but most presented symptoms are not life-threatening and duplicated organs are in fact functional in many cases.<ref name=":3" /><ref name=":5" /> For instance, patients with genital duplication are mostly expected to have normal menstruation, sexual intercourse, and even pregnancy,<ref>{{cite journal | vauthors = Lin PC, Bhatnagar KP, Nettleton GS, Nakajima ST | title = Female genital anomalies affecting reproduction | language = English | journal = Fertility and Sterility | volume = 78 | issue = 5 | pages = 899–915 | date = November 2002 | pmid = 12413972 | doi = 10.1016/S0015-0282(02)03368-X }}</ref> although their self-esteem and quality of life may be influenced.<ref name=":7" /> Since the clinical presentation of each patient and its complexity vary greatly, the management which usually includes surgery are carefully planned and individualised based on the extent of duplication and functionality of the involved organs.<ref name=":6" />
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| An extensive medical work-up is required primarily before prognosis to understand the anatomy of patients and to decide appropriate treatment. Imaging modalities such as [[echocardiography]], [[X-ray|conventional X-ray]], [[magnetic resonance imaging]] (MRI), [[Medical ultrasound|ultrasonography]], [[barium enema]], [[CT scan|computed tomography]] (CT) scan, and [[voiding cystourethrography]] (VCU) can be used to examine anomalies in detail.<ref name=":4" /><ref name=":6" /><ref name=":10" /> [[Exploratory laparotomy]] can also be conducted when needed.<ref name=":0" /> In most cases, surgical approach is utilised to excise or fuse the duplicated organs;<ref name=":4" /><ref name=":0" /><ref name=":8">{{cite journal| vauthors = Molinaro F, Cerchia E, Bulotta AL, Angotti R, Di Maggio G, Bianchi A, Messina M |date=Oct 2013|title=Caudal 'duplication' or 'split' syndrome: Is there a misnomer?|journal=Journal of Pediatric Surgery Case Reports|language=en|volume=1|issue=10|pages=351–356|doi=10.1016/j.epsc.2013.09.007|doi-access=free}}</ref> however, surgical intervention is not a compulsory procedure for patients that do not exhibit functional deterioration and symptoms.<ref name=":6" /> Based on the work-up results, a multidisciplinary team consisting of a (paediatric) surgeon, a urologist, and a neurosurgeon plans individualised, staged correction.<ref name=":11">{{cite journal | vauthors = Ramzan M, Ahmed S, Ali S | title = Caudal duplication syndrome | journal = Journal of the College of Physicians and Surgeons--Pakistan | volume = 24 | issue = 1 | pages = 64–6 | date = January 2014 | pmid = 24411548 | url = https://jcpsp.pk/archive/2014/Jan2014/18.pdf }}</ref>
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| If a prenatal or after birth diagnosis is made, medical paediatric and surgery care are organised soon after delivery.<ref name=":12">{{cite journal| vauthors = Bandré E, Wandaogo A, Kabre SM, Ouedraogo I, Napon M |date=Nov 2015|title=Challenges in the management of a rare case of caudal duplication syndrome in a poor resource setting|journal=Journal of Pediatric Surgery Case Reports|language=en|volume=3|issue=11|pages=508–511|doi=10.1016/j.epsc.2015.10.003|doi-access=free}}</ref> Adults with the syndrome, however, usually do not require surgical treatment unless accompanied by symptoms or psychological issues.<ref name=":6" /><ref name=":5" /> Reconstructive surgeries are performed to resolve the issue of functional impairments such as obstruction of colon, anatomic anomalies that hinder movement or cause infertility, and to improve cosmetic appearances in the case of genital duplication.<ref name=":6" />
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| === Gastrointestinal tract === | |
| Treatments for colonic duplication varies from conservative management for asymptomatic cases to excision of duplicated colon to avoid potential issues such as colon structure and obstruction.<ref name=":6" /><ref name=":0" /> Resection is possible when each duplicated colon has a complete blood supply. If the duplicated colons share a wall, a septotomy can be performed to create a small hole to connect two colons.<ref name=":6" /><ref name=":0" /> In cases where rectum is also duplicated, either the rectums should be converted into one reservoir through septotomy followed by anorectoplasty or the duplicated colon and rectum should be removed and colostomies should be constructed.<ref name=":6" /> For patients with poor prognosis for bowel control, a [[Malone procedure]] can be utilised during the colostomy.<ref name=":6" /> Alternatively, in cases of renal duplication, the [[Mitrofanoff procedure]] is performed instead.<ref name=":6" />
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| === Genitourinary tract ===
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| Duplication of genital tract that does not involve functional impairment does not require surgical intervention; however, plastic surgery can be carried out to improve patients’ self-esteem and social status.<ref name=":8" /> For duplication of female genital tract, the septum between duplicated organs such as vagina, cervix, and vulva are resected to combine two duplicated organs into one or one duplicated organ could be detached and excised.<ref name=":8" /> For male patients, one duplicated genitalia can be removed, and duplicated scrotum and testis can be either combined or excised.<ref name=":12" /> The external genitalia of both male and female can be reconstructed by midline apposition of tissues.<ref name=":8" /> For duplication of urinary tract, bladders can be combined to form a single larger bladder without altering the duplicated ureters.<ref name=":8" />
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| === Spinal and lower limb tract ===
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| In cases of spine duplication, [[Preventive healthcare|prophylactic]] surgery can be done to remove one duplicated spine, although it depends on the severity of duplication.<ref>{{cite journal | vauthors = Cebesoy O, Mete A, Karsli B | title = Complete lumbar spine duplication in a neurologically intact man | journal = The Journal of Spinal Cord Medicine | volume = 32 | issue = 1 | pages = 99–102 | date = Feb 2009 | pmid = 19264056 | pmc = 2647508 | doi = 10.1080/10790268.2009.11760759 }}</ref> In cases of malformation of the neural cord such as [[myelomeningocele]] and tethered cord which accompanies severe symptoms, preserving neurological function is the utmost importance by closing myelomeningocele and detethering the neural cord.<ref name=":6" /> This is a highly complex procedure that requires extreme caution not to injure the rectum in which case can cause a cerebrospinal fluid infection.<ref name=":6" /> For duplication of lower limb which not only hinders patients’ gait but also is highly visible and may affect patients’ self-esteem, excision of the supernumerary, non-functional pair of limb is often carried out.<ref name=":12" />
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| Due to the spectrum of symptoms present within caudal duplication, there is no uniform surgical treatment in relieving symptoms.<ref name=":11" /> The type and severity of surgical intervention is often dependent upon the type and complexity of symptoms presented.<ref name=":11" /> Thus, the primary goal of surgical treatment for the syndrome is to relieve symptoms, not to restore normal anatomy, and hence, potentially life-threatening malformations are addressed first and often followed by other anatomic or aesthetic reconstructions in later stages.<ref name=":6" /><ref name=":5" /> After the medical treatment, the patients are periodically monitored.<ref name=":8" /><ref name=":12" />
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| == Epidemiology ==
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| Caudal duplication syndrome is a rare condition with only less than 100 patients in literature worldwide as of 2014 with only 2 patients diagnosed in adulthood.<ref name=":5" /> The prevalence of the syndrome is less than one per 100,000 births.<ref name=":7" /> The sex ratio of male to female patients is about 1:2, with no familial or racial predisposition has been found.<ref name=":11" />
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| == History ==
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| The first systematic review for caudal duplication symptoms was done and the term "caudal duplication syndrome" was first proposed in 1993.<ref name=":3" /> The term was coined to describe rare anomalies associated with complete or partial duplication of caudal structures resulted from insults during embryogenesis to distinguish symptoms of spinal duplication syndrome which only involves spinal duplicity, only when there is associated complete or partial duplicity of vascular structures and/or organs such as bladder and distal gastrointestinal tract the term caudal duplication syndrome can be used.<ref name=":9">{{cite journal | vauthors = Pang D, Dias MS, Ahab-Barmada M | title = Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations | journal = Neurosurgery | volume = 31 | issue = 3 | pages = 451–80 | date = September 1992 | pmid = 1407428 | doi = 10.1227/00006123-199209000-00010 }}</ref>
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| However, recently in 2013, it was suggested that “duplication” is a misnomer based on an analysis of two cases and literature review in which researchers found “hemi” organs was “split” not duplicated, proposing caudal “split” syndrome may be a more appropriate title.<ref name=":7" />
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| == References ==
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| {{reflist|30em}} | |
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| [[Category:Congenital disorders of digestive system]] | | [[Category:Congenital disorders of digestive system]] |
| [[Category:Congenital disorders]] | | [[Category:Congenital disorders]] |
| [[Category:Embryology of urogenital system]] | | [[Category:Embryology of urogenital system]] |
| [[Category:Embryology of digestive system]] | | [[Category:Embryology of digestive system]] |
| {{dictionary-stub1}}
| | [[Category:Rare diseases]] |
| {{No image}}
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