Tethered Spinal Cord in Patients with Anorectal and Urogenital Malformations

Warf, Benjamin C.; Scott, R. Michael; Barnes, Pat; Hendren, W. Hardy

doi:10.1159/000120696

Cited by 73 publications

(38 citation statements)

References 8 publications

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“…If an abnormal sacrum is present, spinal magnetic resonance imaging (MRI) is necessary to look for a possible tethered spinal cord [Karrer et al, 1988;Warf et al, 1993].…”

Section: Discussionmentioning

confidence: 99%

Partial urorectal septum malformation sequence: A report of 25 cases

2001

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We have identi®ed 25 cases with what we are calling the partial urorectal septum malformation (URSM) sequence, which were seen in our institution over the past 27 years. The partial URSM sequence is de®ned as a single perineal/anal opening that drains a common cloaca in combination with an absent (imperforate) anus. In the 25 patients reported here, the partial URSM sequence was more common in females, with a female to male ratio of 18 to 7. Ambiguous genitalia were common in both sexes. Internal pelvic structures typically showed a cloaca with the bladder and rectum (and vagina in females) coalescing into a common canal that connected to the external surface in the perineal or anal area. Abnormalities of the internal genitalia were also common, with 12 females having a bi®d or septate vagina and 11 having a bicornuate uterus. Renal anomalies were frequent in both sexes, with 10 of 25 patients having unilateral cystic renal dysplasia and 7 of 25 patients having unilateral renal agenesis. Twenty-one of 25 patients survived long term. By de®nition, the partial URSM sequence is a milder expression of the full URSM sequence, which is de®ned as having no perineal or anal openings and is typically associated with an internal cloaca. The URSM spectrum, which encompasses the partial and full URSM sequences, is believed to be caused by abnormalities of septation of the primitive cloaca. The URSM spectrum is distinct from the VATER association and conditions caused by sex hormone abnormalities, such as congenital adrenal hyperplasia. ß

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“…If an abnormal sacrum is present, spinal magnetic resonance imaging (MRI) is necessary to look for a possible tethered spinal cord [Karrer et al, 1988;Warf et al, 1993].…”

Section: Discussionmentioning

confidence: 99%

Partial urorectal septum malformation sequence: A report of 25 cases

2001

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“…In addition to a large number of reports of tethered cords, several series of tethered cords with anorectal malformations have been reported [1, 2, 3, 4, 5, 6]. …”

Section: Introductionmentioning

confidence: 99%

Tethered Cord Associated with Anorectal Malformation

Morimoto

Takemoto

Wakayama³

2003

Pediatr Neurosurg

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Ten children with a tethered cord and also an anorectal malformation are reported in this document. The anorectal malformations comprised 5 vesicointestinal fissures, 2 cloacal exstrophies, 2 rectovesical fistulas and 1 rectobulbar fistula. All of the patients underwent colostomy in advance of surgery for untethering of the spinal cord. Although their neurologic deficits had previously been considered static, they were subjected to radiographic examination of the caudal spine and found to have a tethered cord. These 10 children were among 55 children with a tethered cord surgically treated at the Division of Neurosurgery of the Osaka Medical Center and Research Institute for Maternal and Child Health during the last 11 years. Data were obtained for these 10 children (6 boys and 4 girls, mean age 1.7 years) who underwent surgical untethering. Several hypotheses are offered to explain this association. Anorectal malformations are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. Eight of our patients had no skin stigma of the lumbosacral region, in contrast to an ordinary tethered cord. Spinal cord imaging is necessary to closely scrutinize these children.

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“…12,71 Approximately 10 to 50% of the patients with anorectal anomalies will also have an element of OSD. 12,71 OCCULT DYSRAPHIC ELEMENTS Intraspinal anomalies common to OSD include the lipomyelomeningocele, the dermal sinus, diastematomyelia, the tight terminal filum, the neurenteric cyst, the terminal myelocystocele, and meningocele manqué.…”

Section: Anorectal Anomaliesmentioning

confidence: 99%

“…12,71 Mechanical traction on the spinal cord may be a cause of progressive symptoms. 48 Yamada, et al, 73 have proposed that symptoms appear after hypoxic damage within the conus medullaris of patients with TCS.…”

mentioning

confidence: 99%