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Frontiers in Pediatrics 2023Congenital perineal groove (CPG) of male patients has rarely been reported before. The purpose of this study was to review our cases and describe their characteristics...
BACKGROUND
Congenital perineal groove (CPG) of male patients has rarely been reported before. The purpose of this study was to review our cases and describe their characteristics and treatment.
METHODS
Four male patients diagnosed with CPG were included in this study. Medical records were retrospectively reviewed. Type of CPG and anal position index (API) of the patients were recorded. Follow-up was through outpatient visits.
RESULTS
Their age ranged from 4 years and 2 months to 10 years and 9 months. Among the four patients, two complained of intermittent CPG mucosal hemorrhage and the other two had mucous secreting and soiling. The API was 0.24, 0.35, 0.36, and 0.40 for each patient, respectively, all represented anterior displacement. Type of CPG for the four patients were all partial, and the sulcus was from the posterior perineum to the edge of anus. Two patients were associated with hydrocele, imperforated anus, and rectoperineal fistula; one patient had left varicocele; the remaining patient had sacrum split. All the patients had no postoperative complication, and during the follow-up period of 5-8 months, no symptoms recurred in the four patients; they all had normal defecation.
CONCLUSIONS
Both genders share the common three characteristics. In addition, shortened perineum with anterior anus, association of perineal malformations, and partial type occurrence are the extra morphological features in male patients. Furthermore, CPG in males are rarely accompanied by urinary tract infection. Favorable prognosis could be reached after operation.
PubMed: 36816386
DOI: 10.3389/fped.2023.1103867 -
Surgical Neurology International 2022Filum terminale arteriovenous fistulas (FTAVFs) are rare and usually classified as intradural ventral AVFs or Type IVa perimedullary fistulas, located on the pia surface...
BACKGROUND
Filum terminale arteriovenous fistulas (FTAVFs) are rare and usually classified as intradural ventral AVFs or Type IVa perimedullary fistulas, located on the pia surface along the course of filum terminale internum (FTI). We report an extremely rare case of sacral dural arteriovenous fistula of the FT. We also review the occurrence of FTAVFs in the sacral region.
CASE DESCRIPTION
A 64-year-old man presented with progressive weakness of the lower extremities for 3 months and bowel/bladder dysfunction following long history of back pain radiating to both legs. Magnetic resonance imaging of the lumbosacral and thoracic spine showed spinal cord congestion, extending from the conus medullaris to the level of T3, and partial thrombosis within the abnormal tortuous and dilated flow void, running from the sacral area to conus medullaris. Further findings were compression fracture of L2 vertebra, Grade I degenerative spondylolisthesis at the level of L2-3, and L3-4, and spinal stenosis at L2-3, L3-4, and L4-5. Spinal angiography, maximum intensity projection reformatted image of angiographic computerized tomography, and three-dimensional reconstructed image clearly demonstrated dural AVF of the FT at the level of S2 supplied by bilateral lateral sacral and middle sacral arteries with cranial drainage to perimedullary vein through the enlarged vein of the filum. The patient was indirectly treated by transection of the filum terminale and the draining vein at the level of L5 rostral to the fistula.
CONCLUSION
Sacral DAVFs of the FT are extremely rare. In our case, the formation of fistula may cause by venous hypertension secondary to partial thrombosis within the filum vein, probably resulting from long-standing spinal canal stenosis. Sacral FTAVFs may be found on the pia surface of the terminal FTI, dural component at the area of dural sac termination, or dural extension covering the filum terminale externum.
PubMed: 35399884
DOI: 10.25259/SNI_980_2021 -
BMJ Case Reports Feb 2021Spinal dural arteriovenous fistula (SDAVF) is a rare pathological communication between arterial and venous vessels within the spinal dural sheath. Clinical presentation...
Spinal dural arteriovenous fistula (SDAVF) is a rare pathological communication between arterial and venous vessels within the spinal dural sheath. Clinical presentation includes progressive spinal cord symptoms including gait difficulty, sensory disturbances, changes in bowel or bladder function, and sexual dysfunction. These fistulas are most often present in the thoracolumbar region. Diagnoses of SDVAFs are commonly missed, possibly due to the low index of suspicion, non-specific symptoms and challenging imaging. In this case report, we describe a rare presentation of a sacral SDAVF which was detected by collective efforts between endovascular neurosurgery and interventional radiology. We outline the diagnostic and imaging challenges we faced to discover the fistula. In particular, mechanical pump injection instead of hand injection during angiography was required to reveal the fistula. Following identification, the fistula was successfully treated endovascularly by using onyx (ethylene vinyl alcohol glue), a less invasive alternative to surgical intervention.
Topics: Angiography; Central Nervous System Vascular Malformations; Diagnosis, Differential; Embolization, Therapeutic; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Sacrum
PubMed: 33547130
DOI: 10.1136/bcr-2020-239256 -
Journal of Spine Surgery (Hong Kong) Jun 2023Enterothecal fistulas are pathological connections between the gastrointestinal system and subarachnoid space. These rare fistulas occur mostly in pediatric patients...
BACKGROUND
Enterothecal fistulas are pathological connections between the gastrointestinal system and subarachnoid space. These rare fistulas occur mostly in pediatric patients with sacral developmental anomalies. They have yet to be characterized in an adult born without congenital developmental anomaly yet must remain on the differential diagnosis when all other causes of meningitis and pneumocephalus have been ruled out. Good outcomes rely on aggressive multidisciplinary medical and surgical care, which are reviewed in this manuscript.
CASE DESCRIPTION
A 25-year-old female with history of a sacral giant cell tumor resected via anterior transperitoneal approach followed by posterior L4-pelvis fusion presented with headaches and altered mental status. Imaging revealed that a portion of small bowel had migrated into her resection cavity and created an enterothecal fistula resulting in fecalith within the subarachnoid space and florid meningitis. The patient underwent a small bowel resection for fistula obliteration, and subsequently developed hydrocephalus requiring shunt placement and two suboccipital craniectomies for foramen magnum crowding. Ultimately, her wounds became infected requiring washouts and instrumentation removal. Despite a prolonged hospital course, she made significant recovery and at 10-month following presentation, she is awake, oriented, and able to participate in activities of daily living.
CONCLUSIONS
This is the first case of meningitis secondary to enterothecal fistula in a patient without a previous congenital sacral anomaly. Operative intervention for fistula obliteration is the primary treatment and should be performed at a tertiary hospital with multidisciplinary capabilities. If recognized quickly and appropriately treated, there is a possibility of good neurological outcome.
PubMed: 37435328
DOI: 10.21037/jss-22-89 -
AJNR. American Journal of Neuroradiology Oct 2018Conventional MR imaging can provide important clues regarding the location of a spinal vascular malformation. We hypothesized that a dilated vein of the filum terminale,...
BACKGROUND AND PURPOSE
Conventional MR imaging can provide important clues regarding the location of a spinal vascular malformation. We hypothesized that a dilated vein of the filum terminale, identified as a curvilinear flow void on T2WI, could be an imaging marker for a lower lumbar (L3-L5) or sacral fistula.
MATERIALS AND METHODS
We retrospectively identified all spinal dural and spinal epidural arteriovenous fistulas from 2 large tertiary referral centers from 2005 to 2018. All patients had a lumbar spinal MR imaging and a conventional spinal angiography. Images were reviewed by 2 neuroradiologists who categorized the level of the arterial feeder to the fistula and the presence or absence of a dilated vein of the filum terminale on T2WI and T1 postcontrast images. We calculated the sensitivity, specificity, positive predictive value, and negative predictive value of the presence of a dilated filum terminale vein for a deep lumbar or sacral fistula.
RESULTS
One hundred sixty-two patients were included. An enlarged filum terminale vein was identified in 39 patients. Sensitivity, specificity, positive predictive value, and negative predictive value of the presence of a dilated filum terminale vein for a deep lumbar or sacral fistula were 86%, 98.3%, 94.9%, and 95.1%, respectively.
CONCLUSIONS
The presence of a dilated vein of the filum terminale can accurately localize a spinal dural arteriovenous fistula/spinal epidural arteriovenous fistula to the lower lumbar or sacral spine in patients being evaluated for such lesions. This finding can be used to facilitate both noninvasive and conventional spinal angiography.
Topics: Adult; Aged; Arteriovenous Fistula; Cauda Equina; Central Nervous System Vascular Malformations; Female; Humans; Lumbosacral Region; Magnetic Resonance Imaging; Male; Middle Aged; Retrospective Studies; Veins
PubMed: 30237303
DOI: 10.3174/ajnr.A5784 -
Surgical Endoscopy Jan 2016We propose a new minimally invasive technique by laparoscopic approach which minimizes parietal damage and allows precise location of the fistula, hence reduces blind...
BACKGROUND
We propose a new minimally invasive technique by laparoscopic approach which minimizes parietal damage and allows precise location of the fistula, hence reduces blind dissection.
METHODS
Ten consecutive patients suffering from a HRVF benefited from the described technique. Location and time frame were east of the Democratic Republic of Congo and September 2012 through January 2014. By laparoscopy, dissection of the mesorectum in the "holy plane" is taken posteriorly as distally on the sacrum as possible. Dissection subsequently continues laterally beyond the fistula in an effort to maximally circumvene the fistulous area where no plane of cleavage can be found. If the cleavage plane beyond the fistula addresses a healthy rectum, a suture of vaginal and rectal defect is performed. If the cleavage plane beyond the fistula involves significant laceration of the rectum, while leaving at least 2 cm of healthy rectum above the sphincter, rectal resection and colorectal anastomosis are performed. If the rectal laceration involves the distal 2 cm but halts short of the sphincter (large fistula), the pull-through technique is performed.
RESULTS
Of ten participants, four had large HRVF and two presented significant fibrosis. Three underwent simple suture of rectal and vaginal defect, one rectal resection and six a "pull-through" technique. The median procedure time was 1h50 (1h00-3h30). There was no morbidity. None of the patients required protective ileostomy or colostomy. Nine patients were declared clinically cured with a median follow-up of 14.3 months (11-36). The Cleveland Clinic Incontinence Score was 20 in all patients before the treatment and was significantly (p = 0.004) reduced to 2.6 [0-20] after the treatment.
CONCLUSIONS
This minimally invasive technique allowed us to treat HRVF, including complex ones in ten patients without significant morbidity. Clinical success with a median follow-up of 14.3 months was 90%.
Topics: Adult; Fecal Incontinence; Female; Follow-Up Studies; Humans; Laparoscopy; Middle Aged; Obstetric Labor Complications; Pregnancy; Prospective Studies; Rape; Rectovaginal Fistula; Rectum; Severity of Illness Index
PubMed: 25847136
DOI: 10.1007/s00464-015-4192-z -
Obstetrics & Gynecology Science Sep 2019Currarino syndrome is a hereditary disease characterized by the triad of sacral agenesis, anorectal malformation, and presacral mass. Most patients are diagnosed in...
Currarino syndrome is a hereditary disease characterized by the triad of sacral agenesis, anorectal malformation, and presacral mass. Most patients are diagnosed in childhood, and this condition rarely manifests in adulthood. In women, gynecological malformations associated with Currarino syndrome have been reported, such as bicornuate uterus, rectovaginal fistula, and septate uterus. We present a rare case of a 29-year-old woman with a suspected pelvic mass who was diagnosed with Currarino syndrome.
PubMed: 31538082
DOI: 10.5468/ogs.2019.62.5.367 -
Interventional Neurology Mar 2017The authors report 2 cases of sacral dural arteriovenous fistula (AVF) presenting with gradual neurologic decline characterized by progressive lower extremity weakness...
The authors report 2 cases of sacral dural arteriovenous fistula (AVF) presenting with gradual neurologic decline characterized by progressive lower extremity weakness and bowel and bladder incontinence. Spinal angiography demonstrated a sacral dural AVF with perimedullary vein engorgement and drainage extending to the level of the cervical spine in both cases. The fistulas were completely obliterated with n-BCA (-butyl cyanoacrylate) embolic agent in one case, and with ethylene vinyl alcohol (Onyx) in the second, resulting in improvement of the symptoms in both patients. The unique features of this case report include the rare location of the fistula's origin, the necessity for complete spinal angiography, and the use of intraoperative monitoring in one case to guide embolization treatment.
PubMed: 28611837
DOI: 10.1159/000454929 -
Radiology Case Reports Jan 2019We present a case of an iatrogenic rectothecal fistula in a 34-year-old man who underwent repair of a congenital anterior sacral meningocele, intraoperatively...
We present a case of an iatrogenic rectothecal fistula in a 34-year-old man who underwent repair of a congenital anterior sacral meningocele, intraoperatively complicated by rectal perforation. Postoperatively, the patient developed symptoms of meningitis prompting concern for the cerebrospinal fluid leak. Subsequent workup with computed tomography (CT) and magnetic resonance imaging demonstrated a postoperative pseudomeningocele and fistulization with an abdominal fluid collection. CT myelography confirmed the fistulous connection was between the pseudomeningocele and the rectum. Clinical suspicion of a rectothecal communication should be elevated for patients who undergo anterior sacral meningocele repair and postoperatively develop symptoms concerning for meningitis. We suggest that CT myelography be considered in the evaluation of viscero-thecal fistulas if clinical or other initial radiologic evaluation suggests the possibility of this diagnosis.
PubMed: 30386449
DOI: 10.1016/j.radcr.2018.09.011 -
Clinical Gastroenterology and... Dec 2017The purpose of this clinical practice update expert review is to describe the key principles in the use of surgical interventions and device-aided therapy for managing... (Review)
Review
The purpose of this clinical practice update expert review is to describe the key principles in the use of surgical interventions and device-aided therapy for managing fecal incontinence (FI) and defecatory disorders. The best practices outlined in this review are based on relevant publications, including systematic reviews and expert opinion (when applicable). Best Practice Advice 1: A stepwise approach should be followed for management of FI. Conservative therapies (diet, fluids, techniques to improve evacuation, a bowel training program, management of diarrhea and constipation with diet and medications if necessary) will benefit approximately 25% of patients and should be tried first. Best Practice Advice 2: Pelvic floor retraining with biofeedback therapy is recommended for patients with FI who do not respond to the conservative measures indicated above. Best Practice Advice 3: Perianal bulking agents such as intra-anal injection of dextranomer may be considered when conservative measures and biofeedback therapy fail. Best Practice Advice 4: Sacral nerve stimulation should be considered for patients with moderate or severe FI in whom symptoms have not responded after a 3-month or longer trial of conservative measures and biofeedback therapy and who do not have contraindications to these procedures. Best Practice Advice 5: Until further evidence is available, percutaneous tibial nerve stimulation should not be used for managing FI in clinical practice. Best Practice Advice 6: Barrier devices should be offered to patients who have failed conservative or surgical therapy, or in those who have failed conservative therapy who do not want or are not eligible for more invasive interventions. Best Practice Advice 7: Anal sphincter repair (sphincteroplasty) should be considered in postpartum women with FI and in patients with recent sphincter injuries. In patients who present later with symptoms of FI unresponsive to conservative and biofeedback therapy and evidence of sphincter damage, sphincteroplasty may be considered when perianal bulking injection and sacral nerve stimulation are not available or have proven unsuccessful. Best Practice Advice 8: The artificial anal sphincter, dynamic graciloplasty, may be considered for patients with medically refractory severe FI who have failed treatment or are not candidates for barrier devices, sacral nerve stimulation, perianal bulking injection, sphincteroplasty and a colostomy. Best Practice Advice 9: Major anatomic defects (eg, rectovaginal fistula, full-thickness rectal prolapse, fistula in ano, or cloaca-like deformity) should be rectified with surgery. Best Practice Advice 10: A colostomy should be considered in patients with severe FI who have failed conservative treatment and have failed or are not candidates for barrier devices, minimally invasive surgical interventions, and sphincteroplasty. Best Practice Advice 11: A magnetic anal sphincter device may be considered for patients with medically refractory severe FI who have failed or are not candidates for barrier devices, perianal bulking injection, sacral nerve stimulation, sphincteroplasty, or a colostomy. Data regarding efficacy are limited and 40% of patients had moderate or severe complications. Best Practice Advice 12: For defecatory disorders, biofeedback therapy is the treatment of choice. Best Practice Advice 13: Based on limited evidence, sacral nerve stimulation should not be used for managing defecatory disorders in clinical practice. Best Practice Advice 14: Anterograde colonic enemas are not effective in the long term for management of defecatory disorders. Best Practice Advice 15: The stapled transanal rectal resection and related procedures should not be routinely performed for correction of structural abnormalities in patients with defecatory disorders.
Topics: Fecal Incontinence; Humans; Practice Guidelines as Topic; Prostheses and Implants
PubMed: 28838787
DOI: 10.1016/j.cgh.2017.08.023