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Surgical Neurology International 2023Sacral dural arteriovenous fistulas (AVFs) are often undiagnosed at the initial presentation due to their rarity.
BACKGROUND
Sacral dural arteriovenous fistulas (AVFs) are often undiagnosed at the initial presentation due to their rarity.
CASE DESCRIPTION
For 1 year, a 71-year-old man developed progressive motor and sensory disturbances in both legs. Magnetic resonance imaging showed spinal cord edema with mild contrast enhancement at the T9-10 and T12 levels. Although mild venous dilatation was observed only at the cauda equina level, it was not initially recognized as abnormal. Blood and cerebrospinal fluid tests and spinal angiography of the lower thoracic to upper lumbar levels were nonspecific. The patient was unsuccessfully treated with three courses of high-dose intravenous methylprednisolone. Ultimately, following repeat spinal angiography (i.e., including the bilateral internal iliac arteries) that revealed a low-flow sacral dural AVF supplied by the right lateral sacral artery, the patient underwent successful surgical venous AVF occlusion/transection.
CONCLUSION
In cases of spinal cord edema without perimedullary abnormal flow voids, careful spinal angiography including the sacral spine is necessary even if only minimal venous dilation is initially observed at the cauda equina level.
PubMed: 37680920
DOI: 10.25259/SNI_606_2023 -
World Journal of Gastroenterology Feb 2021Fistula and intraabdominal fistula are common complications of Crohn's disease (CD), but complex rectal fistula with abscess formation is rare. Tumor necrosis factor...
BACKGROUND
Fistula and intraabdominal fistula are common complications of Crohn's disease (CD), but complex rectal fistula with abscess formation is rare. Tumor necrosis factor antagonists combined with percutaneous drainage or surgical intervention is optimal treatment for fistulizing CD with intraabdominal abscess. There is no study show the efficacy of vedolizumab in such complicated condition.
CASE SUMMARY
A 47-year-old man has decompensated liver cirrhosis, child B. He suffered from abdominal pain, bloody diarrhea, fever, and body weight loss. CD with rectoprostatic fistula, rectopresacral fistula, pre-sacral abscess and cyto-megalovirus (CMV) infection were noted. He received antibiotics, anti-viral therapy, transverse colostomy and vedolizumab treatment. Six months later, he had deep remission and complete fistula tracts closure.
CONCLUSION
Early vedolizumab and stool diversion are effective and safe in treating CD with complex rectal fistula with abscess formation.
Topics: Abscess; Antibodies, Monoclonal, Humanized; Child; Crohn Disease; Humans; Male; Middle Aged; Rectal Fistula; Treatment Outcome
PubMed: 33584075
DOI: 10.3748/wjg.v27.i5.442 -
Stem Cells International 2019To report our experience in a compassionate use program for complex perianal fistula.
AIM
To report our experience in a compassionate use program for complex perianal fistula.
METHODS
Under controlled circumstances and approved by European and Spanish laws, a compassionate use program allows the use of stem cell therapy for patients with nonhealing diseases, mostly complex fistula-in-ano, who do not meet criteria to be included in a clinical trial. Candidates had previously undergone multiple surgical interventions that had failed. The intervention consisted of surgery (with closure of the internal opening or a surgical flap performance), followed by stem cell injection. Three types of cells were used for implant: stromal vascular fraction, autologous expanded adipose-derived, or allogenic adipose-derived stem cells. Healing was evaluated at 6 month follow-up. Outcome was classified as partial response or healing. Relapse was evaluated 1 year later. Maximum follow-up period was 48 months.
RESULTS
45 patients (24 male) were included; the mean age was 45 years, which ranged from 24 to 69 years. Since some of them received repeated doses, 52 cases were considered (42 fistula-in-ano, 7 rectovaginal fistulas, 1 urethrorectal fistula, 1 sacral fistula, and 1 hidradenitis suppurativa). Regarding fistula-in-ano, there were 18 Crohn's-associated and 24 cryptoglandular. 49 cases (94.2%) showed partial response starting 6.5 weeks of follow-up. 24 cases (46.2%) healed in a mean time of 5.5 months. A year later, all patients cured remained healed. No adverse effects related to stem cell therapy were reported.
CONCLUSION
Stem cells are safe and useful for treating anal fistulae. Healing can be achieved in severe cases.
PubMed: 31191678
DOI: 10.1155/2019/6132340 -
Iranian Journal of Public Health Aug 2017We aimed to explore the prognosis and risk factors influencing tumor recurrence in surgery-treated patients with primary sacral tumors.
BACKGROUND
We aimed to explore the prognosis and risk factors influencing tumor recurrence in surgery-treated patients with primary sacral tumors.
METHODS
Fifty-six patients between February 2011 and December 2016 in Yishui Central Hospital with primary sacral tumors were selected and treated with radical surgeries. The perioperative outcomes and postoperative neurological functions were observed. After postoperative follow-up, the overall survival time (OS), disease-free survival time (DFS), and recurrence were recorded to analyze the potential risk factors influencing tumor recurrence.
RESULTS
The average surgical duration and intraoperative hemorrhagic volume were 3.92 ± 1.46 h and 2, 348.21 ± 813.67 ml, respectively. The postoperative short-term complications included three patients with infection from obstructed drainage and two with skin flap necrosis-induced infection, who recovered after anti-infection therapies; nine with incision-edge necrosis; two with calf muscle venous thrombosis; and one with an endorhachis cerebrospinal fluid fistula, who recovered after conventional treatment. Among patients, the 1-, 2- and 3-year survival rates were 91.07% (51/56), 82.14% (46/56), and 75.00% (42/56) while the 1-, 2- and 3-year DFS rates were 89.29% (50/56), 78.57% (44/56) and 71.43% (40/56), respectively. Of the 56 patients, 16 had recurrence after surgery, with recurrence rate of 28.57%. It was predicated that surgical methods and local infiltration were the independent risk factors influencing tumor recurrence (<0.01).
CONCLUSION
The reservation of bilateral S or > unilateral S nerves can improve quality of life of patients. Surgical methods and local infiltration are the independent risk factors influencing tumor recurrence, and extensive resection can effectively control the recurrence rate.
PubMed: 28894709
DOI: No ID Found -
Journal of Pediatric Surgery Jun 2019The purpose of this study was to identify factors associated with attaining fecal continence in children with anorectal malformations (ARM).
PURPOSE
The purpose of this study was to identify factors associated with attaining fecal continence in children with anorectal malformations (ARM).
METHODS
We performed a multi-institutional cohort study of children born with ARM in 2007-2011 who had spinal and sacral imaging. Questions from the Baylor Social Continence Scale were used to assess fecal continence at the age of ≥4 years. Factors present at birth that predicted continence were identified using multivariable logistic regression.
RESULTS
Among 144 ARM patients with a median age of 7 years (IQR 6-8), 58 (40%) were continent. The rate of fecal continence varied by ARM subtype (p = 0.002) with the highest rate of continence in patients with perineal fistula (60%). Spinal anomalies and the lateral sacral ratio were not associated with continence. On multivariable analysis, patients with less severe ARM subtypes (perineal fistula, recto-bulbar fistula, recto-vestibular fistula, no fistula, rectal stenosis) were more likely to be continent (OR = 7.4, p = 0.001).
CONCLUSION
Type of ARM was the only factor that predicted fecal continence in children with ARM. The high degree of incontinence, even in the least severe subtypes, highlights that predicting fecal continence is difficult at birth and supports the need for long-term follow-up and bowel management programs for children with ARM.
TYPE OF STUDY
Prospective Cohort Study.
LEVEL OF EVIDENCE
II.
Topics: Anorectal Malformations; Child; Fecal Incontinence; Humans; Logistic Models; Prospective Studies
PubMed: 30898398
DOI: 10.1016/j.jpedsurg.2019.02.035 -
Journal of Neurosurgery. Case Lessons Jan 2023Both filum terminale arteriovenous fistulas (FTAVFs) and filum terminale lipomas (FTLs) are rare. Because of this rarity, there is a paucity of data regarding...
BACKGROUND
Both filum terminale arteriovenous fistulas (FTAVFs) and filum terminale lipomas (FTLs) are rare. Because of this rarity, there is a paucity of data regarding concomitant FTAVF and FTL, and the optimal treatment remains to be defined. The authors describe a patient with coexisting FTAVF and FTL treated with single-stage surgery.
OBSERVATIONS
A man in his 70s was referred to the authors' department because of a suspected spinal vascular malformation seen on magnetic resonance imaging that was performed to investigate lower limb weakness, intermittent claudication, and urinary incontinence. Previous imaging had shown a terminal lipoma with an internal flow void. Computed tomography angiography and digital subtraction angiography revealed an FTAVF, the feeder being a lateral sacral artery. The patient was treated surgically with curative intent. The FTL and tethered cord that had been identified by imaging were treated in the same procedure. Postoperative digital subtraction angiography showed absence of the abnormal vessels. The patient was discharged home on the 19th postoperative day.
LESSONS
When considering treatment, it is important to determine whether symptoms are attributable mainly to FTL, tethered cord, or FTAVF. One-stage treatment is useful because it eliminates both the FTAVF and the factors that led to its development.
PubMed: 36647255
DOI: 10.3171/CASE22474 -
BMC Developmental Biology Apr 2017Loss of proprotein convertase subtilisin/kexin type 5 (Pcsk5) results in multiple developmental anomalies including cardiac malformations, caudal regression, pre-sacral...
BACKGROUND
Loss of proprotein convertase subtilisin/kexin type 5 (Pcsk5) results in multiple developmental anomalies including cardiac malformations, caudal regression, pre-sacral mass, renal agenesis, anteroposterior patterning defects, and tracheo-oesophageal and anorectal malformations, and is a model for VACTERL/caudal regression/Currarino syndromes (VACTERL association - Vertebral anomalies, Anal atresia, Cardiac defects, Tracheoesophageal fistula and/or Esophageal atresia, Renal & Radial anomalies and Limb defects).
RESULTS
Using magnetic resonance imaging (MRI), we examined heart development in mouse embryos with zygotic and cardiac specific deletion of Pcsk5. We show that conditional deletion of Pcsk5 in all epiblastic lineages recapitulates all developmental malformations except for tracheo-esophageal malformations. Using a conditional deletion strategy, we find that there is an essential and specific requirement for Pcsk5 in the cranio-cardiac mesoderm for cardiogenesis, but not for conotruncal septation or any other aspect of embryonic development. Surprisingly, deletion of Pcsk5 in cardiogenic or pharyngeal mesodermal progenitors that form later from the cranio-cardiac mesoderm does not affect heart development. Neither is Pcsk5 essential in the neural crest, which drives conotruncal septation.
CONCLUSIONS
Our results suggest that Pcsk5 may have an essential and early role in the cranio-cardiac mesoderm for heart development. Alternatively, it is possible that Pcsk5 may still play a critical role in Nkx2.5-expressing cardiac progenitors, with persistence of mRNA or protein accounting for the lack of effect of deletion on heart development.
Topics: Animals; Gene Expression Regulation, Developmental; Gene Knockout Techniques; Heart; Mesoderm; Mice; Mice, Knockout; Mutation; Organogenesis; Proprotein Convertase 5
PubMed: 28446132
DOI: 10.1186/s12861-017-0148-y -
Surgical Case Reports May 2019Inferior mesenteric arterioportal fistula (APF) is rare as only 35 case reports in the literature. We herein presented a case of simultaneously double cancer in the...
BACKGROUND
Inferior mesenteric arterioportal fistula (APF) is rare as only 35 case reports in the literature. We herein presented a case of simultaneously double cancer in the rectum and stomach with inferior mesenteric APF, which is the first case report by searching using PubMed. Combination of interventional embolization and surgical operation seemed to be optimal treatment for avoiding postoperative complications and the curability.
CASE PRESENTATION
A 66-year-old male with epigastric pain was admitted to a practitioner. He underwent a gastroscopy with biopsy, and cancer located in the lesser curvature of the gastric cardia was found. Enhanced CT did not reveal wall thickening of the stomach and distant metastases, but several swollen lymph nodes were observed in the right cardia. In the arterial phase, dilation of inferior mesenteric vein (IMV) and superior rectal artery (SRA) were noted, which raised suspicions of an arterioportal communication. Colonoscopy revealed a type 2 rectal tumor located 12 cm from the anal verge. The histological diagnosis of well-differentiated tubular adenocarcinoma was confirmed by biopsy. At a first step, we planned to perform a radiological embolization of inflow vessels to APFs except for SRA. Additionally, we determined the interval time of 1 month between the first low anterior rectal resection and the sequential gastrectomy for the purpose of decreasing portal pressure. The postoperative course was uneventful without hemorrhagic complications, and S-1 was taken internally 1 year as adjuvant chemotherapy for gastric cancer. The patient still lives without recurrence of this cancer with APF and portal vein thrombosis 2.5 years after the aforementioned surgeries.
CONCLUSION
Inferior mesenteric APF and/or arteriovenous fistula (AVF) would be consisted of the several inflow arteries as superior rectal, internal iliac, and median sacral arteries, and outflow veins as inferior mesenteric, internal iliac, and median sacral veins. To determine the therapeutic strategy for left-sided colorectal cancers with abnormal vessel communications of the pelvis, it is significant to comprehend distribution and component vessels of APF and/or AVF.
PubMed: 31102060
DOI: 10.1186/s40792-019-0630-9 -
Pediatrics and Neonatology Feb 2020To evaluate the occurrence of constipation after anorectal malformations (ARM) repair and the results of laxative treatment.
BACKGROUND
To evaluate the occurrence of constipation after anorectal malformations (ARM) repair and the results of laxative treatment.
METHODS
Between August 2012 and July 2017, the clinical data of patients with ARMs was prospectively collected. The patients were divided into two groups, good types and poor types. Good types included rectoperineal, rectovestibular, rectourethral bulbar, and no fistula. Risk factors were defined as spinal cord anomalies, sacral ratio <0.4, or cognitive impairment. Success was defined as that laxative could be tapered.
RESULTS
Eighty-four patients were enrolled with mean age of 6.3 ± 7.8 (0.6-59.9) years. The mean age of onset of constipation was 12.8 ± 8.3 months and the mean interval was 5.9 ± 5.1 months after reconstructions. The interval was not significantly different between patients with good types and poor types. In 23 patients with severe constipation being treated for >6 months, 14 of 18 (77.8%) patients with good types were classified as success, whereas only 1 of 5 (20%) patients with poor types was (p = 0.02). In patients with good types, 9 of 9 (100%) patients with no risk factors were successful; however, only 5 out of 9 (55.6%) patients with risk factors were successful (p = 0.02).
CONCLUSION
Constipation occurs shortly after operations. Patients with good types and no risk factors are susceptible to weaning laxatives.
Topics: Adolescent; Adult; Anorectal Malformations; Child; Child, Preschool; Constipation; Female; Humans; Infant; Male; Middle Aged; Postoperative Complications; Risk Factors; Young Adult
PubMed: 31296400
DOI: 10.1016/j.pedneo.2019.05.010 -
AJNR. American Journal of Neuroradiology 1995To examine the clinical and radiographic findings in a large group of patients having or suspected of having a spinal dural arteriovenous fistula.
PURPOSE
To examine the clinical and radiographic findings in a large group of patients having or suspected of having a spinal dural arteriovenous fistula.
METHODS
An analysis of 240 spinal angiograms in 132 patients revealed 97 vascular malformations that included 66 spinal dural arteriovenous fistulas. Sixteen patients had 1 or more normal spinal angiograms that were performed for suspected spinal dural arteriovenous fistulas on other imaging studies. The imaging and clinical data were reviewed in all patients who had or were suspected of having a spinal dural arteriovenous fistula and who had a spinal MR (n = 44) and a myelogram (n = 37).
RESULTS
Spinal dural arteriovenous fistulas were more common in males (3.4:1) with an average age of 62 years (range, 37 to 81 years). The average time from onset of symptoms to diagnosis was 27 months. Clinical findings included weakness (55%), a progressive clinical course (100%), and a myelopathy on exam (84%). The nidus of the fistula was located between T-6 and T-12 in 61%, in the sacrum in 9%, and intracranially in 8%. In the spinal dural arteriovenous fistula group, vessels were seen on supine myelography in all patients. MR findings in this group included increased T2 signal in the cord (100%), gadolinium enhancement (88%), mass effect (45%), and flow voids (T1, 35%; T2, 45%). The patients in the negative spinal angiogram group were younger (average age, 51 years), had symptoms longer (average time from symptom onset to spinal angiogram, 59 months), and presented with numbness or pain (76%). When compared with the patients with spinal dural arteriovenous fistula, acute or stable deficits were more common (31%), and myelopathy on exam was less common (56%). Although the angiogram-negative patients commonly had vessels on the myelogram (92%), abnormal T2 signal in the cord was unusual (17%).
CONCLUSIONS
In the appropriate clinical setting, high T2 signal of the spinal cord is the most sensitive imaging finding in spinal dural arteriovenous fistula. The presence of mass effect and enhancement should not discourage this diagnosis. The likelihood of finding a spinal dural arteriovenous fistula in a patient without T2 signal on MR is low.
Topics: Adult; Aged; Aged, 80 and over; Arteries; Arteriovenous Fistula; Arteriovenous Malformations; Dura Mater; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Myelography; Neurologic Examination; Spinal Cord; Veins
PubMed: 8585493
DOI: No ID Found