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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 -
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 -
European Journal of Pediatric Surgery... Jan 2019Complete colonic duplication is rare, and usually occurs as a part of the caudal duplication syndrome. In such cases, the diagnosis is clinically evident by the presence...
Complete colonic duplication is rare, and usually occurs as a part of the caudal duplication syndrome. In such cases, the diagnosis is clinically evident by the presence of two ani arranged side by side in the perineum, which is commonly associated with duplication of the external genitalia as well (double phallus or double vestibule). In this report, we present a special case of anorectal anomaly that was associated with complete tubular colonic duplication. The diagnosis was initially missed due to the uncommon sagittal arrangement of duplicated rectum: one rectum was ending externally into the perineum by rectoperineal fistula, while the other was hidden by its internal termination into the vagina. Our final diagnosis for this case was a variant of anorectal anomaly in the female, which was associated with complete colonic duplication. One colon (which was in the free mesenteric border) terminated anteriorly into the vagina as a part of a "short common channel" cloaca, while the other colon terminated by rectoperineal fistula. Although the anomaly seems to be rather complex and confusing, yet our case was associated with an excellent outcome due to the benign type of anorectal anomalies (rectoperineal fistula and "short common channel" cloaca) and absence of significant sacral dysplasia; in addition to adequate identification of the abnormal anatomy by appropriate investigations and the staged approach for surgical reconstruction.
PubMed: 31285983
DOI: 10.1055/s-0039-1692193 -
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 -
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 -
Molecular and Clinical Oncology May 2019Chordoma is a rare tumor that originates from the notochord. Half of chordomas involve the sacral region. Surgery is considered to be the standard treatment for sacral...
Chordoma is a rare tumor that originates from the notochord. Half of chordomas involve the sacral region. Surgery is considered to be the standard treatment for sacral chordoma. However, carbon ion radiotherapy (CIRT) has recently emerged as a promising treatment for unresectable sacral chordoma. Little is known about the long-term complications of CIRT. We present two cases of rectotumoral fistula formation that occurred >5 years after CIRT for sacral chordoma. We considered two possible explanations for fistula formation: radiation enterocolitis after CIRT might cause formation of the fistula long-term, and tumor regrowth might compress the rectum and cause fistula formation. A biopsy in Case 1 showed that regrowth tumor was post-CIRT. It is important to be aware of the possibility of rectal complications after CIRT, and if found, resection of the rectum should be considered. This is a first report of rectotumoral fistula formation that occurred >5 years after CIRT for sacral chordoma.
PubMed: 30967943
DOI: 10.3892/mco.2019.1821 -
BMJ Case Reports Mar 2019Sacral dural arteriovenous fistulas (SDAVFs) are rare, constituting no more than 10% of all spinal dural fistulas. They are most commonly fed by the lateral sacral...
Sacral dural arteriovenous fistulas (SDAVFs) are rare, constituting no more than 10% of all spinal dural fistulas. They are most commonly fed by the lateral sacral artery (LSA), a branch of the internal iliac artery (IIA). Catheterization of this vessel requires either a crossover at the aortic bifurcation in cases of right femoral access or retrograde catheterization from the ipsilateral common femoral artery. We present the case of a 79-year-old man with tethered cord syndrome and a symptomatic SDAVF fed by two feeders from the left LSA. Spinal diagnostic angiography was made exceptionally challenging by an aorto-bi-iliac endograft, and selective catheterization of the left IIA was not possible. The patient could not undergo surgery due to multiple comorbidities, therefore embolization was considered the best approach. The procedure was carried out through a transradial access (TRA) with Onyx and n-butyl cyanoacrylate. The SDAVF was successfully treated and the patient made a full neurological recovery.
Topics: Aged; Angiography; Central Nervous System Vascular Malformations; Embolization, Therapeutic; Humans; Male; Neural Tube Defects; Paraparesis; Practice Guidelines as Topic; Recovery of Function; Sacrum; Treatment Outcome; Walkers
PubMed: 30936323
DOI: 10.1136/bcr-2019-014834 -
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 -
The Journal of Obstetrics and... Apr 2019To compare total pelvic floor reconstruction with vaginal mesh (TVM) and laparoscopic uterus/sacrocolpopexy (LSC) for the treatment of pelvic organ prolapse (POP). (Comparative Study)
Comparative Study
AIM
To compare total pelvic floor reconstruction with vaginal mesh (TVM) and laparoscopic uterus/sacrocolpopexy (LSC) for the treatment of pelvic organ prolapse (POP).
METHODS
Six hundred and seventy patients with POP stage 3 and 4 underwent LSC (n = 350) or TVM (n = 320) at the West China Second Hospital, Sichuan University between January 2011 and December 2016. Retrospective analysis was done to compare the POP-Q value before operation and 6 months, 5 years after operation, also compare the, patient global impression of change (PGI-C), pelvic floor distress inventory (PFDI-20) and pelvic floor impact questionnaire (PFIQ-7). Patients were followed for a median 36 months. Thirty-five patients in the LSC and 37 in the TVM groups were lost to follow-up.
RESULTS
Preoperative POP value and disease course were similar (P = 0.075). The LSC group was younger (52.8 ± 6.8 vs. 63.9 ± 8.7 years, P = 0.037). Intraoperative bleeding was smaller in the LSC group (74.4 ± 33.2 vs. 150.4 ± 80.3 mL, P < 0.01), with longer operation time (130.0 ± 34.1 min vs 100.4 ± 40.4 min, P < 0.035). The patients were followed for 10-60 months (median, 36 months). Postoperative PISQ-12 (P < 0.01) was better in the LSC group. PFDI-20 and PFIQ-7 were improved after operation in both groups. Objective satisfaction (94.9% vs 91.9%, P > 0.05) and recurrence rate (8.4% vs 5.1%, P = 0.064) were similar. No infection or fistula occurred after operation in both groups. The complication rate of intraoperative bladder injury and postoperative perineal pain in LSC group was lower than those in the TVM group (P < 0.05).
CONCLUSION
LSC showed no serious adverse events and led to higher postoperative satisfaction than TVM in selected patients. Nevertheless, treatment should be selected in accordance with the willingness and condition of each patient.
Topics: Adult; Aged; China; Female; Gynecologic Surgical Procedures; Humans; Laparoscopy; Middle Aged; Outcome and Process Assessment, Health Care; Pelvic Floor; Pelvic Organ Prolapse; Plastic Surgery Procedures; Retrospective Studies; Surgical Mesh; Uterus; Vagina
PubMed: 30652385
DOI: 10.1111/jog.13908 -
AJNR. American Journal of Neuroradiology Jan 2019Spinal epidural arteriovenous fistulas are rare vascular malformations. We present 13 patients with spinal epidural arteriovenous fistulas, noting the various presenting...
BACKGROUND AND PURPOSE
Spinal epidural arteriovenous fistulas are rare vascular malformations. We present 13 patients with spinal epidural arteriovenous fistulas, noting the various presenting symptom patterns, imaging findings related to bone involvement, and outcomes.
MATERIALS AND METHODS
Among 111 patients with spinal vascular malformations in the institutional data base from 1993 to 2017, thirteen patients (11.7%) had spinal epidural arteriovenous fistulas. We evaluated presenting symptoms and imaging findings, including bone involvement and mode of treatment. To assess the treatment outcome, we compared initial and follow-up clinical status using the modified Aminoff and Logue Scale of Disability and the modified Rankin Scale.
RESULTS
The presenting symptoms were lower back pain ( = 2), radiculopathy ( = 5), and myelopathy ( = 7). There is overlap of symptoms in 1 patient (No. 11). Distribution of spinal epidural arteriovenous fistulas was cervical ( = 3), thoracic ( = 2), lumbar ( = 6), and sacral ( = 2). Intradural venous reflux was identified in 7 patients with congestive venous myelopathy. The fistulas were successfully treated in all patients who underwent treatment (endovascular embolization, = 10; operation, = 1) except 2 patients who refused treatment due to tolerable symptoms. Transarterial glue ( = 7) was used in nonosseous types; and transvenous coils ( = 3), in osseous type. After 19 months of median follow-up, the patients showed symptom improvement after treatment.
CONCLUSIONS
Although presenting symptoms were diverse, myelopathy caused by intradural venous reflux was the main target of treatment. Endovascular treatment was considered via an arterial approach in nonosseous types and via a venous approach in osseous types.
Topics: Aged; Arteriovenous Fistula; Embolization, Therapeutic; Epidural Space; Female; Humans; Male; Middle Aged; Spinal Cord; Treatment Outcome
PubMed: 30523143
DOI: 10.3174/ajnr.A5904