Surgical Management of an Obstructive Mullerian Anomaly in a Patient with Anorectal Malformation.
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ABSTRACT:
SUBMITTER: Theodorou CM
PROVIDER: S-EPMC8372188 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:One hundred and five patients of surgical obstructive jaundice were admitted to Army Hospital, Delhi from July 1991 to June 1994. Patients were investigated as per the diagnostic protocol. The causes of obstruction were choledocholithiasis (24 patients), periampullary carcinoma and carcinoma head of pancreas (32 patients), carcinoma gall bladder and cholangiocarcinoma (11 patients each). The procedures performed to relieve obstructive jaundice in 89 cases included choledochojejunostomy (17), pancreato-duodenectomy (15), hepaticojejunostomy (15), choledocholithotomy (12) and choledochoduodenostomy (12). Mortality was 7 per cent in pancreatoduodenectomy and 8 per cent in palliative procedures.
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Project description:BACKGROUND:Fifty to sixty percent of patients with anorectal malformation (ARM) have at least one associated anomaly (AA). We determined the incidence of AA with the subtypes of ARM classified in accordance with the Krickenbeck classification and analyzed differences in the incidence rates of major and minor AAs according to organ system. METHODS:From January 1999 to May 2017, we retrospectively analyzed congenital anomalies in patients who underwent an anoplasty for ARM at our institution. The AAs were divided into nine organ systems. To analyze the difference in the incidence of AAs, we calculated odds ratios (ORs) using cases of perineal fistula as the base group. RESULTS:Of the 460 patients, 256 (55.7%) were male, 299 (65%) had at least one anomaly, and 274 (59.6%) had major AAs. According to organ system, AAs were most common in the genitourinary (28%), cardiovascular (25%), and spinal/vertebral systems (22.6%). Major AA was most common in the cardiovascular (23%) and spinal/vertebral and genitourinary systems (19.3%). According to ARM subtype, AAs were common in the order of cloaca (93.9%), rectovaginal fistula (85.7%), and recto-bladder neck fistula (85%). For the incidence of AAs, cloaca (OR, 15.7) and recto-bladder neck fistula (OR, 5.74) showed significantly higher ORs. In the analysis of major AAs, the cloaca (OR, 19.77) showed the highest OR, followed by no fistula (OR, 4.78) and recto-bladder neck fistula (OR, 3.83). CONCLUSION:A considerable number of patients with ARM had AAs. Our data are useful for predicting AAs in patients with ARM.
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Project description:Introductionand importance: Congenital cystic adenomatoid malformation (CCAM) is a rare cystic lesion in the lungs. CCAM might present in the early neonatal period with symptoms of respiratory distress.Case presentationA 2-year-old girl was admitted to our Pediatric Intensive Care Unit with signs of severe respiratory distress. She had been diagnosed with CCAM since she was three months old. She also had undergone several procedures such as thoracotomy and decortication since then, but she still suffered some episodes of pulmonary infection. In this admission, her computerized thoracic tomography revealed a pleuropulmonary blastoma (PPB).Clinical discussionAn obstruction of blood outflow from the left ventricle could happen when an intrathoracic mass exists, leading to a decrease of cardiac output and resulting in an obstructive shock, which could be fatal. One of the malignancy types commonly occurring is pleuropulmonary blastoma (PPB), which has a poor prognosis. Early detection on CCAM can be done by prenatal ultrasound.ConclusionObstructive shock is one of complication that might occur in pleuropulmonary blastoma.
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Project description:The objective of this study was to compare the prevalence of genetic disorders in anorectal malformation (ARM) patients with upper limb anomalies to that in ARM patients with other associated anomalies. A retrospective case study was performed in two pediatric surgery centers. All patients born between 1990 and 2012 were included. VACTERL (vertebral defects (V), anal atresia (A), cardiac malformations (C), tracheoesophageal fistula with esophageal atresia (TE), renal dysplasia (R), and limb anomalies (L)) was defined as at least three components present. We included 700 ARM patients: 219 patients (31 %) had isolated ARM, 43 patients (6 %) had a major upper limb anomaly, and 438 patients (63 %) had other associated anomalies. The most prevalent upper limb anomalies were radial dysplasia (n?=?12) and hypoplastic thumb (n?=?11). Ten of the 43 patients (23 %) with an upper limb anomaly were diagnosed with a genetic disorder-nine also met the VACTERL criteria-vs. 9 % of ARM patients with other anomalies (p?=?0.004, chi-squared test).Genetic disorders are twice as frequently diagnosed in ARM patients with upper limb anomalies than in those with other anomalies. As they also frequently meet the VACTERL criteria, it is important to consider VACTERL as a diagnosis per exclusionem. Genetic counseling is certainly warranted in these patients.• Anorectal malformations (ARMs) often co-occur with other congenital anomalies, including upper limb anomalies, mainly of pre-axial origin. • Co-occurrence of ARMs and upper limb anomalies is seen in disorders such as Townes-Brocks syndrome, Fanconi anemia, and VACTERL association. What is New: • ARM patients with a major upper limb anomaly-with or without other congenital anomalies-have a twofold greater chance of a genetic disorder than have non-isolated ARM patients without upper limb anomalies. • Not all upper limb anomalies in ARM patients are part of the VACTERL association; a workup for genetic evaluation is proposed.
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Project description:Background:Anorectal malformations (ARMs) are the most common congenital malformation of the gut, and regulated by multiple signal transduction pathways. The microRNA (miRNA) expression profiles and their biologial functions in anorectal malformations (ARMs) remain unclear. The aim of our study was to evaluate miRNA and mRNA expression profiles in the ARM rats. Methods and Materials:ARM was induced with ethylenethiourea (ETU) on gestational day 10. Cesarean deliveries were performed to harvest the embryos on gestional day 20. For the extraction of total RNA, 1 cm terminal hindgut samples were obtained from three fetal rats that had similar weights. The microarrays and quantitative RT-PCR analysis were conducted to evaluate the miRNA and mRNA expression profiles in normal fetal rats (n = 3) and ARM fetal rats (n = 3). Results:In total, 33 miRNAs and 772 mRNAs were significantly and differentially expressed in terminal hindgut tissues of ARM group versus control group. Functional annotation was performed to understand the functions and pathways of differentially expressed mRNAs. Also, we constructed a miRNA-target gene regulatory network including 25 differentially expressed miRNAs and 76 mRNAs. Furthermore, the credibility of the microarray-based results were validated by using qRT-PCR. Conclusion:The miRNA and mRNA expression in terminal hindgut tissue of ARM fetal rats might provide a basis for further research on the pathogenesis of ARMs.
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