BACKGROUND: In recent decades, magnetic resonance imaging (MRI) has gained prominence as a standard diagnostic method for preoperative assessment in patients with anorectal malformations and a colostomy, with the potential to replace the classic fluoroscopic distal pressure colostogram (FDPC). Three MRI techniques are available: MRI-distal pressure colostogram with gadolinium (MRI-DPCG) or saline (MRI-DPCS) instillation into the colostomy and native MRI without colostomy instillation. OBJECTIVE: To evaluate and compare the diagnostic accuracy of MRI (native MRI, MRI-DPCG and MRI-DPCS) in the preoperative workup of boys with an anorectal malformation and a colostomy and to compare it to FDPC. MATERIALS AND METHODS: Sixty-two boys with preoperative MRI using one of the three approaches and 43 with FDPC met the inclusion criteria for this retrospective study. The presence and localization of rectal fistulas according to the Krickenbeck classification were evaluated and compared with intraoperative findings. RESULTS: The accuracy of fistula detection for MRI in general (regardless of the technique), MRI-DPCS, MRI-DPCG, native MRI and FDPC was 95% (59/62, P<0.001), 100% (12/12, P=0.03), 100% (30/30, P<0.001), 85% (17/20, P=0.41) and 72% (31/43, P=0.82), respectively. The accuracy of describing fistula type in patients with a correctly detected fistula using these methods was 96% (45/47, P<0.001), 100% (9/9, P<0.001), 100% (23/23, P<0.001), 87% (13/15, P<0.001) and 67% (13/21, P=0.002), respectively. CONCLUSION: MRI is a reliable method for detecting and classifying fistulas in boys with an anorectal malformation and a colostomy and can be considered the modality of first choice for preoperative workup.
- MeSH
- Anorectal Malformations * diagnostic imaging surgery MeSH
- Colostomy MeSH
- Humans MeSH
- Magnetic Resonance Spectroscopy MeSH
- Magnetic Resonance Imaging methods MeSH
- Rectal Fistula * surgery MeSH
- Rectum diagnostic imaging surgery abnormalities MeSH
- Retrospective Studies MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
INTRODUCTION: Postoperative constipation (PC) in patients with imperforate anus and perineal fistula (PF) has been reported in up to 60%. Histological studies of PF revealed innervation anomalies which seem to be one of the reasons for PC. Perioperative histologically controlled fistula resection (PHCFR) allows appropriate resection of PF and pull-down normoganglionic rectum at the time of posterior sagittal anorectoplasty (PSARP). MATERIALS AND METHODS: A total of 665 patients with anorectal malformations underwent surgery between 1991 and 2021. Of these, 364 presented PF; 92 out of them (41 F) were studied. Patients with sacral and spinal cord anomalies, neurological disorders, and cut-back anoplasty were excluded. PSARP was done on all patients. Hematoxylin-eosin staining and NADH Tetrazolium-reductase histochemical method were used. Four and more ganglion cells in the myenteric plexus represented a sufficient length of the resection. The continence was scored according to the modified Krickenbeck scoring system. Final scores ranged from 1 to 7 points. Values are given as median. RESULTS: A total of 65 (70.7%) patients presented an aganglionic segment in PF, and 27 patients presented hypoganglionosis. The median length of the resected fistula was 25 mm (interquartile range [IQR]: 20-30). The median total continence score was 7 (IQR: 6-7). Post-op constipation was observed in 6/92 (6.5%) patients. CONCLUSION: PHCFR diminished PC to 6.5% of patients.
- MeSH
- Anus, Imperforate * surgery MeSH
- Child MeSH
- Infant MeSH
- Humans MeSH
- Infant, Newborn MeSH
- Perineum surgery MeSH
- Postoperative Complications etiology MeSH
- Child, Preschool MeSH
- Rectal Fistula * surgery MeSH
- Rectum surgery abnormalities MeSH
- Retrospective Studies MeSH
- Treatment Outcome MeSH
- Constipation etiology surgery MeSH
- Plastic Surgery Procedures methods MeSH
- Check Tag
- Child MeSH
- Infant MeSH
- Humans MeSH
- Male MeSH
- Infant, Newborn MeSH
- Child, Preschool MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
Cíl: Popis enterální cystické duplikatury v peripartálním období. Typ studie: Kazuistika.název a sídlo pracoviště: Masarykova městská nemocnice v Jilemnici. Předmět studie: Kazuistickou formou sdělení popsaný případ enterální cystické duplikatury po porodu a relaps v průběhu následující gravidity. V obou případech histologicky totožný nález, řešený otevřenou exstirpací. Závěr: Cystická duplikatura rekta patří ke kongenitálním anomáliím vzácně se vyskytujícím i v dospělosti. V případu sehrál klíčovou roli druhý názor a kvalitní preoperační diagnostika, vedoucí v obou případech k primární kompletní exstirpaci, bez nutnosti reoperace či zakládání stomie. Operačním přístupem byl zvolen parasakrální řez, stranově rozlišený podle umístění dominantní části cysty.
Objective: Author demonstrates case of enteral cystic duplicature located between coccyx and rectum in peripartal period. Design: Case report. Setting: Masarykova městská nemocnice, Jilemnice. Subject and method: Case of enteral cystic duplicature in adult, 25-29 year female, first case after natural birth, second one in the end of second pregnancy. Open surgical solution with exstirpation. Conclusion: Cystic duplication of the rectum belongs to congenital anomalies, rarely occurring even in adulthood. The second opinion and quality preoperative diagnostics played a key role in the case, leading in both cases to primary, complete exstirpation, without any reoperation or complications. The surgical approach was chosen parasacral section, laterally differentiated according to the location of the dominant part of the cyst.
- Keywords
- parasakrální řez,
- MeSH
- Cysts * surgery diagnosis pathology MeSH
- Adult MeSH
- Obstetric Labor Complications MeSH
- Humans MeSH
- Magnetic Resonance Imaging MeSH
- Peripartum Period * MeSH
- Recurrence MeSH
- Rectum * abnormalities surgery MeSH
- Sigmoidoscopy MeSH
- Pregnancy MeSH
- Ultrasonography MeSH
- Treatment Outcome MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Pregnancy MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
- Keywords
- Currarinův syndrom,
- MeSH
- Digestive System Abnormalities * surgery diagnosis genetics MeSH
- Anal Canal abnormalities MeSH
- Child MeSH
- Genetic Counseling MeSH
- Sacrum abnormalities MeSH
- Humans MeSH
- Rectum abnormalities MeSH
- Congenital Abnormalities MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Publication type
- Review MeSH
- MeSH
- Digestive System Abnormalities * surgery diagnosis MeSH
- Anal Canal abnormalities surgery diagnostic imaging MeSH
- Anorectal Malformations surgery diagnosis MeSH
- Child MeSH
- Humans MeSH
- Rectum abnormalities surgery diagnostic imaging MeSH
- Intestine, Large abnormalities surgery MeSH
- Congenital Abnormalities MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Publication type
- Review MeSH
Polymastia je pomerne dobre opísaná a nie celkom neobvyklá vývojová anomália v embryogenéze prsníka. Vyskytuje sa v oblasti mliečnej lišty, najčastejšie v axile, ale môže sa nachádzať aj mimo túto oblasť. Ku klinickej manifestácii polymastie dochádza pôsobením pohlavných hormónov, hlavne počas puberty, gravidity a laktácie. Akcesorné (aberantné) prsníky môžu podliehať rovnakým patologickým procesom, či už benígnym alebo malígnym, ako normálne lokalizované prsia. V našej kazuistike prezentujeme prípad tehotnej pacientky s polymastiou jednak v axile bilaterálne, kde boli navyše v aberantných prníkoch popísané podľa UZ benignity, ďalej v oblasti konečníka a veľkého pyska ohanbia.
Polymastia is a fairly well-described and not entirely uncommon developmental anomaly in embryogenesis of the breast. It occurs in the milk line, most often in the axila but may also be present outside of this area. Clinical manifestation of polymastia is due to sex hormones, especially during puberty, pregnancy and lactation. Supernumerary/ aberrant breasts can undergo the same pathological changes, such as benign and malignant, as normally positioned breasts. In our case report we present a case of a pregnant patient with axillary polymasty bilaterally with benign tumors by ultrasound, further in anal and labia majora area.
- MeSH
- Adult MeSH
- Ergolines therapeutic use MeSH
- Hypothyroidism MeSH
- Incidence MeSH
- Comorbidity MeSH
- Pregnancy Complications * MeSH
- Humans MeSH
- Mammaplasty MeSH
- Mastectomy MeSH
- Mastitis * diagnosis etiology surgery MeSH
- Breast abnormalities MeSH
- Raynaud Disease MeSH
- Rectum abnormalities MeSH
- Treatment Outcome MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
Contrast fistulogram (FG) and distal pressure colostogram (DPCG) are standard diagnostic methods for the assessment of anorectal malformations. Pelvic magnetic resonance imaging (MRI) earned a place among essential diagnostic methods in preoperative investigations after the Currarino syndrome and a high incidence of associated spinal dysraphism were described. The aim of our study was to evaluate the possibility of substituting FG and DPCG by a modified pelvic MRI, e.g. MRI fistulogram (MRI-FG) and MRI colostogram (MRI-DPCG). The prospective study involved 29 patients with anorectal malformations who underwent a modified pelvic MRI. The length and course of fistulas and rectum, and the presence of sacral anomalies were studied on MRI images and compared with images obtained by radiologic examinations. Modified MRI brought identical results as contrast studies in 25 patients when related to the fistula and rectum length and course. MRI was more accurate for the detection of sacral anomalies. MRI-FG was the only imaging method used in the four most recent patients. The results support the assumption that conventional contrast examinations for the assessment of anorectal malformations can be replaced by MRI, thus reducing the radiation dose.
- MeSH
- Anal Canal abnormalities pathology MeSH
- Anesthesia, General MeSH
- Infant MeSH
- Colostomy MeSH
- Contrast Media administration & dosage MeSH
- Sacrum abnormalities MeSH
- Humans MeSH
- Magnetic Resonance Imaging methods MeSH
- Abnormalities, Multiple MeSH
- Urinary Fistula congenital diagnosis surgery MeSH
- Anus Diseases congenital diagnosis surgery MeSH
- Urethral Diseases congenital diagnosis surgery MeSH
- Urinary Bladder Diseases congenital diagnosis surgery MeSH
- Infant, Premature, Diseases diagnosis surgery MeSH
- Infant, Premature MeSH
- Infant, Low Birth Weight MeSH
- Infant, Newborn MeSH
- Perineum pathology MeSH
- Preoperative Care methods MeSH
- Prospective Studies MeSH
- Rectal Fistula congenital diagnosis surgery MeSH
- Rectovaginal Fistula congenital diagnosis surgery MeSH
- Rectum abnormalities pathology MeSH
- Spinal Dysraphism diagnosis pathology MeSH
- Intestinal Fistula congenital diagnosis surgery MeSH
- Check Tag
- Infant MeSH
- Humans MeSH
- Male MeSH
- Infant, Newborn MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Clinical Trial MeSH
- Research Support, Non-U.S. Gov't MeSH
BACKGROUND AND PURPOSE: Stimulation electromyography (sEMG) is useful in identifying the sphincter muscle (M) in patients with anorectal malformations (ARMs). This study evaluates the effect of anesthetic agents and M relaxants on sEMG findings. MATERIALS AND METHODS: Seventeen infants (10 boys and 7 girls) with a mean age of 6.3 months and mean body weight of 6.7 kg were included in a prospective randomized study. Anesthesia was induced by sevoflurane and opioids, and an epidural catheter was inserted caudally. Stimulation electromyography of levator ani M using 14 mA current was used, and latency and amplitude of the evoked compound M action potential (CMAP) were recorded. Patients were randomized into 2 groups. Group A received a local anesthetic epidurally, and sEMG was performed. Administration of the M relaxant and measurement of M response followed. In group B drug administration, sEMG and response measurement were performed after administration of M relaxant. RESULTS: Baseline CMAP was recorded in all patients. Average latency was 4.1 milliseconds, and average amplitude was 0.43 mV. In group A, the average latency was 4.0 milliseconds, and average amplitude was 0.65 mV. After administration of the M relaxant, the CMAP disappeared. In group B, no CMAP was observed immediately after administration of the M relaxant. CONCLUSION: Administration of the inhalational anesthetic, opioids, and local anesthetic did not influence the M response of M fibers in the levator ani M on sEMG and enables its localization during ARM reconstruction. Nondepolarizing M relaxation completely abolished the response. If M relaxant is necessary, cisatracurium is used. The most suitable method of anesthesia for ARM surgery appears to be inhalational anesthesia supplemented by opioids and epidural analgesia.
- MeSH
- Digestive System Abnormalities diagnosis MeSH
- Anal Canal abnormalities MeSH
- Anesthetics, Inhalation pharmacology MeSH
- Anesthetics, Intravenous pharmacology MeSH
- Anesthetics, Local pharmacology MeSH
- Atracurium analogs & derivatives pharmacology MeSH
- Bupivacaine analogs & derivatives pharmacology MeSH
- Equipment Design MeSH
- Electrodes MeSH
- Electromyography instrumentation methods MeSH
- Anesthesia, Epidural MeSH
- Anesthesia, Inhalation MeSH
- Anesthesia, Intravenous MeSH
- Infant MeSH
- Humans MeSH
- Methyl Ethers pharmacology MeSH
- Evoked Potentials, Motor drug effects MeSH
- Neuromuscular Nondepolarizing Agents pharmacology MeSH
- Infant, Newborn MeSH
- Intraoperative Care methods MeSH
- Prospective Studies MeSH
- Reaction Time drug effects MeSH
- Rectum abnormalities MeSH
- Sufentanil administration & dosage pharmacology MeSH
- Check Tag
- Infant MeSH
- Humans MeSH
- Male MeSH
- Infant, Newborn MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Randomized Controlled Trial MeSH
- Comparative Study MeSH
Caudal regression syndrome is a rare congenital condition characterized by varying degrees of developmental failure ranging from a partial sacral agenesis to the absence of lumbosarcal spine, hypoplasia, or fusion of the lower extremities and visceral anomalies. This is the third case of only one of the twins involved by this syndrome described in the literature and the second case of the selective involvement in dizygotic twins. Selective involvement of only one twin suggests that factors other than hyperglycemia and 7q deletions may be involved in the pathogenesis.
- MeSH
- Digestive System Abnormalities genetics pathology MeSH
- Anal Canal abnormalities pathology MeSH
- Twins, Dizygotic MeSH
- Sacrum abnormalities pathology MeSH
- Humans MeSH
- Infant, Newborn MeSH
- Rectum abnormalities pathology MeSH
- Syringomyelia genetics pathology MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Infant, Newborn MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
- Twin Study MeSH
OBJECTIVES: Enterolithiasis (multiple calcifications of intraluminal meconium) is a rare, prenatal ultrasonographic finding. In this study, our aim was to evaluate the prenatal diagnostic features and discuss the management of the patients. METHODS: The data of two cases of prenatally diagnosed fetal enterolithiasis were collected from ultrasound scan, magnetic resonance imaging (MRI) and neonatal or postnatal autopsy records. The findings were evaluated in both prenatal and postnatal periods. Chromosomal analysis was performed in one case. An evaluation of primary and secondary malformations was done. Coexisting anomalies were searched for via radiology, neonatal surgery and histopathology. RESULTS: Malformations in two cases (both males) with partial and complete urorectal septum malformation (URSM) sequence were described. The absence of an anal opening and presence of a fistula between the urinary and gastrointestinal tract were common findings. These features were considered as primary malformations contributing to the formation of enterolithiasis. Secondary anomalies (urinary and gastrointestinal system malformations, pulmonary hypoplasia, genital and other coexisting anomalies) were evaluated. CONCLUSIONS: The prenatal detection of enterolithiasis carries a poor prognosis. Most of the previously reported cases were invariably associated with major fetal malformations of the urinary and gastrointestinal tract. It is a warning sign for large bowel obstruction with or without enterourinary fistula. Therefore, adequate gastrointestinal and urologic studies must be undertaken after birth for the final diagnosis. There is a high mortality rate in the reported cases, mostly attributed to associated anomalies, and all survivors required neonatal surgery. It is important to differentiate the partial from the full URSM sequence because the prognosis in the partial URSM sequence is generally good, with long-term survival being common. Copyright 2006 John Wiley & Sons, Ltd.
- MeSH
- Adult MeSH
- Financing, Organized MeSH
- Calcinosis diagnosis MeSH
- Humans MeSH
- Magnetic Resonance Imaging MeSH
- Meconium chemistry ultrasonography MeSH
- Urinary Tract abnormalities MeSH
- Fetal Diseases diagnosis ultrasonography MeSH
- Intestinal Diseases diagnosis MeSH
- Diseases in Twins diagnosis ultrasonography MeSH
- Oligohydramnios ultrasonography MeSH
- Prenatal Diagnosis MeSH
- Rectum abnormalities MeSH
- Intestines abnormalities MeSH
- Pregnancy MeSH
- Ultrasonography, Prenatal MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Male MeSH
- Pregnancy MeSH
- Female MeSH
- Publication type
- Case Reports MeSH