Project description:BackgroundTotal colonic aganglionosis (TCA) is a rare variant of Hirschsprung's disease occurring in 3-10% of the cases. Only few studies reported the long-term clinical and metabolic outcomes of patients with TCA. The aim of this study was to evaluate the functional and metabolic long-term outcomes of children undergoing surgical treatment for TCA.MethodsA 15-year retrospective study was performed. Blood chemistry tests and stool analysis performed at the last follow-up visit were recorded. Height and weight development were assessed using the corresponding percentiles for age. Faecal continence and quality of life were evaluated using a detailed questionnaire.ResultsEleven patients were included in the study. The median age at surgery was 6 months (range: 3-72 months). After histological confirmation, all patients underwent a total colectomy. Ileoanal anastomosis (n = 6), ileorectal anastomosis (n = 1), J-pouch (n = 1) and Duhamel procedure (n = 3) were performed. Temporary ileostomy was closed after a median of 8 weeks in 10/11 patients. After a median follow-up of 78 months (range: 27-199 months), all evaluated patients were continent. Height and weight were appropriate for age in only 5 patients. Vitamin B12 and folic acid serum levels were normal in all examined patients. Ten patients had normal hemoglobin serum levels. Seven patients had low transferrin saturation in serum. Hemoccult tests were negative in all examined patients. Despite complex postoperative courses in some cases, patients and parents showed good overall satisfaction in terms of quality of life.ConclusionThe majority of patients reported a good quality of life. This can result from the adaptation of the patients to certain disease states. The failure to thrive seems to be related with the extent of aganglionosis. The inclusion of these patients in interdisciplinary long-term follow-up care, in which pediatric surgeons, gastroenterologists, and dieticians are involved, is essential.
Project description:BackgroundThe functional outcome after the treatment of laryngeal cancer is tightly related to the quality of life of affected patients. The aim of this study is to describe the long-term morbidity and functional outcomes associated with the different treatment modalities for laryngeal cancer.MethodsRetrospective chart review of 477 patients undergoing curatively intended treatment for laryngeal cancer at our tertiary referral center from 2001 to 2014: Details on patient and disease characteristics, diagnostics and treatment related functional outcomes were analyzed.ResultsWith a median follow-up of 51 months, the crude rate of functional larynx preservation was 74.6%. Radiotherapy +/- chemotherapy was the dominant treatment modality (n = 359-75.3%), whereas 24.7% (n = 118) underwent primary surgery, with 58.5% (69) receiving adjuvant treatment. The 5-year laryngectomy-free survival was 57% (95% CI, 48-66%) after surgery vs. 69% (95% CI, 64-75%) after chemoradiotherapy (p < 0.01). In stage III-IVB, these rates were 26% (95% CI, 16-39%) vs. 47% (95% CI, 36-59%), respectively (p < 0.01). Aspiration occurred in 7%, tracheostomy was necessary in 19.8% and feeding tube placement in 25.4%. Feeding tube and tracheostomy necessity was higher in the initially surgically treated group. Primary surgery (HR: 1.67, 95% CI: 1.19-2.32; p < 0.01), stage III-IVB (HR: 4.07, 95% CI: 2.97-5.60; p < 0.01) and tumor recurrence (HR: 3.83, 95% CI: 2.79-5.28; p < 0.01) remained as adverse factors for laryngectomy-free survival.ConclusionsPreserving the laryngeal function after cancer treatment is challenging. Advanced tumor stages, primary surgery and recurrence are related to a poor functional outcome. Therefore, the criteria for initial decision-making needs to be further refined.
Project description:ObjectiveTo study the long-term treatment outcome of vestibular paroxysmia (VP).Study designRetrospective study.SettingTertiary referral hospital.MethodsWe analyzed records of 29 consecutive patients who were diagnosed with VP and who were treated with VP-specific anticonvulsants for at least 3 months. Patients were followed for a minimum of 6 months. We recorded and assessed starting and target dosage of medications, time to achieve adequate therapeutic response, adverse effects, and the rates of short-term and long-term remission without medication.ResultsAll 29 patients were started on oxcarbazepine as first-line treatment, and 93.1% and 100% of patients reported good-to-excellent therapeutic response within 2 and 4 weeks, respectively. Three patients switched to other anticonvulsants at 3 months. At long-term follow-up (8-56 months), most (84.6%) oxcarbazepine-treated patients maintained good therapeutic response at doses between 300 and 600 mg/day. Eleven (37.9%) patients experienced complete remission without medication for more than 1 month, of which six (20.7%) had long-term remission off medication for more than 12 months. Nineteen (65.5%) patients had neurovascular compression (NVC) of vestibulocochlear nerve on MRI, but its presence or absence did not predict treatment response or remission.ConclusionLow-dose oxcarbazepine monotherapy for VP is effective over the long term and is generally well-tolerated. About 20% of patients with VP in our study had long-term remission off medication.
Project description:Introduction: Fourth branchial anomalies, the rarest among anomalies of the branchial apparatus, often present diagnostic and therapeutic challenges. We evaluated the clinical presentation and radiographic features, the treatment and the long-term outcome of patients in this setting. Patients and Methods: Of 12 patients treated in the University Hospitals Leuven from 2004 until 2020, 12 variables were collected: date of birth, gender, age of onset of the symptoms, age at final diagnosis, presentation, laterality, previous procedures, diagnostic tools, treatment (open neck surgery, endoscopic laser excision, or combination), complications, recurrence, and period of follow-up. Descriptive statistics were calculated and results were compared to the existing literature. Results: The most common clinical manifestations were recurrent neck infections with and without abcedation. Definitive diagnosis using direct laryngoscopy, visualizing the internal sinus opening, was possible in all patients. A CT study revealed the typical features of fourth branchial anomalies in seven patients out of nine, an ultrasound study in five out of nine patients. All patients underwent open neck surgery. If this was insufficient, secondary endoscopic laser resection of the ostium at the apex of the piriform sinus was performed (n = 4). In eight patients a thyroid lobectomy was needed for safe complete resection. Postoperative complications were minimal and at long-term, none of the patients showed further recurrence. Average time of follow-up was 8.6 years. Conclusions: Direct laryngoscopy and CT are the most accurate diagnostic tools. Our recommended treatment schedule consists of complete excision of the sinus tract by open neck surgery as the primary treatment because this ensures the best results. In case of recurrence afterwards, endoscopic laser resection of the pharyngeal ostium solved the problem.
Project description:Primary congenital hypothyroidism is a disease associated with low serum thyroxine and elevated thyroid-stimulating hormone (TSH) levels. The processes of screening and treating congenital hypothyroidism, in order to prevent neurodevelopmental impairment (NDI) in newborns, have been well investigated. Unlike term infants, very preterm infants (VPIs) may experience low thyroxine with normal TSH levels (<10.0 μIU/mL) during long-stay hospitalization. In the current literature, thyroxine treatment has been evaluated only for TSH-elevated VPIs. However, the long-term impact of low thyroxine levels in certain VPIs with normal TSH levels deserves more research. Since July 2007, VPIs of this study unit received screenings at 1 month postnatal age (PNA) for serum TSH levels and total thyroxine (TT4), in addition to two national TSH screenings scheduled at 3-5 days PNA and at term equivalent age. This study aimed to establish the correlation between postnatal 1-month-old TT4 concentration and long-term NDI at 24 months corrected age among VPIs with serial normal TSH levels. VPIs born in August 2007-July 2016 were enrolled. Perinatal demography, hospitalization morbidities, and thyroid function profiles were analyzed, and we excluded those with congenital anomalies, brain injuries, elevated TSH levels, or a history of thyroxine treatments. In total, 334 VPIs were analyzed and 302 (90.4%) VPIs were followed-up. The postnatal TT4 concentration was not associated with NDI after multivariate adjustment (odd ratios 1.131, 95% confidence interval 0.969-1.32). To attribute the NDI of TSH-normal VPIs to a single postnatal TT4 concentration measurement may require more research.
Project description:INTRODUCTION: Unlike malignant liver tumours, the indications for hepatic resection for benign disease are not well defined. This is particularly true for focal nodular hyperplasia (FNH). Here we summarize a single-centre experience of the diagnosis and management of FNH. MATERIALS AND METHODS: Using a prospectively collected database, a retrospective analysis of consecutive patients who were managed at our centre for FNH between January 1997 and December 2006 was performed. RESULTS: The cohort was divided into two groups of patients: those who were managed surgically (n=15) and those managed conservatively (n=37). There was no correlation between tumour size and number of lesions with oral contraceptive use (p=0.07 and 0.90, respectively) and pregnancy (p=0.45 and 0.60, respectively). However, tumour size (p=0.006) and number of lesions (p=0.02) were associated with the occurrence of pain in these patients. Pain was the commonest symptom of patients (13/15) who were managed surgically. All patients underwent radiological imaging before diagnosis. The sensitivities of ultrasound, CT scanning and MRI scanning in characterizing these lesions were 30%, 70% and 87%, respectively. There were no postoperative deaths and three postoperative complications that were successfully managed non-operatively. With a median follow-up of 24 months in the surgically treated group, one patient has developed recurrent symptoms of pain. CONCLUSION. In this series, there was no mortality directly due to the surgical procedure and a modest morbidity, justifying surgical resections in selected patients.
Project description:BackgroundTo analyze the clinical characteristics and long-term treatment outcomes of patients with posterior scleritis.MethodsThis retrospective, observational case series analyzed medical records of 14 patients diagnosed with infectious or non-infectious posterior scleritis between May 2005 and March 2020 at Severance Hospital and Gangnam Severance Hospital.ResultsA total of 12 patients with non-infectious and two with infectious posterior scleritis were treated. Conjunctival injection (85.7%) was the most common symptom, followed by pain on eyeball movement (57.1%), and decreased visual acuity (42.9%). Anterior uveitis (64.3%) was the most common associated clinical finding. In five eyes (35.7%), immunosuppressive agents were administered in addition to corticosteroids to control the inflammation. Recurrence was noted in three eyes (21.4%), all of them showing non-infectious scleritis. The final visual acuity of the patients did not show significant change compared to that at the first visit (P=0.878).ConclusionsMost posterior scleritis patients were of non-infectious type and some needed additional immunosuppressive treatment. In patients with a history of ocular surgery or trauma, especially with the presence of pus-containing nodules, infectious posterior scleritis should always be considered. Since impaired vision does not improve significantly after treatment of posterior scleritis, prompt diagnosis and aggressive treatment are recommended.
Project description:Controversies remain regarding the preferred treatment strategy for central introital dyspareunia. The primary goal of this retrospective study was to evaluate the short- and long-term outcomes after operative management of central introital dyspareunia by widening hymenoplasty. In total, 513 patients were included, with a follow-up time of 10 years. We assessed the repair of sexual activities, quality of sexual life, and the prevalence of pregnancies after the procedure. In addition, general health status was assessed using the EuroQol-5D questionnaire. Of the 513 women operated on during the period of January 2009 until December 2019, 380 (74%) agreed to participate by sending a valid response. Eighty-seven percent of the respondents mention no to moderate pain for longer than one week after the procedure, while 9.5% and 4% of patients recalled the procedure as severely or extremely painful, respectively. Seventeen percent of patients recalled a complication; 13.2% reported prolonged healing or postoperative pain, 4.7% reported infection, and 2.4% reported bleeding. Twelve months after surgery, 72% experienced no or only slight pain during penetration. We can conclude that widening plasty of the posterior hymenal rim and vestibulum, performed as an ambulatory care procedure under local anesthesia, resolves more than 70% of the central introital pain problems one year after surgery.