Outcomes of one-stage feminizing genitoplasty in children with congenital adrenal hyperplasia and severe virilization.

PURPOSE: To present our surgical experience and outcomes in congenital adrenal hyperplasia (CAH) patients with severe virilization using a combined technique of total urogenital mobilization (TUM) and a modified pull-through vaginoplasty to perform a safe and effective one-stage feminizing genital reconstruction for these children. METHODS: Fourteen CAH patients with severe virilization, defined by a Prader IV and V rating of the external genitalia, underwent TUM followed by a limited vaginal pull-through procedure from June 2016 to December 2020. Postoperative anatomical and cosmetic outcomes, and urinary continence, were evaluated. RESULTS: Out of the 14 cases in this study, 8 were classified as prader IV and 6 as Prader V. The median age at surgery was 11 months (range 6-36 months), and the mean urethral length was 1.4 cm (range 1.2-1.8 cm). The median follow-up period was 4 years. Our cosmetic outcomes were good in 11 (78.5%), satisfactory in 2, and poor in one case. All patients achieved age-appropriate toilet training without urinary incontinence. CONCLUSION: Adopting our surgical approach of TUM with modified pull-through vaginoplasty has simplified feminizing surgical reconstruction in CAH cases with severe genital atypia and a very high vaginal confluence with short urethral length, yielding adequate introitus with good anatomical and cosmetic appearance and adequate urinary continence outcomes.

Wake up to gastro-oesophageal reflux disease: The interplay between arousal and night-time reflux.

This review examines the temporal association between nocturnal gastro-oesophageal reflux and sleep-arousal cycles. Most nocturnal gastro-oesophageal reflux events occur during the awake cycle, and arousals precede most nocturnal gastro-oesophageal reflux events, indicating that arousal from sleep predisposes to nocturnal gastro-oesophageal reflux. This sheds light on the complex relationship between nocturnal gastro-oesophageal reflux and sleep, and has implications for managing nocturnal gastro-oesophageal reflux symptoms. The appearance of symptoms and the pathophysiology of nocturnal gastro-oesophageal reflux are influenced by sleep hygiene, sleep disturbances and the misalignment of circadian rhythms. Nocturnal gastro-oesophageal reflux and its related sleep disorders are prevalent and negatively impact the quality of life. There is conflicting evidence on whether nocturnal gastro-oesophageal reflux and sleep disturbances are causally linked, and whether sleep disturbances drive nocturnal gastro-oesophageal reflux. Poor sleep quality increases oesophageal hypersensitivity and overall acid exposure. The nocturnal gastro-oesophageal reflux is linked to the more severe forms of gastro-oesophageal reflux disease, especially with atypical/extra-oesophageal manifestations and complications of mucosal damage such as oesophagitis and stricture, Barret's oesophagus, and oesophageal adenocarcinoma. This review highlights the role of sleep problems in presenting nocturnal gastro-oesophageal reflux, and the potential benefits of treating sleep disturbances in enhancing patient care and quality of life.

A Cost-Effectiveness Analysis of Wittmann Patch-Assisted Abdominal Closure Compared to Planned Ventral Hernia in Management of the Open Abdomen.

BACKGROUND: Inability to achieve primary fascial closure after damage control laparotomy is a frequently encountered problem by acute care and trauma surgeons. This study aims to compare the cost-effectiveness of Wittmann patch-assisted closure to the planned ventral hernia closure. METHODS: A literature review was performed to determine the probabilities and outcomes for Wittmann patch-assisted primary closure and planned ventral hernia closure techniques. Average utility scores were obtained by a patient-administered survey for the following: rate of successful surgeries (uncomplicated abdominal wall closure), surgical site infection, wound dehiscence, abdominal hernia and enterocutaneous fistula. A visual analogue scale (VAS) was utilized to assess the survey responses and then converted to quality-adjusted life years (QALYs). Total cost for each strategy was calculated using Medicare billing codes. A decision tree was generated with rollback and incremental cost-utility ratio (ICUR) analyses. Sensitivity analyses were performed to account for uncertainty. RESULTS: Wittmann patch-assisted closure was associated with higher clinical effectiveness of 19.43 QALYs compared to planned ventral hernia repair (19.38), with a relative cost reduction of US$7777. Rollback analysis supported Wittmann patch-assisted closure as the more cost-effective strategy. The resulting negative ICUR of -156,679.77 favored Wittmann patch-assisted closure. Monte Carlo analysis demonstrated a confidence of 96.8% that Wittmann patch-assisted closure was cost-effective. CONCLUSIONS: This study demonstrates using the Wittmann patch-assisted closure strategy as a more cost-efficient management of the open abdomen compared to the planned ventral hernia approach.

Early detection and assessment of intensive care unit-acquired weakness: a comprehensive review.

Intensive care unit-acquired weakness (ICU-AW) is a serious complication in critically ill patients. Therefore, timely and accurate diagnosis and monitoring of ICU-AW are crucial for effectively preventing its associated morbidity and mortality. This article provides a comprehensive review of ICU-AW, focusing on the different methods used for its diagnosis and monitoring. Additionally, it highlights the role of bedside ultrasound in muscle assessment and early detection of ICU-AW. Furthermore, the article explores potential strategies for preventing ICU-AW. Healthcare providers who manage critically ill patients utilize diagnostic approaches such as physical exams, imaging, and assessment tools to identify ICU-AW. However, each method has its own limitations. The diagnosis of ICU-AW needs improvement due to the lack of a consensus on the appropriate approach for its detection. Nevertheless, bedside ultrasound has proven to be the most reliable and cost-effective tool for muscle assessment in the ICU. Combining the Sequential Organ Failure Assessment (SOFA) score, Acute Physiology and Chronic Health Evaluation (APACHE) II score assessment, and ultrasound can be a convenient approach for the early detection of ICU-AW. This approach can facilitate timely intervention and prevent catastrophic consequences. However, further studies are needed to strengthen the evidence.

Recurrent chordee in 59 adolescents and young adults following childhood hypospadias repair.

BACKGROUND: As children transition to adolescence, penile curvature may recur several years, sometimes, decades later. Herein we review our experience with a group of symptomatic patients, their surgical repairs and outcome. MATERIAL & METHODS: Reviews were done on the charts of 59 symptomatic adolescents aged 14-21 years who presented with recurrent penile curvature, causing either sexual dysfunction or significant deformity and had undergone surgical correction between 2000 and 2017. Their initial hypospadias repairs were: TIP & dorsal midline plication (28), Tiersch-Duplay (T-D) urethroplasty and Nesbit dorsal repair [9], prepuce mucosal island onlay and Nesbit repair [6], Two-stage Byar repair and ventral dermal graft (3 patients). 13/59 patients were repaired elsewhere, and their records were unavailable. Surgical correction included one stage dorsal replication and skin detethering (32patients), one-stage urethral mobilization and corporal/dermal grafts (12patients), staged corporal/dermal graft and skin coverage followed by urethroplasty (Grafted TIP) 8-12 months later (15patients), and 11/59 had urethral fistula, which were repaired concomitantly. RESULTS: 55 out of 59 patients were followed up for 6-48 months (median 30 months) by periodic office visits, and 2-4 weeks in 4 patients whose subsequent follow up was by email. Of the 55 patients, 53 healed well, and 2patients developed wound breakdown and scarring, which resulted in mild recurrent curvature but to a lesser degree than preoperatively. The other 4 patients who corresponded by email were pleased with the surgical outcome. Of the total 59 patients, 28 reported satisfactory sexual activity. None of the patients who had corporal/dermal grafts reported erectile abnormalities. CONCLUSIONS: Recurrent curvature in adolescents following hypospadias repair, may be caused by peri-urethral and skin fibrosis and/or disproportionate growth of the relatively hypoplastic ventral corporal wall or the reconstructed urethra. Surgical correction of symptomatic patients by dorsal shortening or ventral lengthening procedures depends on the degree of curvature following skin degloving of the penis. We have been recommending to parents of children born with proximal hypospadias who had what appeared to be a good surgical result to follow up after puberty.