Pemphigus Vulgaris and Bullous Pemphigoid of the Upper Aerodigestive Tract: A Review Article and Novel Approaches to Management.

BACKGROUND: Autoimmune bullous diseases are rare conditions characterized by blistering of the skin and mucous membranes. The 2 commonest forms are pemphigus vulgaris and bullous pemphigoid. The oral cavity or oropharynx may be the initial site of presentation or often the only site involved. SUMMARY: These conditions are often misdiagnosed or overlooked leading to poorer patient outcomes. Due to the chronic nature of these conditions and the systemic effects of treatment, there is a significant associated morbidity and mortality. As such, an understanding of the fundamentals of autoimmune bullous diseases is vital to those working in otolaryngology. The mainstay of management in both conditions is topical and systemic corticosteroids. There is also a role for immunomodulating and non-steroidal anti-inflammatory drugs as adjunct or alternative therapies. Surgical intervention may be required to protect the airway. Often multimodality treatment is required involving multidisciplinary input from otolaryngologists, oral surgeons, dermatologists, and rheumatologists. This review article will highlight the aetiology, pathology, clinical features, investigations, and management of both pemphigus vulgaris and bullous pemphigoid including recent advances in management.

Delays in surgery for cholesteatoma due to COVID-19: is there an impact on rates of recidivism and major complications?

PURPOSE: To evaluate the relationship between the waiting time for surgery, and cholesteatoma recidivism rates and major complications. The secondary aims were to identify any other prognostic factors for cholesteatoma recidivism. METHODS: A retrospective single-centre study of 312 patients who underwent cholesteatoma surgery under the care of a single-surgeon, between 2004 and 2018, was performed. Waiting times for surgery were categorised into ≤ 90 days, 91-180 days, 181-270 days and > 271 days. The outcome measures were cholesteatoma recidivism and major complications (facial nerve palsy or intracranial complications). RESULTS: The mean age was 36.1 years ± 21.5 with 242 adults (77.6%) and 70 children (22.4%). The mean waiting time for surgery was 126.2 days (4.1 months) ± 96.0 days and the overall rate of recidivism was 11.2% (35/312 patients). No instances of facial nerve palsy or intracranial complications were identified. Rates of recidivism by waiting time for surgery were: 15.3% for 118 patients who waited ≤ 90 days, 9.7% for 134 patients who waited 91-180 days, 6.7% for 30 patients who waited 181-270 days and 4.3% for 23 patients who waited > 271 days. There was no significant difference amongst the different waiting time groups for rates of recidivism (p = 0.266). CONCLUSION: Increased waiting times for cholesteatoma surgery do not appear to be associated with increased rates of recidivism or major complications. Clinical judgement will always be required for complicated disease or patients with additional risk factors. The other prognostic factors for recidivism identified in this study were age (< 15 years) and congenital cholesteatoma.

Tracheal swab from front of neck airway for SARS-CoV-2; a bronchial foreign body.

We report the case of a bronchial foreign body, following a tracheostomy site swab for SARS-CoV-2, aiming to raise awareness and vigilance. A qualified nurse was performing a routine SARS-CoV-2 swab on a 51-year-old woman, fitted with a tracheostomy in the recent past following a craniotomy. This was part of the discharging protocol to a nursing home. During the sampling, part of the swab stylet snapped and was inadvertently dropped through the tracheostomy site. Initial CT imaging was reported as showing no signs of a foreign body but some inflammatory changes. Bedside flexible endoscopy through the tracheostomy site revealed the swab in a right lobar bronchus. This was subsequently removed by flexible bronchoscopy. This case highlights the need for clear guidance on how samples for SARS-CoV-2 are taken from patients with front of neck airways (laryngectomy/tracheοstomy) and the potential pitfalls involved.

Semirigid cervical spine collar and risk of missing significant soft tissue injuries.

We report the case of an initial misdiagnosis of significant scalp soft tissue hidden beneath a semirigid cervical collar. A 16-year-old unrestrained rear seat passenger was involved in an RTA sustaining a subdural haematoma, cervical spine fractures and what was initially diagnosed as a significant degloving scalp injury. The patient was admitted to the intensive care unit intubated sedated, and with a cervical collar. The scalp injury was dressed with a view that potential reconstructive surgery would be needed subsequently.This case demonstrates that while cervical collars remain a fundamental aspect of initial prehospital care in trauma, they have potential drawbacks to their use, which need to be carefully noted. Some of these drawbacks have been reported extensively in the literature. This case outlines the necessity of a thorough secondary survey in the trauma patient as the cervical collar can obscure the assessment of significant head and neck soft tissue injuries.

Outcomes of laparoscopic Kasai portoenterostomy for biliary atresia: A systematic review.

AIMS: Biliary atresia (BA) is a rare disease for which mainstay of treatment consists of open Kasai portoenterostomy. The aim of this review was to assess the outcomes of laparoscopic Kasai portoenterostomy, which offers potential benefits of minimally invasive surgery. Outcomes identified were postoperative cholangitis rates, incidence of adhesions at subsequent liver transplantation, native liver survival rates and actuarial survival rates. METHODS: A comprehensive systematic literature search was conducted in the PubMed and Cochrane databases using the keywords hepatic portoenterostomy, biliary atresia and laparoscopy. Robotic cases were excluded. RESULTS: Ten studies (n=149 patients) were included in this review. The mean age at the time of operation was 66 (range 14-119) days. The mean operative time was 261 (range 120-662) minutes. The rate of postoperative cholangitis was 34% (range 11%-50%). The mean native liver survival rate was 57% (range 33%-78%) at 6months and 47% (range 8%-76%) at 2years. Mean actuarial survival rate was 87% (range 54%-100%) at 2years. Subsequent adhesions were reported in 4 patients. Two patients had dense adhesions and 2 had no adhesions. CONCLUSIONS: Although laparoscopic Kasai portoenterostomy is a feasible operation, outcomes in terms of native liver survival rates and actuarial survival rates are unfavourable compared to conventional surgery. There is no evidence that laparoscopic Kasai is associated with fewer adhesions at subsequent liver transplantation. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Treatment study.