Traumatic Abdominal Wall Hernias Following High-Velocity Trauma in Children.

Traumatic abdominal wall hernias following blunt high-velocity trauma are uncommon in children and can result in concurrent abdominal visceral injuries. We present one such case of a 9 year-old boy requiring a trauma laparotomy to repair visceral injuries following a motor vehicle accident.

Temporal bone fractures: sequelae and their impact on quality of life.

PURPOSE: To present a prospective temporal bone fracture database, and study facial and cochleovestibular sequelae and their impact on quality of life. MATERIALS AND METHODS: Prospective study of consecutive cases of 39 patients with 45 temporal bone fractures over 11-month period in a university tertiary referral center. Based on epidemiological data, clinical and imaging findings, treatment modalities and outcome of patients with follow-up of one year, the present study focused on facial and cochleovestibular sequelae and their impact on quality of life after one-year period. RESULTS: After 12 months, 44% of patients present with balance problems, 56% with hypoacusis, 56% with tinnitus, and 15% with facial paralysis. In 75%-80% of patients, the cochleovestibular sequelae are described as disabling. Post-trauma quality of life was significantly impaired compared with pre-trauma quality of life, even after 12 months. Long-term cochleovestibular sequelae were significantly associated with poor long-term quality of life. CONCLUSIONS: The study demonstrates the need to focus on prevention of temporal bone fractures, notably by promoting the use of helmets and improvements in helmet design. The rapid diagnosis of temporal bone fracture is crucial as it enables effective initial management aimed at avoiding sequelae. The frequency of cochleovestibular sequelae after temporal bone fracture and their impact on quality of life demonstrate the importance of, and need for, ongoing follow-up by a local medical team who can diagnose and manage these long-term sequelae.

Study of pediatric appendicitis scores and management strategies: A prospective observational feasibility study.

OBJECTIVE: The objective was to investigate the feasibility of prospectively validating multiple clinical prediction scores (CPSs) for pediatric appendicitis in an Australian pediatric emergency department (ED). METHODS: A literature search was conducted to identify potential CPSs and a single-center prospective observational feasibility study was performed between November 2022 and May 2023 to evaluate the performance of identified CPSs. Children 5-15 years presenting with acute right-sided or generalized abdominal pain and clinician suspicion of appendicitis were included. CPSs were calculated by the study team from prospectively clinician-collected data and/or review of medical records. Accuracy of CPSs were assessed by area under the receiver operating characteristic curve (AUC) and proportions correctly identifiable as either low-risk or high-risk with the best performing CPS compared to clinician gestalt. Final diagnosis of appendicitis was confirmed on histopathology or by telephone/email follow-up for those discharged directly from ED. RESULTS: Thirty CPSs were identified in the literature search and 481 patients were enrolled in the study. A total of 150 (31.2%) patients underwent appendectomy with three (2.0%) having a normal appendix on histopathology. All identified CPSs were calculable for at least 50% of the patient cohort. The pediatric Appendicitis Risk Calculator for pediatric EDs (pARC-ED; n = 317) was the best performing CPS with AUC 0.90 (95% confidence interval [CI] 0.86-0.94) and specificity 99.0% (95% CI 96.4%-99.7%) in diagnosing high-risk cases and a misclassification rate of 4.5% for low-risk cases. CONCLUSIONS: The study identified 30 CPSs that could be validated in a majority of patients to compare their ability to assess risk of pediatric appendicitis. The pARC-ED had the highest predictive accuracy and can potentially assist in risk stratification of children with suspected appendicitis in pediatric EDs. A multicenter study is now under way to evaluate the potential of these CPSs in a broader range of EDs to aid clinical decision making in more varied settings.

The clinical potential of optogenetic interrogation of pathogenesis.

BACKGROUND: Opsin-based optogenetics has emerged as a powerful biomedical tool using light to control protein conformation. Such capacity has been initially demonstrated to control ion flow across the cell membrane, enabling precise control of action potential in excitable cells such as neurons or muscle cells. Further advancement in optogenetics incorporates a greater variety of photoactivatable proteins and results in flexible control of biological processes, such as gene expression and signal transduction, with commonly employed light sources such as LEDs or lasers in optical microscopy. Blessed by the precise genetic targeting specificity and superior spatiotemporal resolution, optogenetics offers new biological insights into physiological and pathological mechanisms underlying health and diseases. Recently, its clinical potential has started to be capitalized, particularly for blindness treatment, due to the convenient light delivery into the eye. AIMS AND METHODS: This work summarizes the progress of current clinical trials and provides a brief overview of basic structures and photophysics of commonly used photoactivable proteins. We highlight recent achievements such as optogenetic control of the chimeric antigen receptor, CRISPR-Cas system, gene expression, and organelle dynamics. We discuss conceptual innovation and technical challenges faced by current optogenetic research. CONCLUSION: In doing so, we provide a framework that showcases ever-growing applications of optogenetics in biomedical research and may inform novel precise medicine strategies based on this enabling technology.

Rapid utilisation of telehealth services for specialist paediatric general surgery outpatient clinics in response to COVID-19.

Background: The COVID-19 pandemic is highly infectious and prompted, amongst other changes, strict social distancing requirements for medical practitioners in Western Australia. Despite significant previous research into telehealth, uptake had been limited beyond servicing rural patients, in spite of numerous purported benefits. Results: Rapid adoption of telehealth for a majority of outpatient appointments was instituted in the sole tertiary paediatric general surgery with high overall success rates - a satisfactory outcome being achieved without requiring an in-person appointment (97.1% for telephone consults, 93.8% for videoconferencing) during the initial COVID-19 crisis from April to June 2020. Success of appointments was lowest for new referrals for undescended testicles at 81.3%. Operations booked through telehealth consultations were only altered in 1 case (5%), and this was not significantly different to in-person bookings (p > 0.05). No cases of COVID-19 were incurred by the surgical team or patients during the study period. Conclusions: We found that with existing technology and minimal training, paediatric surgical consultations were able to be performed via telehealth with high success, high accuracy, and without significant adverse outcomes.

Minimally invasive surgical treatment of pilomatrixoma.

BACKGROUND: Pilomatrixoma is a benign skin tumour often presenting as a firm irregular mass in the paediatric population. The most common site is on the head and neck. Traditionally, a wide local excision has been the method of management. We propose an incision and curettage (I&C) technique for an improved cosmetic outcome. METHODS: A retrospective review of children who underwent I&C for pilomatrixoma was done between January 2010 and June 2020. The I&C technique involved making a small incision over or near the lesion in a discrete location such as behind the hairline and removing the tumour piecemeal. Four to six weeks of routine post-operative follow-up was conducted. Patients and families were also subsequently contacted via a survey to assess for late recurrence, any other complications and ascertain their level of satisfaction with the outcome. RESULTS: Twenty lesions were removed in 11 patients over this time with a female predominance (seven) and most lesions were on the face (11). No patients had a recurrence in a mean follow-up time of 6 years (1-10 years). All parents are very satisfied with the cosmetic result. CONCLUSION: I&C may be an effective and cosmetically pleasing method to removing pilomatrixoma.

Pediatric transplantation: An international perspective.

Abdominal solid-organ transplantation has revolutionized the life of children with end-stage organ failure. The international practice of transplant in the pediatric population is heterogeneous. Global trends in pediatric transplant activity are increasing, with diffusion of transplant activities into developing and emerging economies. The organization of deceased donor programs varies internationally (with strong association to a country's gross domestic product (GDP) per capita and health spending). While deceased donor programs are well established in advanced economies, emerging and developing countries rely heavily on living donor programs. There are efforts underway to increase availability of pediatric and neonatal donor organs. Prioritization of organs for children exists in different forms throughout the world. Pediatric transplantation as a subspecialty is young but growing around the world with a need to train surgeons and physicians in this discipline. Outreach efforts with multinational and multi-institutional partnerships have enabled resource poor countries to establish new transplant programs for children. Further international collaboration, good quality data collection and audit, prospective research and ongoing mentorship, and education are needed to further improve outcomes of all children receiving solid-organ transplants.

Low-Dose Naltrexone: A New Therapy Option for Complex Regional Pain Syndrome Type I Patients.

Naltrexone (an opioid antagonist) has long been used in patients overcoming alcohol and opioid dependency. However, at doses one-tenth of those commonly prescribed for the above conditions, an unexpected effect occurs that aids in alleviating pain. Although there are currently no randomized clinical trials supporting the use of low-dose naltrexone, we present a case study describing the impact of compounding low-dose naltrexone that has dramatically improved the patient's pain symptoms which were refractory to other treatments.

Internal Auditory Canal Decompression for Hearing Maintenance in Neurofibromatosis Type 2 Patients.

BACKGROUND: In neurofibromatosis type 2 (NF2), multiple therapeutic options are available to prevent bilateral hearing loss that significantly affects the quality of life of patients. OBJECTIVE: To evaluate the morbidity and functional results of internal auditory canal (IAC) decompression in NF2 patients with an only hearing ear. METHODS: Twenty-one NF2 patients operated on for IAC decompression in a 3-year period with a minimum follow-up of 1 year were included in this retrospective study. They presented unilateral deafness due to previous contralateral vestibular schwannoma removal in 16 patients or contralateral hearing loss due to the tumor in 5 patients. Hearing level was of class A (American Academy of Otolaryngology-Head and Neck Surgery classification) in 7 patients, B in 8 patients, C in 1 patient, and D in 5 patients. Pure-tone average and speech discrimination score evaluations were performed at 6 days, 1 year, and during the follow-up. Eight patients had postoperative chemotherapy. RESULTS: No case of facial nerve palsy was observed. In the early postoperative period; all patients maintained the hearing class of the preoperative period. At 1-year follow-up, all but 3 patients maintained their hearing scores; at last follow-up (mean follow-up, 23 ± 8 months; range, 12-44 months), hearing classes remained stable with only 1 patient worsening from class B to C and 1 patient improving from class D to B. CONCLUSION: Decompression of IAC seems to be a useful procedure for hearing maintenance in NF2 patients, with very low morbidity. Ideal timing and association with chemotherapy should be evaluated in the future. ABBREVIATIONS: FN, facial nerveIAC, internal auditory canalNF2, neurofibromatosis type 2PTA, pure tone averageSDS, speech discrimination scoreVS, vestibular schwannoma.

A modern era comparison of right versus left sided congenital diaphragmatic hernia outcomes.

BACKGROUND/PURPOSE: This study aims to retrospectively review outcomes, including neurodevelopmental outcomes, of neonatal right sided congenital diaphragmatic hernias (RCDH) compared with left sided congenital diaphragmatic hernias (L-CDH) treated surgically at our institute. METHODS: A retrospective review was undertaken of all cases of congenital diaphragmatic hernia (CDH) treated at Princess Margaret Hospital for Children (PMH), Perth, born between 1st January 2002 and 1st August 2012. The outcomes of R-CDH cases were compared with L-CDH cases. We examined duration of ventilatory support, use of patch versus primary closure, the CDH recurrence rates, the number of reoperations and neurodevelopmental follow-up at one year of age. RESULTS: Forty-nine cases of CDH were operated on at PMH during the 10-year period. Of these, ten cases were R-CDH with 39 L-CDH cases. Of 49 cases, 34 were diagnosed antenatally, 5 R-CDH versus 29 L-CDH. Only 8/39 cases of L-CDH required patch repair for larger defects, while 5/10 R-CDH required patch repair. Postoperative mortality was 6/49 (1/10 right sided versus 5/39 left sided). Recurrence was observed in 5/10 R-CDH versus 6/39 L-CDH with p=0.03. Thirty-three of 43 surviving patients received one-year follow-up with Griffiths general quotient (GQ) assessment demonstrating a median score of 98 for L-CDH (IQR 86 to 104.25) and 91 for R-CDH (IQR 76.5 to 93). CONCLUSIONS: R-CDH required patch repair more commonly than L-CDH because of larger defect size or complete agenesis. The rate of recurrent herniation was the only morbidity significantly higher in the R-CDH group. Survivors of R-CDH did not have a significant difference in neurodevelopmental outcome compared to L-CDH cases, with both groups exhibiting normal median GQ scores at one year of age.

Inflammatory myofibroblastic tumour of the bladder in children: a review.

INTRODUCTION: Inflammatory myofibroblastic tumours of the bladder (IMTB) are rare, and feature a benign and reactive proliferation of myofibroblasts. 25% of the reported IMTB cases in the literature occur in children. The present study presents a review of IMTB in children. DISCUSSION: The data from 42 reported cases of paediatric IMTB in the world literature are summarised, including two recent cases from the present centre. Paediatric IMTB equally affects males and females. It mainly presents with haematuria, dysuria or abdominal pain. Lesions can vary in size, but mean size is 5.5 cm. Mean age is 7.5 years. The aetiology of IMTB is poorly understood, but includes infective or traumatic aetiologies, or a possible clonal lesion. IMTB may specifically show clonal gene rearrangements involving the anaplastic lymphoma kinase (ALK-1) gene. To differentiate IMTB from rhabdomyosarcoma, tissue diagnosis and careful histological analysis are essential. Tumour biopsy can be achieved by a transurethral approach or a transcutaneous approach with ultrasound guidance. Between 35 and 89% of cases of IMTB express ALK-1 by immunohistochemistry. ALK-1 expression is much less common in other bladder soft tissue tumours. ALK-1 is thus useful in the diagnosis of IMTB. The treatment of choice for IMTB is complete surgical resection of the lesion. In children, no proven recurrent or metastatic IMTB episodes are reported after excision. However IMTB recurrences are reported in adults, likely due to incomplete excision. Follow-up after excision is therefore recommended. CONCLUSIONS: Paediatric IMTB is uncommon. Tissue biopsy is essential for diagnosis. Careful histological assessment is required to differentiate IMTB from malignant paediatric bladder tumours such as rhabdomyosarcoma. ALK-1 expression is useful in confirming the diagnosis of IMTB. Treatment of choice is complete surgical resection of the lesion. Recurrence is reported in adult IMTB. Follow-up is therefore recommended.

Trend of management of traumatic thoracic aortic injuries in a single center.

The purpose of this study was to review the shift in the trend of management and mid-term outcomes of all patients who sustain thoracic aortic injury. A Retrospective analysis was performed of all patients sustaining blunt thoracic aortic trauma admitted to our unit. Forty-seven patients were presented with injury to the thoracic aorta following blunt chest injury. Ten patients underwent open surgical repair of their thoracic aortic injury. The mean age ± SD (range) was 29.4 ± 7.9 years (18-41) with a mean Injury Severity Score (ISS) of 41 ± 14.7 (25-75). Fifteen patients underwent thoracic endovascular repair for blunt aortic transections with a mean age of 35.1 ± 14.5 years (17-65), mean ISS of 40.8 ± 13.9 (20-75) and an average length of hospital stay of 25.6 ± 14.5 days (3-77). The mean aortic diameter proximal to the aortic injury was 23.46 ± 3.02 mm (19-28) with a mean aortic angulation of 58.46° ± 17.73 (44-80°). The mean oversizing was 24.4 ± 5.4% (17-32%). At our institution, there has been a paradigm shift in the emergent repair of blunt thoracic aortic injury from open surgery to endovascular repair. Oversizing of the stent-graft did not translate to a poorer outcome.

Meningioma of geniculate ganglion: case report and review of the literature.

Although meningioma is a frequent intracranial tumor, it rarely affects the geniculate ganglion of the facial nerve. Facial palsy is the most common symptom. When hearing is preserved (class A or B, AAO-HNS), tumor is best removed through a middle cranial fossa approach. We report the case of a geniculate ganglion meningioma and present its clinical, radiological, and pathological features. Surgical management is discussed. A literature review revealed that only 17 previous cases have been reported during the last 50 years.