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Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital condition, characterized by multiple venous malformations that may involve any organ system, most commonly the skin or the gastrointestinal tract. These lesions are often responsible for chronic blood loss and secondary anemia, and in rare situations may cause severe complications such as intussusception, volvulus, and intestinal infarction. Intussusception as a complication of BRBNS, although a known complication of the disease, has rarely been reported, especially in the Philippines. In the Philippine Society for Orphan Disorders, only 2 cases of BRBNS are currently included in the organization, including the patient presented in the case report. The treatment of BRBNS that involves the gastrointestinal tract depends on the extent of intestinal involvement and severity of the disease. The treatment aims to preserve the GI tract as much as possible due to the high recurrence in the disease. In this case report, we present a 13 year-old male with BRBNS with previous history of intussusception, successfully managed conservatively;however, upon recurrence, underwent exploratory laparotomy wherein a subcentimeter perforation in the antimesenteric border of the proximal ileum was noted, together with a gangrenous intussuscipiens, and multiple mulberry-like formations on the antimesenteric border of the small bowels. Histopathological findings of the resected bowels showed multiple cavernous hemangiomas consistent with BRBNS. The postoperative course of the patient was unremarkable.Copyright © 2023 The Authors
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Objective: To describe a case of SARS-CoV-19-associated encephalitis in a neonate. Method(s): Case report. Report: A 9-day-old term neonate presented with two focal motor seizures (right upper limb jerking and facial twitching). He had a 1-day history of coryzal illness with reduced feeding, but was afebrile. Antenatal course was uneventful. He was born at term via vaginal delivery. He did not require resuscitation or admission to SCBU. Maternal history was notable for symptomatic SARS-CoV-19 infection at time of delivery. Two siblings subsequently tested positive for SARS-CoV-19. He had further seizures in the emergency department and was loaded with phenobarbitone. The infant was stabilised locally and transferred to a tertiary paediatric hospital for the management of neonatal sepsis. He never required respiratory support. However, he was diffusely hypotonic with poor suck, necessitating nasogastric feeding. Nasopharyngeal PCR was positive for SARS-CoV-19. Lumbar puncture microscopy was negative (WCC 6). All CSF bacterial and viral investigations were negative. CSF testing of SARS-CoV-19 was not available. Brain MRI revealed bilateral asymmetric areas of reduced diffusivity involving the subcortical white matter, medulla and the corpus callosum with frontal lobe predominance. He made a full neurologic recovery with supportive therapies and was discharged following a 9-day admission. He had no further clinical seizures and phenobarbitone was successfully weaned pre-discharge. Conclusion(s): In the absence of another aetiology or antenatal risk factor, SARS-CoV-19 infection was presumed causative in this case of focal seizures and white matter changes in this term neonate. White matter abnormalities on MRI imaging are reported in neonates with seizures in the context of other viral infections. Single case reports have been published of SARS-CoV-19 infection with associated abnormal MRI brain findings, particularly diffusion abnormalities of the corpus callosum, as seen in our case.
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Background and Aims Sarcopenia can be defined as loss of muscle mass, strength and function and has been shown to be associated with increased morbidity and mortality in the adult population. Sarcopenia has been assessed by decreased psoas muscle surface area (PMSA) on Computer tomography (CT) and has been validated in paediatric studies. The impact of Sarcopenia in children with end stage liver disease and oncological conditions is now being recognised. There is scarce literature on the effect of sarcopenia on motor function. CT imaging exposes children to radiation and hence is done in a select group of children at the time of transplant assessment. The aim of this audit was to assess the prevalence of Sarcopenia in children undergoing liver transplant assessment and its relationship on laboratory variables, functional activity and clinical outcomes. Methods Retrospective single centre case review of patients with liver disease undergoing transplant assessment and CT imaging between 2018-2020. Psoas muscle was analysed at the level of L4/L5. The z-Scores were calculated using ageand gender-specific reference values. Sarcopenia was defined as tPMA z score less than -2. We assessed the relationship of Sarcopenia to the biochemical parameters, nutritional status, effect on motor delay/physical abilities (assessed by a range of age appropriate standardised developmental and physical assessments due to COVID pandemic isolation restrictions) and post-transplant complications. Results Thirty one children that met the inclusion criteria were included. Sarcopenia was prevalent in 17 children (6 males: 11 females), with a median age of 3.5 years (SD = 4.9). The common conditions were biliary atresia (n= 11, 35%), hepatoblastoma (n=6, 19%), Autoimmune hepatitis (n=3) etc. Twenty- four patients required additional nutritional support (77% nasogastric feeding, 13% PN and 6% oral supplementation). Mean tPMA z-score was -2.27. Data for the assessment of physical abilities/functional activity was available in 21 children. Impairment of motor skills/physical abilities was overall noted in 14/21 children (67%);9/13 (69%) in the sarcopenic group (6 significant impairment) vs 5/8 (63%) in non sarcopenic group (4 significant impairment). Sarcopenia was associated with increased complications (27 vs 7, p = 0.005) and hypoalbuminaemia (p=0.01), but was not statistically significant (p> 0.05) for the overall length of stay (total and intensive care). Discussion Sarcopenia was commonly identified in children with liver diseases undergoing transplant assessment. Reduction in physical abilities/functional activity was observed in both groups which may be a consequence of loss of muscle mass in children secondary to liver diseases or underlying oncological conditions leading to delay in gross motor skills. Although there was no statistical difference in the duration of stay or impairment of motor skills, complications were higher in the sarcopenic group. Conclusion In this pilot study, sarcopenia is prevalent in children being assessed for liver transplantation and was associated with increased complications. Better non-invasive methods (aside from CT scan) of assessing sarcopenia needs to be developed and validated for the paediatric age group, which would help to better characterise the true incidence and prevalence of sarcopenia in children with chronic liver disease. There is a need to offer nutritional support and assess physical function early in the pre transplant period in order to initiate appropriate physiotherapy interventions to halt and even reverse the progression of sarcopenia.
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Introduction: Intensive care patients often have complex swallowing and communication needs. These require coordinated input from the multi-disciplinary team. Increasing evidence highlights the role of speech and language therapy (SLT) within the critical care environment1 and this is represented well in national recommendations specific to patients with tracheostomies. Approximately 10-15% of ICU patients will have a tracheostomy nationally.2 SLT provide expertise in assessment and management of communication and swallowing difficulties, which can vastly improve psychosocial well-being and promote early safe enteral feeding for our patients.3 In 2014 On the right trache?4 found that 52% of patients with a tracheostomy suffered with dysphagia, however only 27% critical care patients had input from SLT. Objectives: To improve the assessment of swallowing and communication in patients undergoing tracheostomy ventilation at the Royal Infirmary of Edinburgh, to comply with Scottish Intensive Care Society Audit Group (SICSAG) quality indicator 2.3. This guidance stipulates all tracheostomy patients should have communication and swallowing needs assessed during ventilator wean.5 Methods: Four distinct areas of intervention were implemented. Pre-populated review text was added to NHS Lothian's clinical notation system (InterSystems TrakCare®), prompting nursing staff to consider swallowing, tracheostomy issues and SLT referral. These were refined between audit cycles. SLT were invited to join safety briefs to identify tracheostomy patients, as well as other patients with complex swallowing needs. This was an opportunity to raise awareness, educate, and prioritise workload. New guidelines for nurse-led swallowing observations were developed and disseminated amongst teams. Finally, staff were offered relevant educational sessions. Baseline data was collected in 2019;serial data collection was then during October - November 2020 and in June - July 2021 following the interventions. Results: All patients who received tracheostomy ventilation were audited (n=31). This showed that very few patients had swallowing and communication assessed adequately. Only 16.1% (n=5) patients had a regular nursing swallowing assessment on the majority of critical care days (>50%). Referral to SLT was often very late when patients were approaching de-cannulation and on many occasions by the time the SLT team were involved patients had already been de-cannulated. Following the intervention period, it became apparent that awareness of swallowing requirements had improved. By mid 2021, 58.9% more patients had swallowing assessed as part of daily care. Additionally, 81.2% of patients had SLT reviews on the unit demonstrating a sustained increase from late 2020. There was a notable increase in the quality of assessments after initial SLT review. Conclusion: Using quality improvement methodology our multidisciplinary team was able to substantially increase the quality of swallowing assessment within our ICU, despite the challenges of the COVID-19 pandemic. Our unit now complies with SICSAG quality indicator 2.3. This is in keeping with national recommendations for a multidisciplinary approach to care of tracheostomy patients. Patients with increased risk of dysphagia are being identified earlier and are more likely to progress and be established on enteral feeding early, which may subsequently reduce the burden of nasogastric feeding, total parenteral nutrition and even related invasive IV access.
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Introduction: Unintentional weight loss is common in lung cancer, with 40-60% of patients presenting with this at diagnosis. Weight loss and depleted nutritional status have been identified as negative prognostic variables for survival and directly impact the effectiveness of cancer treatments. The Lung Oncology team at the University Hospital Southampton (UHS) received external funding from Bionical Solutions and AstraZeneca for a part time oncology Dietitian to join the team in treating this patient group. The aim was to determine if specialist Dietitian input improves patients’ nutritional outcomes in those diagnosed with Stage III Non-Small Cell Lung Carcinomas (NSCLC) undergoing radical treatment. Methods: Over 12 months (February 2021-February 2022) all patients with stage III NSCLC received specialist Dietitian input during their radiotherapy treatment. Non-patient identifiable data was collected, which included;whether patients were enterally fed, admission rates and reason, and their weights at the start, middle, end and 2 weeks post radiotherapy. This data was compared to previously collected data in 2018 with the same patient group. No ethical approval was required. Results: A total of 50 patient data sets and 11 feedback questionnaires were collected over 11 months. Between the start and end of radiotherapy, patients experienced;2.1% overall average weight loss, 64% experienced <3.0% unintentional weight loss and 0% experienced >9.6% unintentional weight loss. In 2021 patients reached their lowest weight earlier at 70% of their way through radiotherapy compared with 86% in 2018. Patients maximum unintentional weight loss was 3.2% in 2021 compared with 4.4% in 2018. This is likely due to closer monitoring and dietitian input in 2021. Admission rates were higher in 2021 (n=13, 26%) compared to 2018 (n=5, 18.5%). However, this may be due to changes to patients’ radiotherapy treatment plans in 2021 due to the covid pandemic, resulting in more intense treatments. Patients requiring nasogastric (NGT) feeding increased from 0 in 2018 to 6 in 2021. This is likely due to increased awareness of the importance of nutritional support attributable to dietetic involvement in the multidisciplinary team. All patients who completed the feedback questionnaire found dietetic consultations useful and were able to follow most, or all dietary advice. 91% felt well supported during their treatment with dietetic input. Final Outcomes: Patients experienced reduced weight loss during treatment with Dietitian input compared to 2018 data where there was minimal dietetic input. Increased number of patients required NGTs compared to 2018, therefore Dietitian input is required in this area of oncology. Most patients felt well supported receiving dietetic input during treatment. Increased admission rates compared to 2018, however more nutrition support related admissions in 2021. Conclusions: Overall, patients lost less weight during treatment with Dietitian involvement in their care which is a positive factor in the prognostic outcomes. In addition, most patients felt seeing a Dietitian during treatment improved their experience and felt well supported. The final outcomes support the British Dietetic Associations’ recommendation that there is a dedicated dietetic service for lung cancer patients’, and they are seen by a Dietitian during their treatment. Keywords: Dietitian involvement, Reduced weight loss, Improved patient outcomes
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Aims and objectives: To establish that non-invasive ventilation (NIV) can be substituted by high flow nasal cannula (HFNC) for respiratory support during oral feeding of a patient with COVID-19 patients. Materials and methods: This prospective case series was conducted after taking informed and written consent from the patients. Ten patients with severe COVID-19 disease requiring NIV with inspiratory pressure of <10 cm H2O, positive end-expiratory pressure of <6 cm H2O and FiO2 <0.6 were included in this study. Patients with altered consciousness, circulatory failure, or worsening acidosis were not included in the study. Patients underwent HFNC trial for 10 minutes and were screened for risk of dysphagia and aspiration using a 3-ounce water swallowing test. The patients were given a trial of HFNC for 10 minutes with a flow of 60 L/minute and FiO2 of 0.1 more than their requirement on NIV. The patients were observed for hypoxemia (SpO2 <88%) or signs of respiratory distress, e.g., increase in respiratory rate (>35/minute), laboured breathing pattern, use of accessory muscle of respiration, heart rate (>20% change), blood pressure (>20% change), perspiration, and anxiety. Then, HFNC was used for supporting respiration during oral feeding for up to 20 minutes. Feeding was started with a hypocaloric target on starting day and was increased progressively as per European Society for Clinical Nutrition and Metabolism guidelines to the target estimated caloric requirement. Results: The HFNC support for oral feeding was successful with adequate diet intake in eight patients without desaturation/respiratory distress during oral feeding. Other than COVID-19, co-morbidities in these eight patients included diabetes mellitus, obesity, chronic obstructive pulmonary disease, coronary artery disease, and dilated cardiomyopathy. Six patients, previously on enteral nutrition using the nasogastric tube, were successfully switched to oral feeding with help of HFNC. Four patients were directly started on the oral diet with help of HFNC support. HFNC could not support respiration adequately in two of these four patients. The initial trial was successful for one of the patients and HFNC support for oral feeding was used for 3 days, but a progressive increase in ventilatory requirements resulted in failure of HFNC trial subsequent days and the patient was switched to nasogastric feeding. In another patient, the initial trial of HFNC failed due to rapid desaturation within a few minutes of the trial. The eight patients in whom HFNC was used successfully for feeding were switched to HFNC completely and discharged from the hospital after weaning off from oxygen support. The patients who failed the HFNC support for feeding required higher ventilatory requirements and needed endotracheal intubation. Conclusion: Based on our case series, using daily screening trial of oral feeds with HFNC support in selected patients of severe COVID-19 pneumonia on NIV seems thought-provoking and should be explored for its potential in improving patient's nutrition with a positive impact on the outcome.
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Patients receiving Continuous Positive Airway Pressure (CPAP) therapy for severe COVID-19 are at high risk of malnutrition1,2. This is related to poorer outcomes and longer durations of hospital stay3. British Dietetic Association guidance recommends nasogastric (NG) feeding for all COVID-19 patients on CPAP4. The aim of this Quality Improvement Project (QIP) was to optimise nutrition in COVID-19 patients receiving CPAP therapy in a ward-based setting at a UK District General Hospital. The QIP included 222 patients in total across four QI cycles. Four domains: meeting nutritional requirements (outcome measure), dietitian review, accurate weight and NG feeding (process measures) were studied. Whether patients were reviewed by dietitians, weighed and NG fed was determined for all 222 patients. Meeting of estimated nutritional requirements could be determined in 108 patients. Interventions included use of a local nutrition pathway, COVID-19 diet sheet, NG feeding and involvement of dietitians at a daily multi-disciplinary meeting. Descriptive statistical analysis in the form of a Chi- Squared test was used to compare the first two COVID19 waves. No ethical approval was required. Results showed significant improvement in the proportion of patients reviewed by dietitians, accurately weighed and NG fed between the first two waves of COVID19 (p<0.05). Comparing the 1st to the 4th QI cycle;meeting requirements improved from 50% to 68%, dietitian review from 29% to 91%, accurate weights from 44% to 87% and NG feeding from 6% to 65%. These improvements were achieved through novel approaches, rapid implementation of new guidance and multi-disciplinary working. Next steps should include introduction of a standardised care proforma and study in future QI cycles. These interventions could be trialled in other inpatient wards to deliver wider benefit. This QIP was successful in optimising nutrition in this cohort of patients and thereby delivering better patient care. References 1. Turner P, Montgomery H, Stroud M, et al. (2021) Malnutrition risk in hospitalised COVID-19 patients receiving CPAP. Lancet 397:1261. 2. Terzi N, Darmon M, Reignier J, et al. (2017) Initial nutritional management during noninvasive ventilation and outcomes: a retrospective cohort study. Crit Care 21:293. 3. Singer P, Blaser AR, Berger MM, et al. (2019) ESPEN guideline on clinical nutrition in the intensive care unit. Clin Nutr 38:48–79. 4. BDA Critical Care Specialist Group COVID-19 Best Practice Guidance: Feeding Patients on Critical Care Units in the Prone Position (awake and sedated). Second Edition. [accessed 31st May 2021]
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It has been a hugely challenging task proving nutritional support in adult intensive care units during the COVID-19 pandemic. We therefore reviewed the nutritional parameters of patients admitted to intensive care during both surges of the virus to provide detailed information and to ensure we are fully informed to provide the best service in future surges. Retrospective data was collected from 168 patients using dietetic electronic handovers from 04.10.20 to 04.04.21 and compared to the data collected for 122 patients in surge one, (data collected 20.03.20 to 8.5.20). This was analysed using an excel spreadsheet. The results are outlined in table one below: [Formula presented] The critical care ventilation plan for these patients had been reviewed1 and this impacted the route of feeding as follows: Nasogastric feeding on admission: 50.6% (96%), oral nutritional support: 35% (4%), oral nutritional support with subsequent NG feeding: 11% (3%), parenteral nutrition: 1.8% (0), PEG: 0.62% (0). Using data collected from surge one, we were able to prepare training for the dietetic team with regards to the demographics of the patient and the impact on nutritional care. This is particularly important with regards to the challenge of meeting energy and protein requirements of obese patients2. We reviewed the difficulties gathering accurate weights from the first wave and purchased new patient transfer scales®. This allowed us to gain weights that we would not have been able to. More patient heights were available as dietitians were present on the unit in surge two (remote working in surge one) and were able to do bedside measures of ulna length where a height was unavailable. Using surge one data we were prepared for high numbers of patients requiring enteral feeding on admission. However there was a different picture in surge two with more patients awake, using high flow oxygen therapy or on a CPAP hood. This brought different challenges with meeting macro and micronutrients orally. Patients with breathing difficulties were not keen to consent to an NGT and when they did, the NGT insertion procedure was tricky in those patients with high Fi02. We reviewed our range of macronutrient supplements and started using an oral protein supplement containing 30 mls, 10 g protein and 100 kcal to help with this. We attended MDT meetings to discuss feeding route and for further surges we plan to implement a standard operating procedure for enteral feeding patients on CPAP. 1. Intensive Care Society (2021) Clinical Guideline for the management and care of critically ill adults with COVID-19 during the coronavirus pandemic. Faculty of Intensive care Medicine. 2. Singer et al (2019) ESPEN guideline on clinical nutrition in the intensive care unit: Clinical Nutrition 38:48-79
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Early enteral feeding is important in maintaining the integrity of the gastrointestinal tract mucosal barrier and associated with less bacterial translocation and decreased stimulation of the systemic inflammatory response and subsequent improved outcomes in intensive care (ICU) patients. Enteral feeding by nasogastric (NG) tubes is the preferred route of nutritional support for most ICU patients. However, ICU patients with delayed gastric emptying and poor intestinal motility may not tolerate gastric feeding and may therefore benefit from post-pyloric feeding via nasojejunal (NJ) tubes1. We reviewed the effectiveness of 35 NJ tube placement in 24 patients on ICU between January and March 2021. The M:F ratio was 4:1, median age 69 years (30–80 years) and 54% of patients were non-White British. 10 patients (42%) had diabetes and 54% had COVID-19 as part of their admitting diagnoses. The median BMI was 25 (range 20 – 32.3) and none of the patients were identified as high risk for refeeding syndrome at the time of NJ tube insertion. Nutritional information was unavailable on 5 patients. Of the remaining 19 patients, 26% of patients (n=5) were commenced on parenteral nutrition (PN) within 48 hours of NJ insertion. Only 1 patient was able to meet their nutritional requirements enterally via NJ tube at 5 days;a further 2 patients had their nutritional requirements met with supplemental PN. In 8 of 22 referrals the indication for NJ tube insertion was because an NG tube could not be passed. The evaluation revealed discrepancies in adherence to protocols for high gastric residual volumes and prokinetic use. Documentation surrounding decision making, requesting and inserting an NJ tube was poor and probably reflects the complexity of the patients, involvement of multiple clinical teams, and various documentation modalities (i.e., verbal, written and different electronic systems). There was clinical dispute regarding the indication for NJ tube insertion in 23% of cases (documented in 3 of 13 referrals for NJ tube insertion). Where documentation was available 43% of patients (n=10) had an NJ tube placed on the day of request;the median time from request to insertion was 1 day (range 0-10). 5 patients had more than one NJ tube inserted (median 3;range 2–5). There was variation in experience and expertise of the endoscopists placing the NJ tubes. NJ tube feeding is considered to be less expensive and have less complications than PN2. However, our evaluation has revealed a range of issues relating to both the insertion and use of NJ tubes in an ICU setting. The true resource ‘cost’ of NJ tube insertion is probably underestimated in the literature and the complications of PN probably overestimated in the context of modern ICU and nutrition support team clinical practices. We suspect that our clinical experience is not unique and that more research is needed in this area. We are using this work to educate clinical teams, standardise documentation, provide better support and supervision for endoscopists, and raise awareness of the benefit and need for supplemental PN where nutritional requirements are not consistently reached enterally. 1 Schröder S, Hülst S V, Claussen M et al. Postpyloric feeding tubes for surgical intensive care patients. Anaesthetist 2011;60 (3): 214-20. 2 Lochs H, Dejong C, Hammarqvist F et al. ESPEN Guidelines on enteral nutrition: Gastroenterology. Clin Nutr 2006;25(20: 260-74.
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With the increase in use of smaller magnets in gadgets and toys at home, magnets pose a growing aspiration risk in children. We present two simultaneous cases of magnet-related foreign body aspiration (FBA) in two children, in two different cities: Karachi, and Lahore. They presented with similar signs and symptoms: tachypnea, tachycardia and asymmetric breath sounds on auscultation. They were initially diagnosed with the help of a chest X ray. Both the cases were complicated by failed bronchoscopy attempts due to the slippery texture of the magnet. Due to the failed bronchoscopy, both patients had a prolonged and complicated course including a 24–48 hour stay in the PICU prior to magnet removal. They eventually had to undergo thoracotomy for successful removal of the magnet. Both had an unremarkable post-operative course and were discharged in good health.
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Case Report Anorexia Nervosa is a mental health disorder with significant morbidity and mortality. Acute food refusal is one of the indications for admission. We present a patient who went to extreme lengths to restrict food intake, requiring intensive care sedation and ventilation to enable enteral feedings. 12 year old male, was admitted with symptoms of anorexia nervosa and BMI of 12.0, (<1%ile) with baseline BMI of 16 (25%ile), K of 3.3 and glucose of 54. He was treated with supervised eating on an inpatient pediatric floor with no need for enteral feeding. Psychiatry consultation confirmed the diagnosis of anorexia nervosa and recommended the addition of Olanzapine to his Sertraline. He was discharged pending placement in an eating disorder center after 21 days of hospitalization with discharge BMI of 14. He was followed as an outpatient by his pediatrician, dietician and counselor but unfortunately, he required readmission 11 days after discharge due to acute food refusal, with BMI that had dropped to 13.1. Patient was readmitted and started on nasogastric (NG) feeds but he became severely agitated, pulling NG out multiple times and continued to lose weight with BMI dropping to 12. Sedation was attempted to facilitate maintenance of NG feedings, with Benadryl, Haldol and Ativan, but was ineffective at levels deemed safe without compromising his airway and breathing. Due to severe malnourishment and unsuccessful NG feeds he was transferred to PICU for sedation, endotracheal intubation and continuous nasoduodenal (ND) tube feedings on two separate occasions while inpatient. He was able to wean from the ventilator but once awake he found ways to manipulate delivery of his calories, even finding scissors and cutting the ND tube. The patient ultimately agreed to eat in order to avoid replacement of the feeding tube. He was finally transferred to an eating disorder facility, with a BMI of 13.9 and persistent anorexia thinking with restriction of eating anything but pizza. Patient completed three months of an inpatient program and had significant improvement in BMI to 19.3 (70%ile). He was subsequently discharged for continued outpatient follow-up and since discharge from the eating disorder center, his BMI has shown steady improvement in outpatient follow-up. He shows no signs of food refusal and is doing well with Family Based Therapy. This case highlights several unique characteristics in management of eating disorder patients. The age and being male along with extreme food refusal and resistance to enteral feeding that led to the requirement of deep sedation are quite unusual and not well described in the medical literature. The severity of his illness was a significant barrier to inpatient placement. In addition, despite a nationwide attempt to find an inpatient facility for him, which took several weeks, we identified shortages in eating disorder beds that have been exacerbated by the COVID-19 pandemic.