Sutton-Kadir Syndrome can be treated safely with endovascular embolisation alone.

INTRODUCTION: Sutton-Kadir Syndrome (SKS) describes true inferior pancreaticoduodenal artery (IPDA) aneurysms in the setting of coeliac artery (CA) stenosis or occlusion. Although rare, SKS aneurysms can rupture and cause morbidity. Due to its rarity and lack of controlled treatment data, correct treatment for the CA lesion is currently unknown. Our aim was to assess if endovascular embolisation alone was safe and effective in treatment of SKS aneurysms, in emergent and elective settings. Secondary objectives were to describe presentation and imaging findings. METHODS: A retrospective cohort study of patients treated at Sir Charles Gairdner Hospital between January 2014 and December 2021 was done. Data on presentation, diagnostics, aneurysm characteristics, CA lesion aetiology, treatment and outcomes were extracted from chart review. RESULTS: Twenty-four aneurysms in 14 patients were identified. Rupture was seen in 7/15 patients. Most aneurysms (22/24) were in the IPDA or one of its anterior or posterior branches. Median arcuate ligament (MAL) compression was identified in all. There was no difference in median (IQR) maximal transverse diameter between ruptured and non-ruptured aneurysms (6 mm (9), 12 mm (6), P = 0.18). Of ruptures, 6/7 had successful endovascular embolisation and 1/7 open surgical ligation. Of non-ruptures, 6/7 had successful endovascular embolisation, 1/7 open MAL division then endovascular CA stenting and aneurysm embolisation. No recurrences or new aneurysms were detected with computed tomography or magnetic resonance angiography over a median (IQR) follow-up period of 30 (10) months in 12 patients. CONCLUSION: Endovascular embolisation of SKS aneurysms without treatment of MAL compression is safe and effective in both the emergent and elective settings.

Readmission Within the First Day of Discharge Is Painful: Experience From an Australian General Surgical Service.

Background Unplanned readmission to the hospital after discharge is a costly issue for healthcare systems and patients. It is a delicate balance between the resolution of the surgical problem and the length of hospital stay. Most studies have focused on readmissions within 28 or 30 days after discharge, despite data showing that many occur early in this period. This study examined the reasons for unplanned readmission within the first day after discharge.  Methods A retrospective cohort analysis of readmissions between 1st May 2016 and 1st May 2021 was undertaken by chart review. Readmissions on the "day of" and the "day after" discharge and their respective index admissions were identified via the hospital's patient administration database, webPAS (DXC Technology, USA). Results There were 126 readmissions (0.5%) across 25,119 admissions. Common reasons for readmission were pain (28%, n=35), readmission for the same diagnosis (21%, n=26), surgical site infection (SSI) (11%, n=14), bleeding (11%, n=14) and ileus (6%, n=7). Analysis of index admissions showed that 18/35 readmissions for pain had inadequate pain management based on pain scores, analgesic use and discharge medications and 7/14 readmissions for SSI did not have appropriate treatment of a recognised SSI or did not have antibiotic prophylaxis guidelines adhered to. Fourteen of 26 readmissions for the same diagnosis received just continuation of treatment initiated at index admission.  Conclusion Pain is the most common reason for readmission within the first day after discharge in surgical patients. Better pain management, following antibiotic prophylaxis guidelines, and involving patients in discharge planning could prevent many readmissions.

Body composition in patients with congenital myotonic dystrophy.

INTRODUCTION: Congenital myotonic dystrophy (CDM) is a rare neuromuscular disorder characterized by severe hypotonia and muscle weakness at birth that tends to improve with age. Understanding lean body and bone mass in this population has important research and clinical implications. The main objective of this study was to determine whether older children with CDM had muscle mass similar to healthy controls. METHODS: Thirty-five patients with CDM (3-13 years old) were enrolled. We analyzed lean body mass (LBM) and bone mineral content using the mechanostat framework, which allows calculation of z-scores for sex, age, and height. RESULTS: All patients had low LBM z-scores (muscle mass); however, higher LBM z-score was positively correlated with age (r = 0.45, P = 0.006), showing that it is closer to normal in older patients. DISCUSSION: Unlike other neuromuscular diseases, older children with CDM have a muscle mass closer to age-matched controls, consistent with the clinical profile of increasing strength in childhood. Muscle Nerve 60: 176-179, 2019.

Agitated behavior scale in pediatric traumatic brain injury.

Agitation following TBI commonly occurs during the acute recovery phase. The ABS is a valid measure of agitation in adults following TBI. The objective of the study was to determine if ABS scores accurately measure acute agitation in pediatric patients recovering from TBI. The ABS was completed twice daily for 4 days and mean ABS scores were calculated. Physicians assessed patients' agitation daily using a VAS. In addition, interventions for agitation were recorded. The association between ABS and VAS scores was assessed using Spearman's correlation. The relationship between the number of medication classes taken for agitation (0, 1-2, or ≥3) and ABS scores was assessed using one-way analysis of variance. Finally, the association between the use of hand restraints and ABS scores was examined using an unpaired two-sample t-test. Twenty-six pediatric patients with acute TBI were included. ABS scores significantly associated with VAS scores. Patients that required interventions for agitation (hand restraints or ≥3 medication classes) had higher ABS scores than patients that did not receive any intervention. The study supports the use of ABS scoring to measure agitation in pediatric patients with TBI. However, additional studies are warranted to further support the validity of this scale. Abbreviations: TBI: Traumatic brain injury; ABS: Agitated Behaviour Scale; VAS: visual analog scale; PCCU: Pediatric Critical Care Unit.

Prenatal, Neonatal, and Early Childhood Features in Congenital Myotonic Dystrophy.

BACKGROUND: Congenital myotonic dystrophy (CDM) is the neonatal onset and most severe presentation of Myotonic Dystrophy type 1. Since it first description, perinatal complications have been detailed including prolonged hospital stay, respiratory and feeding therapy during the neonatal period, although long-term complications are less documented. OBJECTIVE: Present a prospective cohort of CDM and compare it to the literature of other CDM case series, to adequately describe and contrast the prenatal, neonatal and infancy features of CDM. METHODS: A 5-year cohort of CDM eligible cases was conducted via the Canadian Pediatric Surveillance Program. 38 patients met the inclusion criteria. Comparison to other CDM case series published in the literature between 1992 and 2016 about perinatal and infancy morbidity. RESULT: From a total of 118 cases, the most frequent features were Polyhydramnios (58%), feeding therapy (77%), intubation and ventilation (58%); neonatal death was reported in 16% of the cases; the most frequent long-term morbidity were respiratory tract infections. CONCLUSIONS: We performed a detailed description of the main perinatal features of CDM and precise documentation of the mortality and morbidity during the first five years of life. This is an essential step in the knowledge of the natural history of CDM.

Developmental Milestones and Quality of Life Assessment in a Congenital Myotonic Dystrophy Cohort.

BACKGROUND: Congenital myotonic dystrophy (CDM) is a neuromuscular disorder caused by a CTG triplet repeat expansion in the DMPK gene. In addition to the expected motor delay, affected children often have significant developmental disability in language and cognitive realms, which ultimately impacts on quality of life. OBJECTIVE: In a prospective cohort of children with CDM to 1) present the profile of language and motor developmental milestones, and 2) describe their early childhood health related quality of life (HRQOL). METHODS: A five year cohort study of eligible incident cases of CDM was performed via the Canadian Pediatric Surveillance Program (CPSP). Consenting subjects were then followed from infancy in a prospective cohort study. Caregivers were contacted every 3 months for the first year of life, and then twice yearly in order to obtain data concerning language skills, motor development and parent proxy HRQOL from the PedsQL and Infant and Toddler Quality of life (ITQOL) questionnaires. RESULTS: Milestones were achieved at later ages in patients when compared to healthy children. Girls appeared to be more delayed than boys in both language and motor skills. HRQOL scores remained stable in this cohort, for both the PedsQL and ITQOL. CONCLUSIONS: Understanding developmental milestones and quality of life are important parameters when judging a child's overall health. For CDM patients delineating developmental milestones and QOL have important clinical care and research implications.