The association between sinonasal anatomical variants and the laterality of orbital complications in pediatric acute rhinosinusitis.

INTRODUCTION: As the role of sinonasal anatomical variants as predisposing factors in determining the lateralization of acute rhinosinusitis-related orbital complications (ARS-OC) in pediatrics remains a topic of debate, this study further explores the potential association between anatomical variations and ARS-OC. METHODS: A retrospective study was conducted on children who had been admitted with ARS-OC using medical records and sinus CT scans to compare anatomical differences between the affected and contralateral sides. This study aimed to identify bony anatomical disparities that may impact OC laterality secondary to ARS. The anatomical features examined included septal deviation, concha bullosa, lamina papyracea dehiscence (LPD), and uncinate process abnormalities. RESULTS: The CT scans of 57 pediatric patients (114 sides) were reviewed. Our results indicated that bony anatomical variations were associated with ARS-OC laterality (63 % vs. 37 %, P = 0.006), yielding an odds ratio of 2.91. Additionally, our study revealed a significant association between ipsilateral LPD with the increased risk of ARS-OC (39 % vs. 1.8 %, P < 0.05), with an odds ratio of 34.3 compared to the opposite side. CONCLUSIONS: LPD might play a role in the pathophysiology of pediatric ARS-OC, as it is associated with a significantly higher risk of affecting the ipsilateral side. Further research is necessary to determine whether LPD is a causative factor or a result of ARS.

Hypoparathyroidism-retardation-dysmorphism syndrome-Clinical insights from a large longitudinal cohort in a single medical center.

Background: Hypoparathyroidism, retardation, and dysmorphism (HRD) Syndrome is a rare disease composed of hypoparathyroidism, retardation of both growth and development, and distinctive dysmorphic features. Here, we describe the long-term morbidity and mortality in a large cohort of HRD patients and suggest recommendations for follow up and treatment. Methods: Medical records of 63 HRD syndrome patients who were followed at Soroka Medical Center during 1989-2019 were reviewed retrospectively. Information regarding demographics, medical complications, laboratory findings, and imaging studies was collected. Results: The mortality rate was 52%. The main causes of death were infectious diseases including pneumonia, septic shock, and meningitis. Multiple comorbidities were found including brain anomalies in 90% of examined patients (basal ganglia calcifications, tightening of corpus callosum, Chiari malformation, hydrocephalous, and brain atrophy), seizures in 62%, nephrocalcinosis and/or nephrolithiasis in 47%, multiple eye anomalies were recorded in 40%, bowel obstructions in 9.5%, and variable expression of both conductive and senso-neural hearing loss was documented in 9.5%. Conclusion: HRD is a severe multisystem disease. Active surveillance is indicated to prevent and treat complications associated with this rare syndrome.

Pregnancy in Pateints With Exstrophy-Epispadias Complex: Are Higher Rates of Complications and Spontaneous Abortion Inevitable?

OBJECTIVE: To report on the characteristics of pregnancy in female patients with EEC (exstrophy-epispadias complex), determining in particular whether they are at higher risk of spontaneous abortion or complications. MATERIALS AND METHOD: Fifty patients diagnosed with EEC and treated in a reference center for this pathology were reviewed. Those with an incomplete medical history were excluded, leaving a total of 37 women with a median follow-up of 26 years (1-48 years). The outcome measurements were successful pregnancies, miscarriages, urological, gynecological and obstetric complications, impaired renal function, newborn characteristics, and postpartum urogynecological complications. Descriptive statistics was used. RESULTS: Eight patients achieved 17 pregnancies (88.2% spontaneous). Of these pregnancies, 10 (58.8%) were successful, while 7 (41.2%) terminated in miscarriages. Urinary tract infection (UTI) was the most frequent complication (41.6%) and intestinal occlusion was the most severe. A total of 62.5% of the patients presented genital prolapses after pregnancies. A total of 85.7% of patients were dry during the follow-up after their pregnancies. No newborn presented EEC or any other type of malformation. Our study has the limitation of being a retrospective review of a very heterogeneous and small group of patients. CONCLUSION: EEC patients can achieve spontaneous pregnancies but have an increased risk of miscarriage. For this reason, monitoring and control by a specialized and integrated multidisciplinary team is required to minimize complications.

Computer tomography findings in necrotizing otitis externa based on the offending pathogens.

PURPOSE: High-resolution temporal bone computer tomography (HRTBCT) is the most common initial radiological modality used for the assessment of necrotizing otitis externa (NOE). OBJECTIVES: (1) To compare the extension of disease, as seen on HRTBCT, in patients with NOE caused by different pathogens and (2) assess whether radiological findings may suggest the offending pathogen in cases of sterile-NOE. METHODS: All NOE patients were hospitalized between 1990 and 2018. All patients underwent HRTBCT at admission. Three groups (fungus-NOE, PA-NOE and sterile-NOE) comprising of ten patients each were randomly selected. HRTBCT was reevaluated by a senior radiologist. Thirteen radiological subsites were selected for reevaluation. RESULTS: All patients in the fungal-NOE group complained of otalgia, compared to nine in the sterile-NOE and six in the PA-NOE groups (p value = 0.044). External ear canal edema and granulation tissue were the most common findings in all groups. Surgery was performed in five patients in the fungal-NOE and PA-NOE and three in the sterile-NOE group (p value = > 0.05). Radiological findings indicating severe bone erosion within the EEC was seen in all patients but 3 (p value = > 0.05). Severe TMJ erosion was seen in one patient within the fungal-NOE and PA-NOE group (p value = > 0.05). When mild and severe involvement were combined, TMJ bone erosion was seen in four patients in the fungal-NOE and only in one patient in the PA-NOE (p = 0.04). CONCLUSIONS: TMJ involvement may be more common in fungal disease, suggesting a different spreading pathway, as opposed to PA-NOE. Accordingly, TMJ involvement on HRTBCT may justify antifungal treatment in sterile culture-NOE.

Case Report: Anti-LGI1 Encephalitis Following COVID-19 Vaccination.

Anti-leucine rich glioma inactivated 1 (LGI1) autoimmune encephalitis (AE) is characterized by cognitive impairment or rapid progressive dementia, psychiatric disorders, faciobrachial dystonic seizures (FBDS) and refractory hyponatremia. Since December 2020, millions of people worldwide have been vaccinated against COVID-19. Several soft neurological symptoms like pain, headache, dizziness, or muscle spasms are common and self-limited adverse effects after receiving the COVID-19 vaccine. However, several major neurological complications, despite the unproven causality, have been reported since the introduction of the COVID-19 vaccine. Herein, we describe a 48 years old man presenting with rapidly progressive cognitive decline and hyponatremia diagnosed with anti LGI1 AE, occurring shortly after the second dose of mRNA COVID -19 vaccine and possibly representing a severe adverse event related to the vaccination. Response to high dose steroid therapy was favorable. As millions of people worldwide are currently receiving COVID-19 vaccinations, this case should serve to increase the awareness for possible rare autoimmune reactions following this novel vaccination in general, and particularly of anti-LGI1 AE.

Incidental Detection of Radiotracer Uptake in Intracranial Dermoid Cyst on 18F-Prostate-Specific Membrane Antigen PET/CT During Staging for Prostate Carcinoma.

ABSTRACT: A 68-year-old man underwent 18F-prostate-specific membrane antigen (PSMA) PET/CT for staging of a newly diagnosed prostate adenocarcinoma. Unexpectedly, PET/CT revealed high focal 18F-PSMA brain uptake, which initially was suspected for a brain metastasis. Corresponding CT and MRI scans revealed characteristic imaging features of an intracranial dermoid cyst at this site. This is an exceptional location for a dermoid cyst, which had been followed up conservatively with no substantial changes. This case shows that dermoid cyst should be added to the reported list of benign neoplasms that shows "false-positive" PSMA uptake during evaluation of patients with prostate carcinoma, representing a potential interpretative pitfall.

Multiple Endocrine Deficiencies are Common in Hypoparathyroidism-Retardation-Dysmorphism Syndrome.

CONTEXT: The rare hypoparathyroidism-retardation-dysmorphism (HRD) syndrome (OMIM #241410) is caused by the mutated tubulin chaperone E (TBCE) gene. This gene encodes a critical protein in the microtubule assembly pathway. OBJECTIVE: To evaluate the endocrine profile of patients with HRD. METHODS: The study used a retrospective analysis of a large cohort of patients in a single university medical center. Sixty-three patients were diagnosed with HRD during 1990 to 2019; 58 of them had an endocrine evaluation. MAIN OUTCOME MEASURES: We investigated somatic growth parameters, the prevalence of hypoglycemia, growth hormone deficiency, hypothyroidism, hypogonadism, and cortisol deficiency. RESULTS: All patients were born small for gestational age, and severe growth retardation was found in all patients with mean height standard deviation score (SDS) of -8.8 (range: -5.1 to -15.1) and weight SDS -18 (range: -5.1 to -61.2). Serum insulin-like growth factor-1 concentrations were very low among the 21 studied patients: -2.32 SDS (range: -0.6 to -2.7). Four out of 14 (28%) investigated patients had growth hormone deficiency, and 55% of patients were hospitalized due to symptomatic hypoglycemia. Adrenal glucocorticoid insufficiency was diagnosed in 22% of those tested. Hypothyroidism was found in 36% of patients. Both hypogonadotrophic and hypergonadotrophic hypogonadism were observed. The main magnetic resonance imaging findings were small anterior pituitary gland, small hippocampus, brain atrophy, thin corpus callosum, Chiari type I malformation, and septo-optic dysplasia. CONCLUSION: Multiple endocrine abnormalities are common in patients with HRD syndrome. Periodic screening of thyroid and adrenal functions is recommended.

[High risk pregnancy in patients with extrophy-epispadia complex: Uro-gynecological characteristics and management.] / Embarazo de alto riesgo en pacientes con complejo extrofia-epispadias: características uro-ginecológicas y manejo.

OBJECTIVE: The exstrophy-epispadias complex (EEC) ranges from distal epispadias to cloacal exstrophy, with serious repercussions on the quality of life of patients. However, reconstructive surgery offers the opportunity to reach adulthood and consider motherhood.The objective of this work is to assess the uro-gynecological characteristics and the risks that pregnant women have with EEC. MATERIALS AND METHOD: Retrospective study of 50 patients diagnosed with EEC and treated in a reference center for this pathology, born between 1968 and 2000. Their medical records were reviewed and all demographic, pathological and gynecological data were collected. RESULTS: 37 patients have the inclusion criteria and of these 8 achieved 17 pregnancies (90% spontaneous and 10% through IVF). 10 were successful (50% at term) and 7 were abortions, 87.5% of which were in the first trimester. Urinary tract infection (UTI) was the most frequent complication (41.6%) and the most severe was intestinal occlusion. None of the patients presented renal function impairment during the gestation or dilation of the pathological upper urinary tract (UUT). 62.5% of the patients presented genital prolapses after pregnancies, 80% of which were grade III and IV. 87.5% were dry in the follow-up after their pregnancies. CONCLUSION: Pregnancy in the EEC patients is high risk and it is crucial that the follow-up is carried out by a specialized and integrated multidisciplinary team to minimize complications.

OBJETIVO: El complejo extrofia-epispadias (CEE) abarca desde las epispadias distales hasta la extrofia de cloaca, con serias repercusiones en la calidad de vida de los pacientes; sin embargo, la cirugía reconstructiva ofrece la oportunidad de llegar a la edad adulta y plantearse la maternidad. El objetivo de este trabajo es valorar las características uro-ginecológicas y los riesgos que presentan las gestantes con CEE. MATERIALES Y MÉTODO: Estudio retrospectivo de 50 pacientes diagnosticadas de CEE y tratadas en un centro de referencia para esta patología, nacidas entre 1968 y 2000. Se revisaron sus historias clínicas y se recogieron todos los datos demográficos, patológicos  y ginecológicos. RESULTADOS: 37 pacientes cumplían los criterios de inclusión y de estas 8 lograron 17 embarazos (90% espontáneos y 10% mediante FIV). 10 fueron exitosos (50% a término) y 7 fueron abortos, 87,5% de los cuales fueron en el primer trimestre. La infección urinaria (ITU) fue la complicación más frecuente (41,6%) y la más severa fue la oclusión intestinal. Ninguna de las pacientes presentó deterioro de la función renal durante la gestación o dilatación del tracto urinario superior (TUS) patológica. 62,5% de las pacientes presentaron prolapsos genitales posteriores a los embarazos, 80% de los cuales fueron grado III y IV. 87,5% se encontraban secas en el seguimiento posterior a sus embarazos. CONCLUSIÓN: El embarazo en el CEE es de alto riesgo y es crucial que el seguimiento sea llevado a cabo por un equipo multidisciplinar especializado e integrado para minimizar las complicaciones.

Association of cerebral activity with MRI scans in infants with neonatal encephalopathy undergoing therapeutic hypothermia.

We aimed to correlate amplitude-integrated EEG (aEEG) in neonatal hypoxic ischemic encephalopathy (HIE) with early magnetic resonance imaging (MRI). In this retrospective study, 32 neonates over 35 weeks' gestation with moderate/severe HIE who were treated with hypothermia were included. Early MRI scans and daily aEEG background were categorized to mild/normal, moderate, and severely abnormal. Time to sleep cycling was noted on aEEG. Mantel-Haenszel test for trends was used to explore associations between aEEG and MRI and outcome. LOESS regression was used for exploring the association of cycling with MRI scores. MRI was normal/mildly abnormal in 20 (63%) infants; in 9 (28%), moderately abnormal; and in 3 (9%), severely abnormal. Twenty-seven (84%) infants s urvived. MRI severity score was significantly associated with aEEG background score on the third and fourth days of life (p < 0.01). An increase in the MRI severity score was noted if sleep cycling appeared after the fifth day of life.Conclusions: Depressed aEEG at the third and fourth days of life and appearance of cycling beyond the fifth day of life are associated with cerebral MRI abnormalities and may be associated with increased risk of abnormal outcome. What is known: • Since therapeutic hypothermia has been shown to change long-term outcome, amplitude-integrated EEG in infants with hypoxic ischemic neonatal encephalopathy soon after birth have a limited predictive power for long-term outcome in treated infants. • Brain MRI after therapeutic hypothermia in the above infants has a significant predictive value for long-term outcome What is new: • Background amplitude-integrated EEG activity depression at the age of 3 and 4 days and delay of appearance of cycling activity are associated with worse MRI scores and may be predictive of worse long-term outcome.

Creating normograms of dural sinuses in healthy persons using computer-assisted detection for analysis and comparison of cross-section dural sinuses in the brain.

Dural sinuses vary in size and shape in many pathological conditions with abnormal intracranial pressure. Size and shape normograms of dural brain sinuses are not available. The creation of such normograms may enable computer-assisted comparison to pathologic exams and facilitate diagnoses. The purpose of this study was to quantitatively evaluate normal magnetic resonance venography (MRV) studies in order to create normograms of dural sinuses using a computerized algorithm for vessel cross-sectional analysis. This was a retrospective analysis of MRV studies of 30 healthy persons. Data were analyzed using a specially developed Matlab algorithm for vessel cross-sectional analysis. The cross-sectional area and shape measurements were evaluated to create normograms. Mean cross-sectional size was 53.27±13.31 for the right transverse sinus (TS), 46.87+12.57 for the left TS (p=0.089) and 36.65+12.38 for the superior sagittal sinus. Normograms were created. The distribution of cross-sectional areas along the vessels showed distinct patterns and a parallel course for the median, 25th, 50th and 75th percentiles. In conclusion, using a novel computerized method for vessel cross-sectional analysis we were able to quantitatively characterize dural sinuses of healthy persons and create normograms.

Predictive analysis of factors associated with percutaneous stone surgery outcomes.

INTRODUCTION: The aim of this study is to identify surgical, patient- and stone-related factors predictive of clinical success and complications after percutaneous nephrolithotomy (PCNL). MATERIALS AND METHODS: We prospectively studied 100 consecutive PCNL procedures. Univariate and multiple regression models were used in order to identify which variables could act as independent predictors of PCNL outcomes. Success was defined as complete absence of fragments in a non-contrast CT. The Clavien-modified grading system was used to classify the complications. RESULTS: Univariate analysis showed that patients rendered stone-free had a significantly lower stone burden, shorter operating times, single stones and non-struvite composed calculi. Patient age, nephrostomy tract dilation with high pressure balloon and a stone composition different to struvite behaved as significant protective factors for complications. Logistic regression models revealed that the main independent prognostic factor for success was stone surface (OR = 0.997 per mm2, p = 0.000), followed by multiple stones (OR = 0.203, p = 0.050). On the other hand, struvite composition (OR = 5.911, p = 0.028) was an independent predictor for the development of complications, whilst age (OR = 0.936, p = 0.012) and high pressure balloon dilation (OR = 0.041, p = 0.007) were rendered independent protective variables. CONCLUSIONS: Stone burden and multiple calculi in the kidney affect the immediate stone-free rate, whilst Amplatz dilation, struvite stones and young patients lead to a higher incidence of postoperative complications. This information can be very useful for patient counseling, regarding percutaneous kidney stone management.

Epiglottic abscess as a complication of acute epiglottitis.

OBJECTIVE: An epiglottic abscess is considered a life-threatening medical situation that can cause death by obstruction the upper airways. We describe a 58-year-old man who presented to our hospital with sore throat, dysphagia and dysphonia. MATERIALS AND METHODS: A fiberoptic laryngoscope (FOL) demonstrated beefy red edematous epiglottis with edema extending from the base of the tongue to the aryepiglottic folds and arytenoids. CT scan showed multiple air bubbles inside the swollen epiglottis, in keeping with the diagnosis of necrotizing epiglottic abscess. RESULTS: Under local anesthesia we performed puncture of the abscess at the tip of the epiglottis. He was dismissed 5days from his admission to the hospital after an improvement was noticed in his epiglottis. CONCLUSION: Treatment consists of airway management if needed under anesthesia and draining of the abscess. An IV antibiotics plus corticosteroids should be administrated the moment a suspicion of epiglottitis is present.

Recurrent urinary retention due to imperforate hymen after hymenotomy failure: a rare case report and review of the literature.

Acute urinary retention (AUR) is unusual in children. We report for the first time a case of recurrent urinary retention due to massive hematocolpos resulting from an imperforate hymen in a 14-year-old girl. In case of AUR in adolescent girls, clinicians should keep in mind that imperforate hymen may be a causative factor and this condition may easily be treated surgically, but follow-up is still necessary to ensure that there is no recurrence.