Israel Medical Association Journal

Background: Fetal facial clefts are among the most common congenital anomalies detected prenatally. This finding may lead to termination of pregnancy in some cases.

Objectives: To compare a cohort of fetuses with facial clefts in which the pregnancy was terminated to the cohort of cases that were born with facial clefts. To investigate risk factors for facial clefts.

Methods: We conducted a retrospective chart review of all women with prenatal and postnatal diagnosis of facial cleft that were managed in our institute. A telephone questionnaire was conducted regarding a positive family history and/or genetic predisposition for facial clefts abnormalities.

Results: The final cohort consisted of two group. One group included 54 cases of termination of pregnancy (TOP) that were performed due to cleft lip (CL) or cleft palate (CLP); 27 women answered the telephone questionnaire. The second group comprised 99 women who delivered children with facial cleft during the same period; 60 answered the questionnaire. Only seven cases were diagnosed prenatal. Among the two groups, no correlation to family history was discovered. Of note, there was one case of three consecutive fetuses with CL in one woman, without any significant genetic findings.

Conclusions: To the best of our knowledge, this is the first study to describe an anatomical malformation posing an ethical dilemma before TOP. Primary prevention with folic acid and early sonographic detection of CL/CLP with multidisciplinary consultation should be considered.

Background: The administration of antenatal corticosteroids (ACS) is standard practice for management of threatened preterm birth. Its benefit, especially in small for gestational age (SGA) late preterm, is unclear.

Objectives: To evaluate the impact of ACS on perinatal outcome of late preterm SGA neonates.

Methods: We conducted a retrospective cohort study of all women carrying a singleton gestation who had late preterm delivery (34–36 gestational weeks) of SGA neonates at a single tertiary university-affiliated medical center (July 2012–December 2017). Exclusion criteria included termination of pregnancy, intrauterine fetal death, and birth weight ≥ 10th percentile. Outcomes were compared between ACS and non-ACS treatment prior to delivery. Neonatal composite outcome included neonatal intensive care unit (NICU) admission, respiratory distress syndrome, mechanical ventilation, and transient tachypnea.

Results: Overall, 228 women met inclusion criteria; 102 (44.7%) received ACS and 126 did not (55.3%). Median birth weight among the non-ACS group was significantly higher (1896.0 vs. 1755.5 grams P < 0.001). Rates of NICU and jaundice requiring phototherapy were higher among the ACS group (53.92% vs. 31.74%, P = 0.01; 12.74% vs. 5.55%, P = 0.05, respectively). Composite neonatal outcome was significantly higher among the ACS group (53.92% vs. 32.53%, odds ratio [OR] 2.42, 95% confidence interval [95%CI] 1.41–4.15, P = 0.01). After adjustment for potential confounders, this association remained significant (OR 2.15, 95%CI 1.23–3.78, P = 0.007).

Conclusions: ACS given during pregnancy did not improve respiratory outcome for SGA late preterm neonates. ACS may be associated with a worse outcome.

Rheumatoid arthritis (RA) is an autoimmune chronic inflammatory disease characterized by synovitis leading to polyarthritis. It affects 1% of the population [1]. Genetic and environmental factors are linked to the development of RA and include the presence of HLA-DR4 and shared epitope, and smoking is the primary representative of the negative environmental factor [1].

However, RA mainly affects middle age. Late-onset RA that initiates after 60 years is sometimes named elderly onset rheumatoid arthritis (EORA) [2]. This disease's prevalence varied from 2.03% to 2.34% in a large study in the United States. EORA affects more women than men [1]. However, to the best of our knowledge, no patient description of RA initiated at 97 years of age has been described.

Klippel-Trenaunay syndrome (KTS) is a rare congenital complex vascular multisystem disorder characterized by bony and soft-tissue hypertrophy. It is famous for its hallmarks like port-wine stains and varicose veins. The syndrome is sporadic, although rare familial cases have been reported [1]. The most common ophthalmological alterations encountered in KTS are conjunctival telangiectasia, anterior chamber malformation, raised episcleral venous pressure with associated glaucoma, and choroidal hemangiomas [2].

The purpose of this report is to raise awareness of KTS and its diverse scale of expressions as well as complications. This study was conducted in accordance with the ethical standards set by the Declaration of Helsinki. The patient gave signed informed consent.

Psoriatic arthritis can present with significant hand and wrist deformity and dysfunction [1]. The development of newer biological therapies has resulted in higher rates of remission [2]. However, surgical intervention is still indicated in pain, disability, and severe deformation cases. The management of patients with rheumatic diseases has a controversial history, characterized by rheumatologists and hand surgeons debating the efficacy of surgical interventions. Some surgeons attribute the controversial results to “too little and too late” referral of patients from rheumatologists [3]. While the availability of new and more effective medication has changed the indications and postponed surgical intervention, it is important to remember that surgery is often more effective when used preventively in the early stages than when forced to salvage. In the following case, we present a patient with psoriatic arthritis who presented with advanced-stage debilitating hand deformity and was treated surgically.

Patients with severe ischemic and hemorrhagic stroke may require invasive mechanical ventilation due to loss of consciousness and increased risk for aspiration pneumonia secondary to new onset dysphagia. Ventilation may also confer airway protection until the patient stabilizes [1]. Mechanically intubated stroke patients who are admitted to the intensive care unit (ICU) have a poor prognosis and a 40–80 % mortality rate [2]. Proceeding to tracheostomy is mandatory in stroke patients to ease the procedure of respiratory weaning and extubation [1]. In the stroke ICU, between 15% and 35% of the mechanically intubated patients cannot proceed to tracheostomy due to weaning and extubation failure [3].

Recent studies using propensity score matching have clearly indicated that contrast nephropathy following computed tomography occurs in hospitalized patients with advanced chronic kidney disease (eGFR < 30 ml/min/1.73 m²) and that this iatrogenic complication is likely underestimated because of concomitant renal functional recovery, unrelated to the imaging procedure. These findings should be considered regarding contrast-enhanced studies in such patients.

Background: There is an unmet need for real-world data regarding laboratory results, co-morbidities, and medication use prior to the first symptoms of amyotrophic lateral sclerosis (ALS). Researchers must identify specific subpopulations at risk for developing ALS and understand pathogenic mechanisms preceding the clinical presentation of ALS as well as possible subclinical disease manifestations.

Objectives: To valuate the role of laboratory results, co-morbidities, and medication use prior to the first symptoms of patients with ALS in Israel so that specific subpopulations at risk for developing ALS can be identified and for possible subclinical disease manifestations. To understand pathogenic mechanisms preceding the clinical presentation of ALS.

Methods: At the ALS clinic at Tel Aviv Sourasky Medical Center, 259 ALS patients insured by Maccabi Healthcare Services and seen between January 1998 and December 2017 were included. Comparisons of demographics, co-morbidities, medications taken, history of trauma, and laboratory tests prior to disease onset were performed between patients and 1295 matched controls.

Results: Prior to disease presentation, ALS patients had a higher frequency of hypertension and cardiovascular disease; presented more frequently with trauma and viral infections; more frequently used analgesics, non-steroidal anti-inflammatory drugs, narcotics, antibiotics, and antiviral medications; and had higher creatine kinase levels.

Conclusions: ALS patients showed higher frequency of cardiovascular disease prior to diagnosis, as well as higher frequency of trauma, infections, and pain medication usage.

Adenoid surgery (adenoidectomy) is one of the most common pediatric surgical procedures. Complications of this surgery include anesthetic issues, bleeding, pain, dysphagia, and velopharyngeal insufficiency. The intraoperative complications are usually the most urgent and therefore require prompt identification and resolution. Tension pneumothorax (TPX) is a rare intraoperative. We present the first case in the English literature, to the best of our knowledge, of TPX during adenoid/tonsil surgery.

Migraine is very common headache disorder, usually starting at a young age with a higher prevalence in females. In this narrative review, we summarize the prevalence, burden, and socio-demographic characteristics of migraineurs worldwide and specifically in Israel. We searched standard electronic bibliographic databases, including MEDLINE (via PubMed), Embase, Google Scholar, and Up to Date using combinations of search terms and reviewed the articles we found. The factors reviewed included prevalence and incidence, burden, sex differences, age distribution, cognitive function, socioeconomic status, religion, country of origin, and urban vs. rural dwelling.

In the position statement on the definition and diagnosis of acute myocarditis on page XXX of this issue of the Israel Medical Association Journal (IMAJ), we discussed contemporary criteria for definition of acute myocarditis and inflammatory cardiomyopathy [1-6]. We also addressed current diagnostic methods including indications for endomyocardial biopsy (EMB) [7-21]. In this position statement, we discuss the management approaches during hospitalization and following hospital discharge, including specific forms of myocarditis and recommendations for returning to physical activity after myocarditis [21-36].

Background: Signet ring cell carcinoma (SRCC) is classified as an undifferentiated gastric carcinoma with poor prognosis. Early SRCCs are associated with improved prognosis.

Objectives: To describe the outcomes of incidental SRCC.

Methods: In this case series, 900 medical charts of patients with SRCC were screened to identify patients with incidental SRCC, defined as diagnosed in random, non-focal-lesion-targeted biopsies.

Results: Six patients were diagnosed with incidental SRCC and underwent gastrectomy. The final pathology of five patients revealed one or more small foci of early SRCC without lymphovascular invasion. Only one patient had no evidence of malignancy. The median follow-up after surgery was 4.2 years (50 months, range 37–90 months). No deaths or recurrences were recorded during the follow-up period. These results resemble the reported survival rate for early SRCC.

Conclusions: An aggressive surgical approach in incidental gastric SRCC patients is recommended, as they have a chance for long-term survival.

Background: Among the most frequent complications following transcatheter aortic valve replacement (TAVR) is hemostasis imbalance that presents either as thromboembolic or bleeding. Deviations in platelet count (PC) and mean platelet volume (MPV) are markers of hemostasis imbalance.

Objectives: To determine the predictive value of pre- and post-procedural PC and MPV fL 1-year all-cause mortality in patients who underwent TAVR.

Methods: In this population-based study, we included 236 TAVR patients treated at the Tzafon Medical Center between 1 June 2015 and 31 August 2018. Routine blood samples for serum PC levels and MPV fL were taken just before the TAVR and 24-hour post-TAVR. We used backward regression models to evaluate the predictive value of PC and MPV in all-cause mortality in TAVR patients.

Results: In this study cohort, MPV levels 24-hour post-TAVR that were greater than the cohort median of 9 fL (interquartile range 8.5–9.8) were the strongest predictor of 1-year mortality (hazard ratio 1.343, 95% confidence interval 1.059–1.703, P-value 0.015). A statistically significant relationship was seen in the unadjusted regression model as well as after the adjustment for clinical variables.

Conclusions: Serum MPV levels fL 24-hour post-procedure were found to be meaningful markers in predicting 1-year all-cause mortality in patients after TAVR.

Background: Nasal obstruction is one of the most common complaints in the practice of rhinology.

Objective: To adapt the Nasal Obstruction Scale Evaluation (NOSE) questionnaire to Hebrew (H-NOSE) and to assess its sensitivity and specificity.

Methods: Candidates for surgical intervention due to isolated nasal obstruction and healthy volunteers (controls) were included in the validation. The English NOSE questionnaire was translated into Hebrew and re-translated for translation validity. Patients completed the H-NOSE questionnaire before and after surgery for nasal obstruction. The same questionnaire was completed by the controls. Test–retest reliability was performed within 2 weeks. Psychometric properties (reliability, reproducibility, validity, and responsiveness) were assessed by a test–retest procedure, internal consistency, correlation to the Hebrew Sino-Nasal Outcome Tool 22 (He-SNOT-22), and response sensitivity.

Results: In total, 179 patients with nasal obstruction and 74 controls completed the questionnaire. Cronbach's alpha score was 0.93 for internal consistency. The receiver operating characteristic curve demonstrated high sensitivity and specificity (< 90%) and area under the curve was 0.97. We found no significant difference in test–retest reliability. The difference between the pre- and postoperative questionnaire scores was highly significant (13.9 ± 4.0 vs. 3.2 ± 4.1, respectively, P < 0.001).

Conclusions: The H-NOSE questionnaire demonstrated reliable internal consistency, sensitivity, specificity, and reliability. The Hebrew version differentiated between patients and heathy controls and was easy to administer. This instrument is useful for Hebrew speaking patients who undergo surgery for nasal obstruction.

Background: While several studies have noted smell impairment in schizophrenia, it is unclear whether this impairment extends to acute psychosis and whether it is associated with more severe illness as expressed in extended hospitalization.

Objectives: To evaluate the olfactory function of patients in an acute psychotic state and correlate it with clinical symptomatology and length of hospitalization.

Methods: Olfactory function was assessed in 20 patients with schizophrenia in their first week of hospital admission for acute psychosis compared with matched controls. Olfaction was evaluated via three stages: threshold, discrimination, and identification of different odors utilizing the Sniffin' Sticks test battery.

Results: Schizophrenia patients scored significantly lower on total smell score, discrimination, and identification abilities. A significant association was observed between hospitalization duration and total smell score and smell discrimination. No significant associations between smell and clinical symptomatology were observed.

Conclusions: Study observations confirm impaired sense of smell in schizophrenia patients and suggest that smell impairment may be a potential marker of more serious illness as expressed in longer hospital stay.