Israel Medical Association Journal

Background: Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine skin tumor with an increasing incidence in Western countries. Predominantly affecting older individuals, MCC represents less than 1% of malignant skin tumors.

Objectives: To characterize the clinical presentation, therapeutic interventions, and follow-up outcomes of MCC patients. To promote heightened clinical awareness regarding the early recognition and diagnosis of MCC.

Methods: We conducted a retrospective cohort study analyzing medical records of MCC patients at the Shaare Zedek Medical Center between 2015–2022. From 19 initially identified patients, 17 met the inclusion criteria. Data collection included demographic, epidemiological, clinical, and pathological characteristics.

Results: The study included 17 patients, predominantly of Jewish origin, with a mean age of 70.06 years; 58.8% female. Medical co-morbidities included 64.7% hypertension and 35.3% diabetes. MCC tumors were predominantly left-sided (58.8%), with varied locations including limbs, trunk, and face. Surgical treatment consisted of excision and primary closure (64.7%) or skin grafting (23.5%). The average tumor diameter was 3.41 cm clinically and 3.83 cm pathologically. Lymph node involvement occurred in 29.4% of cases; 23.5% showed metastatic disease at diagnosis, with metastases diffused in different body areas. Kaplan-Meier survival analysis showed no statistically significant differences across most variables, except for a significantly lower survival rate in patients with ischemic heart disease (P = 0.009).

Conclusions: Our study reveals unique characteristics of MCC, predominance of female patients, and a slightly younger average diagnosis age compared to existing literature. The 2-year survival rate in our cohort was 82%. The study underscores the importance of early detection and diagnosis of MCC, thereby enhancing clinical awareness and improving patient outcomes.

Background: Mycosis fungoides (MF) combined with photosensitive/autoimmune diseases has been reported, yet there are limited data regarding the therapeutic considerations in these patients, specifically phototherapy, a mainstay skin-directed treatment (SDT), being a relative or complete contra-indication.

Objectives: To outline therapeutic considerations for patients with MF who had also been diagnosed with photosensitive/autoimmune diseases.

Methods: We conducted a retrospective analysis of patients with MF who were treated at our center between January 2008 and December 2024with photosensitive/autoimmune diseases, especially collagen vascular diseases (CVD) or autoimmune bullous diseases (AIBD),

Results: Eight patients were diagnosed with MF at a median age of 39 years. Seven had early-stage (4-IA, 3-IB) and one had Sézary syndrome. Six early-stage MF patients were diagnosed with lupus erythematosus (LE, 4) or AIBD (2) and were treated with SDT (topical corticosteroids/chlormethine gel), systemic retinoid or methotrexate. A patient with resistant early-stage MF and discoid LE was treated with electron beam and interferon. One patient who presented with variegate porphyria and localized MF was treated with electron beam. The patient with Sézary syndrome had inclusion body myositis. He was treated with low-dose total skin electron beam, methotrexate, extracorporeal photopheresis, and subsequently with romidepsin. After a median of 8 years, no stage progression of MF was observed. The Sézary syndrome patient achieved down-staging and was at stage IB. There was no aggravation of the co-morbidity in any of the patients.

Conclusions: Effective management of MF and associated photosensitive or autoimmune co-morbidities underscore the need for individualized treatment strategies in patients with these unique dual diagnoses.

Background: Acute cholecystitis (ACC) is one of the most common diagnoses encountered in surgical wards. A number of treatment modalities are available, and various guidelines have been developed to help decision making. Many factors influence treatment strategies, including patient age and frailty. Due to the increasing proportion of older patients, consideration into the best treatment modalities for this population are warranted.

Objectives: To determine outcomes of elderly patients with ACC according to different treatment strategies.

Methods: A retrospective analysis of consecutive patients aged ≥ 80 years who were admitted with a diagnosis of ACC between 2015 and 2019 to a single academic center. Patients were divided into three groups according to treatment: percutaneous cholecystostomy tube placement, cholecystectomy, intravenous antibiotic treatment only.

Results: Overall, 466 patients were included in the cohort. The majority (approximately 75%) were treated with antibiotics only, 17% underwent percutaneous cholecystostomy, and 8% underwent laparoscopic cholecystectomy. One-year mortality was 28.1%. The highest mortality rate was 41.6% in the cholecystostomy group (P = 0.002). In multivariable analysis age, functional status, C-reactive protein, and albumin levels were found to be independent risk factors for 1-year mortality (hazard ratio [HR] 1.08, 0.56, 0.98, 0.4, respectively). Cholecystostomy increased risk of one-year mortality compared to cholecystectomy and antibiotics alone (HR 0.61, 0.23, respectively).

Conclusions: The use of cholecystostomy for ACC in older adults is an independent risk factor for 1-year mortality. Its use in older adults should be reserved for carefully selected cases.

Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting from the accumulation of misfolded protein aggregates in the myocardial extracellular space. Cardiac amyloidosis stems primarily from the misfolding of monoclonal immunoglobulin light chains (AL) originating from abnormal clonal plasma cell proliferation or transthyretin amyloidosis (ATTR).

Diagnosis of amyloidosis demands a high index of suspicion and requires histological confirmation of pathognomonic green birefringence under polarized light when an infiltrated tissue specimen is stained with Congo-red staining [1,2].

Pleural involvement of systemic amyloidosis has rarely been reported and is considered a serious complication [3]. Pleural amyloidosis is diagnosed with the identification of amyloid deposits in pleural biopsy specimens. However, pleural biopsies are rarely performed for this indication.

We describe the case of a patient with AL cardiac amyloidosis presenting as intractable pleural effusion and diagnosed via pleural biopsy.

Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare autoimmune demyelinating disorder of the central nervous system. Optic neuritis (ON) is the most common clinical manifestation of MOGAD in adults. In 2023, new MOGAD diagnostic criteria were proposed, highlighting the importance of supplemental criteria when MOG-immunoglobulin G (IgG) titers are unavailable.

Objective: To investigate the applicability of the 2023 MOGAD criteria in patients diagnosed with MOGAD and treated before the availability of MOG-IgG titers.

Methods: We conducted a retrospective chart review of patients classified as MOGAD between 2010 and 2023 at Rabin Medical Center. Patient demographics as well as clinical and imaging data were collected, including visual acuity, expanded disability status score, core demyelinating events, antibody status, and brain and optic nerve magnetic resonance imaging data. Patients fulfilling the 2023 MOGAD criteria were reported as definite MOGAD.

Results: Fifteen patients met the 2023 MOGAD diagnostic criteria despite lack of MOG-IgG titer. The most common supplemental criterion meeting the 2023 MOGAD criteria was optic disc edema (n=12, 80%), followed by longitudinal optic nerve involvement (53%), bilateral ON (40%), and perineural optic sheath enhancement (33%).

Conclusions: All patients with a clinical diagnosis of MOG-ON in our cohort fulfilled the 2023 MOGAD criteria despite the lack of antibody titers. The 2023 MOGAD criteria can be reliably applied to Israeli cohorts, prior to availability of MOGAD IgG titers, with particular attention to additional supplemental criteria. Since the 2023 MOGAD criteria were published, MOGAD IgG titers have been added to routine testing at our facility.

Background: Blood pressure (BP) is routinely measured while triaging children presenting to the pediatric emergency department (PED).

Objectives: To determine whether a medical clown shortens the time to acquire a BP measurement among children undergoing triage in the PED.

Methods: The study comprised 133 children. Patients were assigned to one of two groups: with a medical clown or without a medical clown.

Results: The presence of a medical clown led to a significantly shorter time to acquire a blood pressure measurement (60 ± 23 seconds vs. 81 ± 43.5 seconds, P < 0.001. Clowns had a significant effect on shortening total triage length among children of Jewish ethnicity compared to Arab ethnicity (113 ± 353.6 seconds vs. 154 ± 418 seconds, P = 0.012).

Conclusions: Using medical clowns while measuring BP during triage when used in a culturally appropriate manner shortens time.

Background: The current approach to performing sacral neuromodulation consists of a two-stage procedure, the first of which includes insertion of the sacral electrode under fluoroscopic visualization of the S3 foramen. Alternatively, in certain situations computed tomography (CT)-guided insertion can be used.

Objectives: To evaluate the use of CT in cases of reinsertion of the electrode due to infection, dislocation, or rupture.

Methods: Medical records of patients who underwent neuromodulation device reinsertion between 2005 and 2016 for fecal incontinence were reviewed. Study outcomes included procedure course, successful placement, and long-term treatment success.

Results: During the study period, we inserted a neuromodulation device in 67 patients. A CT-guided insertion of a sacral electrode was performed in 10 patients. In nine patients, the insertion and the final location of the electrode were successful. In one patient, the electrode migrated upward due to a malformation of the S3 foramen on both sides and had to be placed in S4. In a mean follow-up of 68.4 ± 30.0 months following the re-insertion, there was a significant reduction in the number of incontinence episodes per day (P < 0.001) and the number of pads used per day (P = 0.002).

Conclusions: CT-guided insertion of a sacral electrode is a safe and promising option, especially in recurrent and or selected cases

Background: Adherence to treatment by adolescents and adults with cystic fibrosis (CF) is often poor.

Objectives: To assess the impact of a focused clinical intervention on adherence in individual patients, including help in problem-solving key barriers to adherence. To implement a patient-centered problem-solving intervention using CF My Way tools. To identify and overcome a selected barrier to adherence.

Methods: Medication possession ratios (MPRs), number of airway clearance sessions, forced expiratory volume (FEV₁), body mass index (BMI), and health-related quality of life (HRQoL) were measured before and after the intervention.

Results: Sixteen patients with CF, aged 23.4 ± 6.7 years, participated. MPR increased for colistimethate sodium and tobramycin inhalations from a median of 21 (range 0–100) to 56 (range 0–100), P = 0.04 and 20 (range 0–100) to 33.3 (range 25–100), P = 0.03, respectively. BMI standard deviation score rose from -0.37 to -0.21, P = 0.05. No significant improvements were found in FEV₁, airway clearance, or HRQoL scores.

Conclusions: The CF My Way problem-solving intervention increased adherence to medical treatments by removing barriers directly related to the needs and goals of young adults with CF

Background: The sonographic assessment of estimated fetal weight (EFW) is essential for identification of fetuses in weight extremes and aids in peripartum management. However, there are inconsistent reports regarding EFW accuracy.

Objective: To examine maternal and fetal determinants associated with unreliable EFW.

Methods: A retrospective case-control study was conducted at a single, tertiary medical center between 2011 and 2019. All term, singleton deliveries with a sonographic EFW within 2 weeks of delivery were included. Unreliable EFW was defined as > 500 grams discordance between it and the actual birth weight. We allocated the study cohort into two groups: unreliable EFW (cases) and accurate EFW (controls).

Results: Overall, 41,261 deliveries met inclusion criteria. Of these, 1721 (4.17%) had unreliable EFW. The factors positively associated with unreliable EFW included body mass index > 30 kg/m², weight gain > 20 kg, higher amniotic fluid index, pregestational diabetes, gestational age > 41^0/7, and birth weight ≥ 4000 grams. On multiple regression analysis, pregestational diabetes (odds ratio [OR] 2.22, 95% confidence interval [95%CI] 1.56–3.17, P < 0.001) and a higher birth weight (OR 1.91, 95%CI 1.79–2.04, P < 0.001) were independently associated with unreliable EFW. On analysis of different weight categories, pregestational diabetes was associated with unreliable EFW only among birth weights ≥ 3500 grams (OR 3.28, 95%CI 1.98–5.44, P< 0.001) and ≥ 4000 grams (OR 4.27, 95%CI 2.31–7.90, P < 0.001).

Conclusion: Pregestational diabetes and increased birth weight are independent risk factors for unreliable EFW and should be considered when planning delivery management.

Background: The frequency of increased high-sensitivity C-reactive protein (hs-CRP) and the time course of evolution of their levels in patients with acute idiopathic pericarditis (AIP) are not well established.

Objective: To assess the time course of evolution of hs-CRP levels and the possible clinical significance of maximal hs-CRP levels in patients with AIP

Methods: We retrospectively reviewed the medical files of 241 patients admitted to the hospital with a diagnosis of AIP between March 2006 and March 2017. Data on demographics, time of symptom onset, laboratory and imaging findings, and outcome were collected.

Results: Data on serum hs-CRP levels were available for 225 patients (age 18–89 years, 181 men). Fever, pleural effusion, and age were independently associated with hs-CRP levels. Major cardiac complications (MCC) (death, cardiac tamponade, cardiogenic shock, large pericardial effusion, ventricular tachycardia, pericardiocentesis, or pericardiectomy) were more common in patients with hs-CRP levels above the median compared to those below (21.2% vs. 4.5%, respectively, P < 0.001). Hs-CRP levels were independently associated with MCC (odds ratio [OR] 1.071, 95% confidence interval [95%CI] 1.016–1.130, P = 0.011). Hs-CRP levels were elevated in 76.0%, 92.3% and 96.0% of the patients tested <6 hours, 7-12 hours, and >12 hours of symptom onset, respectively (P = 0.003). The frequency of elevated hs-CRP among patients tested > 24 hours was 98.1%.

Conclusions: Hs-CRP levels rise rapidly among patients with AIP. Maximal hs-CRP levels are associated with MCC. A normal hs-CRP level is rare among patients tested > 24 hours of symptom onset.

Background: Hysterectomy is common in the management of symptomatic uterine prolapse. Vaginal wall repair is often necessary, for which vaginal mesh remains a popular option.

Objectives: To evaluate the risk of mesh erosion following mesh-augmented vaginal prolapse repair, with or without concomitant vaginal hysterectomy.

Methods: This retrospective cohort comprised 70 women who underwent vaginal mesh-augmented pelvic organ prolapse repair from 2007 to 2010. Of the participants, 36 (51.4%) had a vaginal hysterectomy concomitant to the anterior and/or posterior vaginal mesh repair (hysterectomy group) and 34 (48.6%) underwent mesh repair without vaginal hysterectomy (no hysterectomy group).

Results: There were no inter-group differences in age, parity, menopausal state, hormonal use, or presenting symptoms. Previous prolapse repair surgery was much more common in the no hysterectomy group (29.4% vs. 5.5%, P = 0.01). Eleven patients (32.3%) in the no hysterectomy group had previously undergone hysterectomy. Anterior mesh repair was performed in 77.7% and 67.6% of hysterectomy and no hysterectomy patients, respectively. Posterior mesh repair was performed in 27.7% and 44.1%, respectively. One patient in the hysterectomy group underwent immediate removal of mesh due to infection. Surgically treated mesh erosion (limited local excision) occurred in three patients (8.3%) in the hysterectomy group (3, 16, and 18 months following surgery) and in two patients (5.8%) in the no hysterectomy group at 6 months following surgery (P = 0.67).

Conclusions: Vaginal mesh-augmentation concomitant with vaginal hysterectomy for pelvic organ prolapse repair does not carry an increased risk of erosion.

Background: When a woman with an endometrioma presents with acute abdominal pain, it is unclear whether ovarian torsion should be suspected.

Objectives: To compare patient characteristics, imaging results, and surgical management of endometriomas in elective versus emergent surgeries.

Methods: This retrospective cohort study included women treated at our institution during the period 1990–2015 who presented with histologically verified endometrioma and who underwent either planned surgery or emergent surgery due to suspected adnexal torsion.

Results: Of 225 surgeries performed, 174 were elective and 51 emergent. Patients in the emergent group were significantly younger (33.9 ± 11.1 vs. 39.01 ± 10.9 years, P = 0.004). Abdominal pain was the main complaint of all the emergent surgery patients and the leading complaint in 21% of the elective surgery patients (P < 0.001), with right-sided predominance in both groups. Sonographic parameters were similar in both groups. Bilateral ovarian cysts were noted in 11.7% and 11.0% of emergent and elective patients, respectively (P = 0.87). Laboratory evaluation was notable for a higher white blood cell count and CA125 levels among emergent patients. All patients in the emergent group and 93% of patients in the elective group were managed laparoscopically. No cases of torsion were noted. The rate of intra-pelvic adhesions was similar in both groups (56.8% vs. 66.6%, P = 0.19).

Conclusions: Endometrioma may present with acute abdominal pain. However, adnexal torsion in these patients is rare. These cases can be managed using a minimally invasive approach, assuming an optimal surgical setting.

Background: Different clinical and sonographic parameters have been suggested to identify patients with retained products of conception. In suspected cases, the main treatment is hysteroscopic removal.

Objectives: To compare clinical, sonographic, and intraoperative findings in cases of hysteroscopy for retained products of conception, according to histology.

Methods: The results of operative hysteroscopies that were conducted between 2011 and 2016 for suspected retained products of conception were evaluated. Material was obtained and evaluated histologically. The positive histology group (n=178) included cases with confirmed trophoblastic material. The negative histology group (n=26) included cases with non-trophoblastic material.

Results: Patient demographics were similar in the groups, and both underwent operative hysteroscopy an average of 7 to 8 weeks after delivery/abortion. A history of vaginal delivery was more common among the positive histology group. The main presenting symptom in all study patients was vaginal bleeding, and the majority of cases were diagnosed at their routine postpartum/abortion follow-up visit. Sonographic parameters were similar in the groups. Intraoperatively, the performing surgeon was significantly more likely to identify true trophoblastic tissue as such than to correctly identify non-trophoblastic tissue (P < 0.001).

Conclusions: Suspected retained trophoblastic material cannot be accurately differentiated from non-trophoblastic material according to clinical, sonographic, and intraprocedural criteria. Thus, hysteroscopy seems warranted in suspected cases.