Israel Medical Association Journal

Background: neonatal cardiac surgery has evolved over the last 50 years with a large percentage of the patients achieving complete physiological repair in the neonatal period. The remaining patients achieve staged palliation with an increasing amount of success. 

Objectives: To report our experience with 1000 neonatal cardiac surgical procedures performed in the last 10 years.

Methods: We conducted a retrospective analysis of surgical outcome in all neonatal patients who underwent cardiac surgery between January 2007 and July 2016 at Schneider Children's Medical Center of Israel.

Results: A total of 1003 neonates aged < 30 days underwent surgery for congenital heart defects at our center. Neonatal surgery accounted for 22.5% of all cardiac surgeries. Neonatal operative mortality was 7.3%, Operative mortality for individual lesions were: simple aortic coarctation (CoA) (198 patients, 2.5%), CoA with hypoplastic arch (24, 4%), CoA with ventricular septal defect (VSD) (84, 2.3%), transposition of the great arteries (TGA, simple and complex, 185, 6.3%), TGA with VSD (37, 0%), truncus arteriosus (26, 3.8%), interrupted aortic arch (25, 4%), Norwood Sano (71, 19.7%), neonatal tetralogy of Fallot (41, 0%), and shunt (131 patients, 12%).

Conclusions: Neonatal surgical capabilities have improved substantially over the last decades. Excellent results can be expected for lesions that can be repaired to create biventricular circulation. Improved results can be attributed in part to the evolution of surgical strategies and assistive technologies, but essential is the collaborative effort of surgeons, cardiologists, anesthesiologists, and intensive care specialists acting as a cohesive team whose performance far exceeds the sum of its individual members’ contributions. 

 

Background: Once a well-recognized entity, occult bacteremia (OB) is no longer a significant or serious bacterial infection. First following the introduction of the Haemophilus influenzae type B vaccine and now with the implementation of the conjugate pneumococcal vaccine (PCV), the number of cases has declined significantly. This has led to a change in many published guidelines to avoid taking blood cultures in fully vaccinated children presenting with fever. In Israel, the introduction of the PCV13 is now widespread. 

Objectives: To assess the incidence and outcome of OB, specifically by Streptococcus pneumoniae, in a single large pediatric medical center. 

Methods: We conducted a retrospective review of all cases of pneumococcal bacteremias in the years 2008–2013 and specifically those considered occult. 

Results: Of 355 cases of bacteremia diagnosed during the study period, 164 were caused by S. pneumoniae and 20 (12.8%) were considered occult. None of the OB cases had any complications. OB was not found in children over the age of 36 months. There was a change in the serotypes involving pneumococcal OB. 

Conclusions: OB is uncommon in the PCV-vaccinated population and the serotypes involved have changed.

 

Background: Six medical disciplines are responsible for assessment, diagnosis and treatment of people with attention deficiency hyperactivity disorder (ADHD) in Israel: family doctors, pediatricians, adult and child neurologists, adult and child psychiatrists.  

Objectives: To investigate differences in ADHD diagnostic practices between three different pediatric subspecialties in the clinical setting in order to establish a common ground for a future unified approach.

Methods: An anonymous web-based questionnaire was administered to child psychiatrists, pediatric neurologists and general pediatricians who are actively involved in ADHD diagnosis (n=104).

Results: Neurologists and pediatricians rarely use the mental status examination, while psychiatrists rarely perform a neurological or physical examination (P < 0.0001). A general clinical impression of learning abilities and/or neurodevelopmental skills was implemented more often by pediatric neurologists (P < 0.04).

Conclusions: The significant differences found between the three medical specialties with regard to the clinical evaluation of ADHD could be attributed, at least in part, to the ambiguity of available guidelines concerning the clinical examination, and to the adherence of each specialty to its own "skills." Larger surveys in other countries should be considered and an effort made to create a common, "inter-disciplinary" ground on this important part of ADHD evaluation, differential diagnosis, and research.

 

Background: Medical clowns are increasingly used for diminishing pain and anxiety during painful procedures being performed on children in the hospital setting. Cortisol levels rise as a response to emotional distress. 

Objectives: To investigate whether medical clown-assisted interventions to reduce child's distress during venipuncture have an effect on cortisol levels. 

Methods: During a 1 year period, children requiring blood work or intravenous access in the pediatric emergency department were prospectively randomized to either the presence or absence of a medical clown during the procedure. The child's distress was evaluated using the Faces Pain Scale - revised (FPS-R) for the 4–7 year age group and the visual analog scales (VAS) for those aged 8–15 years. Serum cortisol levels were measured in blood samples obtained by venipuncture. 

Results: Fifty-three children aged 2–15 years were randomly assigned to the study group (with medical clown, n=29) or to the control group (without medical clown, n=24). Combined pain scores of the study group and control group were 2.2 and 7.5 respectively (P < 0.001). No difference in mean cortisol levels was found between the study group and the control group at all ages (16.4 µg/dl vs. 18.3 µg/dl, P = 0.65).

Conclusions: In this pilot study, medical clowns reduced the distress from venipuncture in children. No effect on cortisol levels was observed. 

Women who carry the BRCA gene mutation have an up to 80% chance of developing cancer, primarily of breast and ovarian origin. Confirmation of carrier status is described by many women as an overwhelming, life-changing event. Healthy individuals harboring a BRCA mutation constitute a high risk population with unique needs, often overlooked by health authorities. As such, we felt the need to create a specialized service dedicated specifically to this high risk population. The clinic staff comprises an experienced multidisciplinary team of health professionals who can support the medical and emotional needs of this population. Since its inception in 2001 the clinic has served 318 women. The mean age of patients is 46 years. With a median follow-up of 46 months, 21 women have developed malignancies, including 17 breast cancers, 1 ovarian cancer and 3 additional cancers. All but one of the patients above the age of 40 underwent bilateral salpingo-oophorectomy (BSO). The median and mean ages at BSO were 46.5 and 48 years, respectively (range 33–68). However, only 28.3% underwent bilateral preventive mastectomy. A multidisciplinary clinic for BRCA mutation carriers provides a “home” for this unique population with unmet needs. The high rate of BSO in women before natural menopause indicates that both the medical community and this population are aware of international guidelines supporting this procedure. We believe that a dedicated clinic, with a multidisciplinary team, is likely to contribute to the health, quality of life and survival of BRCA carriers.

Background: Only a minority of patients with essential tremor (ET) and Parkinson’s disease (PD) undergo deep brain stimulation (DBS) surgery. Data on patient selection factors are useful.

Objectives: To compare the clinical characteristics of ET and PD patients who underwent DBS surgery with those of patients who had not undergone surgery.

Methods: We abstracted data from the electronic medical records of 121 PD and 34 ET patients who underwent DBS surgery at Columbia University Medical Center during the period 2009–2014. We compared this group with 100 randomly selected PD and 100 randomly selected ET patients at the Center who had not undergone DBS surgery. 

Results: Among other differences, age of onset in PD patients who had undergone surgery was younger than in those who did not: 14.9% vs. 3.0% with onset before age 40 (P = 0.003). They had also tried nearly double the number of medications (3.9 ± 1.7 vs. 2.3 ± 1.5, P < 0.001). Interestingly, there was no difference in the proportion of patients with tremor (81.0% vs. 88.0%, P = 0.16). Medical co-morbidities (heart and lungs) were less common in the PD patients who underwent DBS surgery. In the ET group, tremor causing impairment in activities of daily living occurred in all surgical patients compared to 73.0% of non-surgical patients (P < 0.001). The former had tried nearly double the number of medications compared to the latter (3.2 ± 1.7 vs. 1.3 ± 1.3, P < 0.001).

Conclusions: These data add to our understanding of the numerous clinical factors associated with patient referral to DBS surgery. 

 

Background: Clinicopathological risk factors for cutaneous squamous cell carcinoma of the head and neck (CSCCHN) are associated with local recurrence and metastasis. 

Objectives: To compare the incidence and risk factors of CSCCHN by age and gender in order to help refine the clinical evaluation and treatment process.

Methods: Clinical and pathological data of all patients diagnosed with CSCCHN during 2009–2011 were obtained from a central pathology laboratory in Israel. Estimated incidence rate calculation was standardized to the 2010 Israeli population. Independent risk factors for poorly differentiated CSCCHN were analyzed using logistic regression.

Results: CSCCHN was diagnosed in 621 patients. Mean age was 75.2 years; mean tumor horizontal diameter was 11.1 ± 6.8 mm. The overall estimated incidence rate in males was higher than in females (106.2 vs. 54.3 per 1,000,000, P < 0.001). Twenty cases (3.2%) had poorly differentiated CSCCHN. Scalp and ear anatomic locations were observed more often in males than in females (22.1% vs. 6.1% and 20.3% vs. 3.3%, respectively, P < 0.001). Per 1 mm increment, tumor horizontal diameter increased the risk for poorly differentiated CSCCHN by 6.7% (95%CI 1.3–12.4%, P = 0.014). 

Conclusions: CSCCHN clinicopathological risk factors are not distributed evenly among different age and gender groups. 

 

Background: Vulvar and vaginal malignant and premalignant lesions are uncommon and are clinically heterogeneous diseases with two pathways of carcinogenesis: human papillomavirus (HPV) induced or non-HPV induced.                    

Objectives: To evaluate the demographic and clinical characteristics associated with vulvar or vaginal cancer and vulvar and vaginal intraepithelial neoplasia 3 (VIN3, VAIN3).

Methods: We conducted a retrospective chart review of 148 women with vulvar and vaginal malignancy and pre-malignancy for the period October 2004 to October 2012, and identified 59 and 19 patients with vulvar and vaginal cancer respectively, and 57 and 13 patients with VIN3 and VAIN3 respectively

Results: The median age of vulvar cancer patients was 30 years older than that of VIN3 patients. HPV was found in 60% and 66.6% of vulvar and vaginal cancer patients respectively, and in 82.3% and 84.6% of patients with VIN3 and VAIN3 respectively. A history of cervical intraepithelial neoplasia (CIN) or warts was observed in 10% and 10.5% of vulvar and vaginal cancer patients respectively, and in 57.9% and 46% of patients with VIN3 and VAIN3 respectively. In 52.6% of patients the vaginal cancer was metastases from other organs. 

Conclusions: Most women with vulvar carcinoma are older than 70 years old. VIN3 and VAIN3 are associated with HPV infection and the most prevalent type is HPV16. Almost half the vaginal cancers are associated with metastases from other organs and almost half of VAIN3 is associated with past cervical dysplasia or carcinoma.