Israel Medical Association Journal

IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition. Accumulated research and data since 2003 have increasingly affirmed its classification as a systemic disease. Although IgG4-RD generally presents with involvement of one or several organ systems, it can affect almost any organ. The disease manifests gradually with the development of tumor-like lesions.

Constitutional symptoms, such as systemic fever and elevated inflammatory markers, are generally absent in association with this pathological condition. However, it is common for patients to exhibit allergy characteristics in addition to a slight increase in peripheral blood eosinophilia. The clinical manifestations of the disease exhibit variability on the specific organ system affected. Specific involvement of organs can ultimately result in organ failure. For example, retroperitoneal fibrosis commonly leads to post-renal failure [1]. The 2020 Revised Comprehensive Diagnostic (RCD) criteria for IgG4-RD include three components for diagnosis: clinical and radiological components (1), serological diagnosis (2), and unique pathological diagnosis (3). When all three are present it is a definitive diagnosis. The presence of components 1 + 3 constitutes a probable diagnosis, while the presence of components 1 + 2 indicates possible diagnosis [2].

Testicular involvement of IgG4-RD has been described in a few case reports. However, the uniqueness of our case is the manifestation of a testicular mass as a different target organ of relapse in a patient in remission from retroperitoneal fibrosis.

Background: Perianal abscess is a common condition among adults. The treatment of choice includes early and efficient drainage. The data regarding risk factors for abscess recurrence, fistula formation, and complications are limited as recent publications mainly focus on patients with inflammatory bowel disease.

Objectives: To determine risk factors for abscess recurrence and fistula formation with regard to patient and surgical characteristics.

Methods: A retrospective analysis was performed on patients who presented to the emergency department and were diagnosed with perianal abscess between 2011–2020.

Results: We included 983 consecutive patients; 741 men, average age 43 years. Recurrence was documented in 434 cases. Crohn’s disease was reported in 70, of which 50 had recurrent episodes (P < 0.0001); 121 of the 234 patients who smoked had recurrence (P = 0.0078); 8% had short symptomatic period (< 24 hours), which was a predisposing factor for recurrence, P < 0.0001. Patients in the non-recurrent group waited 2.53 hours less for surgical intervention (P < 0.0005(. The average time for recurrent episode was 18.95 ± 33.7 months. Fistula was diagnosed in 16.9% of all cases, while 11.6% were within the recurrent group. Surgical expertise of the physician did not significantly change the recurrence rate.

Conclusions: Crohn’s disease and smoking were the only significant risk factors for recurrence of perianal abscess. Timely intervention and drainage of sepsis should not be delayed. Involvement of more experienced surgeons did not seem to alter the natural history of the disease.

Background: Perianal abscesses require immediate incision and drainage (I&D). However, prompt bedside drainage is controversial as it may compromise exposure and thorough anal examination.

Objectives: To examine outcomes of bedside I&D of perianal abscesses in the emergency department (ED) vs. the operating room (OR).

Methods: We conducted a retrospective review of all patients presented to the ED with a perianal abscesses between January 2018 and March 2020. Patients with Crohn’s disease, horseshoe or recurrent abscesses were excluded.

Results: The study comprised 248 patients; 151 (60.89%) underwent I&D in the OR and 97 (39.11%) in the ED. Patients elected to bedside I&D had smaller abscess sizes (P = 0.01), presented with no fever, and had lower rates of inflammatory markers. The interval time from diagnosis to intervention was significantly shorter among the bedside I&D group 2.13 ± 2.34 hours vs. 10.41 ± 8.48 hours (P < 0.001). Of patients who underwent I&D in the OR, 7.3% had synchronous fistulas, whereas none at bedside had (P = 0.007). At median follow-up of 24 months, recurrence rate of abscess and fistula formation in patients treated in the ED were 11.3% and 6.2%, respectively, vs. 19.9% and 15.23% (P = 0.023, 0.006). Fever (OR 5.71, P = 0.005) and abscess size (OR 1.7, P = 0.026) at initial presentation were risk factors for late fistula formation.

Conclusions: Bedside I&D significantly shortens waiting time and does not increase the rates of long-term complications in patients with small primary perianal abscesses.

Background: Statins have anti-inflammatory effects that are independent of their lipid-lowering activity.

Objectives: To examine whether prior statins therapy affects the clinical course of the first episode of acute idiopathic pericarditis (AIP) as the 1-year recurrence and length of hospitalization (LOH).

Methods: This retrospective study included 148 subjects with first episode AIP admitted between the years 2015 and 2019. Data were collected from two hospitals in Northern Israel. We divided the patients in into two groups: 117 those without statins use and 31 those with prior statins use. We compared age, sex, co-morbidities, drugs, laboratory data, 1-year recurrence, and LOH.

Results: The mean age of participants was 43.1 ± 19.4 years. Comparisons between subjects without statins and with prior statins use were made according to age (37.5 ± 16.7 years vs. 64.4 ± 12.7 years, P < 0.01), C-reactive protein (50 ± 40 vs. 48 ± 35 mg/dl, P = 0.9), LOH (5.4 ± 2.85 vs. 8.03 ± 4.92 days, P < 0.01), 1-year recurrence of pericarditis (23 vs. 6 cases, P = 0.95), respectively. Multivariate logistic regression analysis revealed that 1-year recurrence (odds ratio [OR] 0.8, 95% confidence interval [95%CI 0 0.6–1.1, P = 0.41), was not associated with prior statin use, while LOH (OR 2.56, 95%CI 2.08–2.75, P = 0.01) was prolonged with prior statins use in patients with first episode of AID.

Conclusions: Prior statins use in patients with the first episode of AIP did not reduce the 1-year recurrence of pericarditis and prolong the LOH.

Background: Laparoscopic removal of ovarian dermoid cysts has been associated with increased risk for recurrence.

Objectives: To investigate the risk factors associated with recurrence of dermoid cysts.

Methods: We conducted a retrospective review of all women who underwent cystectomy for ovarian dermoid cysts by laparoscopy or laparotomy. At discharge, patients were instructed to undergo a yearly ultrasound exam. A follow-up telephone call was conducted to assess whether an additional surgery for dermoid cysts was required and whether ultrasound recurrence of dermoid cysts was suspected.

Results: The study cohort included 102 participants (92 [90.2%] operated by laparoscopy and 10 [9.8%] by laparotomy). The mean follow-up from the index surgery to the interview was 72.1 ± 38.2 months. The rates of recurrent surgery were similar among women who underwent laparoscopic cystectomy compared with laparotomy (5/92 [5.4%] vs. 1/10 [10.0%], respectively; P = 0.5), while the rates of reported ultrasound recurrence were significantly lower in the laparoscopy group compared with the laparotomy group (10/102 [10.9%] vs. 4/10 [40.0%], respectively; P = 0.03). Additional factors including age, cyst diameter, diagnosis of torsion, intraoperative cyst spillage, estimated blood loss, intraperitoneal adhesions, and postoperative fever were not associated with recurrence.

Conclusions: Ultrasound recurrence of dermoid cysts is not uncommon and could be associated with the surgical approach.

Background: Pilonidal disease in the natal cleft is treated traditionally by a wide and deep excision of the affected area. There is growing awareness, however, to the advantages of minimally invasive surgeries.

Objectives: To compare the efficacy of wide excision operations and minimal trephine surgery in patients with primary pilonidal disease.

Methods: In this retrospective study we examined surgical and inpatient records of 2039 patients who underwent surgery for primary pilonidal disease in five private hospitals between 2009 and 2012. Most procedures were of lay-open, primary midline closure, and minimal surgery types. Pilonidal recurrence rates were evaluated in a subset of 1260 patients operated by 53 surgeons each performing one type of surgery, regardless of patient characteristics or disease severity.

Results: With a mean follow-up of 7.2 years, 81.5%, 85%, and 88% of patients were disease-free after minimally invasive surgery, wide excision with primary closure, and lay-open surgery, respectively, with no statistically significant difference in recurrence rates. Minimal surgeries were usually performed under local anesthesia and involved lower pain levels, less need for analgesics, and shorter hospital stays than wide excision operations, which were normally performed under general anesthesia. The use of drainage, antibiotics, or methylene blue had no effect on recurrence of pilonidal disease.

Conclusions: Minimally invasive surgeries have the advantage of reducing the extent of surgical injury and preserving patient’s quality of life. They should be the treatment of choice for primary pilonidal disease

Background: Pilonidal sinus is a chronic, inflammatory condition. Controversy exists regarding the best surgical  management for pilonidal sinus, including the extent of excision and type of closure of the surgical wound.

Objectives: To assess the short- and long-term outcomes and success rate of the trephine procedure for the treatment of pilonidal sinus.

Method: A retrospective observational cohort study was conducted at a single center. Patients who underwent trephine procedure between 2011 and 2015 were included. Data collection included medical records review and a telephone interview to establish long-term follow-up.

Results: A total of 169 patients underwent the trephine technique for the repair of pilonidal sinus. Follow-up included 113 patients, median age 20 years. Initial postoperative period, 35.6% recalled no pain and 58.6% reported a mild to moderate pain. Postoperative complications included local infection (7.5%) and mild bleeding (15.1%). On early postoperative follow-up, 47.1% recalled no impairment in quality of life, and 25%, 21.2 %, and 6.7% had mild, moderate and sever disturbance respectively. The median time to return to work or school was 10 days. At a median follow-up of 29 months (IQR 19–40), recurrence rate was 45.1% (51/113), and 38 (33.9%) of the patients underwent another surgical procedure. Overweight, smoking, and family history were associated with higher recurrence rate.

Conclusion: The trephines technique has a significant long-term recurrence rate. Short-term advantages include low morbidity, enhanced recovery, and minimal to mild postoperative impairment to quality of life. The trephine procedure may be justified as a first treatment of pilonidal disease

Background: Adnexal torsion in pregnancy is often associated with functional adnexal cysts, especially in pregnancies conceived by ovulation induction (OI) or in-vitro fertilization (IVF). During laparoscopy for adnexal de-torsion, drainage of the functional cysts can be attempted, although this procedure may cause bleeding.

Objectives: To investigate the characteristics of ovarian torsion in pregnancy associated with functional cysts and to compare the rate of torsion recurrence following de-torsion alone versus cyst drainage.

Methods: All cases of surgically diagnosed adnexal torsion occurring during pregnancy between January 2007 and April 2019 in our department were retrospectively analyzed. The cases of torsion associated with presumed functional ovarian cysts were selected. The rate of recurrent torsion during the same pregnancy was compared for de-torsion alone versus de-torsion and cyst aspiration.

Results: Of the 113 women who experienced adnexal torsion during pregnancy, 71 (67.0%) of torsion cases were caused by presumed functional ovarian cysts. Among women with torsion of functional ovarian cysts, the rate of torsion recurrence was significantly higher in patients who underwent de-torsion alone (n=28) compared to women who underwent aspiration and drainage of the ovarian cysts (n=43) (14.3% vs. 0, P = 0.021). There were no cases of intra- or post-operative bleeding in the study cohort.

Conclusions: Functional ovarian cysts are the most common adnexal pathology encountered in pregnant women with torsion. Intra-operative cyst aspiration and drainage may reduce the risk of recurrent torsion. Further multi-center studies are required to validate our data prospectively.

Background: Post-pericardiotomy syndrome (PPS) is a major cause of pericarditis, yet data on the risk of recurrence are limited, and the impact of steroids and colchicine in this context is unknown.

Objectives: To examine the effect of prednisone and colchicine on the rate of recurrence of PPS.

Methods: Medical files of patients diagnosed with PPS were reviewed to extract demographic, echocardiographic, X-ray imaging, and follow-up data.

Results: The study comprised 132 patients (57% men), aged 27–86 years. Medical treatment included prednisone in 80 patients, non-steroidal anti-inflammatory agents in 41 patients, colchicine monotherapy in 2 patients, and no anti-inflammatory therapy in 9 patients. Fifty-nine patients were given colchicine for prevention of recurrence. The patients were followed for 5–110 months (median 64 months). Recurrent episodes occurred in 15 patients (11.4%), 10 patients had a single episode, 4 patients had two episodes, and one patient had three episodes. The rate of recurrence was lower in patients receiving colchicine compared to patients who did not (8.5% vs. 13.7%), and in patients not receiving vs. receiving prednisone (7.7% vs. 13.8%) but the differences were non-significant. Twenty-three patients died and there were no recurrence-related deaths.

Conclusions: The rate of recurrence after PPS is low and multiple recurrences are rare. The survival of patients with recurrent PPS is excellent. Prednisone pre-treatment was associated with a numerically higher rate of recurrence and colchicine treatment with a numerically lower rate, but the differences were non-significant.

Background: Recurrence of tracheoesophageal fistula (TEF) is reported in 8–20% patients. Factors that may influence recurrence of fistula beyond the postoperative period are not clear.

Objectives: To evaluate possible factors associated with recurrence of TEF beyond the immediate postoperative period.

Methods: A single center, retrospective comparison of patients with and without recurrence of TEF was conducted. Medical records of patients previously operated for TEF who were followed in our pediatric pulmonary institute between January 2007 and December 2016 were reviewed.

Results: The medical records of 74/77 patients previously operated for TEF were evaluated. Nine patients (12%) had a recurrence of TEF and 65 did not. These groups had similar age and gender distribution and similar prevalence of VACTERL association. In addition, they had similar length of atretic gap, rates of thoracoscopic surgery, rates of prolonged need for respiratory assistance post-surgery, and frequency of gastrointestinal symptoms. Notably, the patients who had recurrent TEF had significantly more hospitalizations for respiratory symptoms (P = 0.011) and significantly more episodes of clinical bronchiolitis per patient (P < 0.0001). In addition, the patients with recurrent TEF had significantly more episodes of positive polymerase chain reaction for viruses (P = 0.009).

Conclusions: Hospitalizations for respiratory symptoms as well as clinical and/or viral bronchiolitis are associated with recurrence of TEF. Even though cause and effect cannot be established, these patients should undergo meticulous evaluation for the possibility of recurrence of TEF.

Background: Chondrosarcoma of the larynx is a rare tumor. The most common symptom is hoarseness. Treatment is controversial.

Objectives: To describe six patients with laryngeal chondrosarcoma from a single center.

Methods: The medical records of a major tertiary hospital were reviewed for all patients with laryngeal chondrosarcoma diagnosed and treated from 1959 to 2010. Data on background, clinical treatment and outcome were collected.

Results: Six patients, all males with a mean age of 53.3 years, were identified. Partial laryngectomy was performed in three patients, and total laryngectomy, local excision, and partial cricoidectomy in one patient each. Four patients had a permanent tracheostomy after surgery. One patient required postoperative chemotherapy and one radiotherapy. Follow-up time was 12–216 months (mean 102 months). Recurrence developed in two patients 2 and 8 years after initial treatment and was treated by salvage surgery in both patients. One patient died during the follow-up from an unrelated cause. The others are currently alive.

Conclusions: This study supports earlier reports recommending initial treatment with partial or total laryngectomy for laryngeal chondrosarcoma. Long-term follow-up for recurrence is advised. We recommend preserving the larynx, if possible, even if a permanent tracheostomy is necessary.
 

Background: The prognostic significance of biologic markers in women with ductal carcinoma in situ is not fully understood. HER2/neu is a marker of prognostic significance that is routinely assessed in invasive cancer but its correlation with clinical outcome in DCIS[1] is still obscure.

Objectives: To evaluate the significance of HER-2/neu expression as a prognostic marker in DCIS.

Methods: Clinical and pathologic data from 84 patients treated for DCIS were analyzed. HER-2/neu expression was determined by immunohistochemical staining. Histopathologic parameters (nuclear grade, histologic subtype, necrosis, calcifications, margins) were reviewed by an experienced pathologist. Local recurrence and/or metastatic spread were used as endpoints to determine the prognostic significance of HER-2/neu expression.

Results: With a median follow-up of 94.8 months, nine recurrences were reported. Neither univariate nor multivariate analysis showed a significant correlation between HER-2/neu expression and disease recurrence or the time to disease recurrence. Although HER-2/neu expression demonstrated a significant association with high nuclear grade (P < 0.0001) and comedo subtype (P < 0.0001), there was no correlation between these histologic features and recurrence rate. The correlation between high nuclear grade and disease recurrence approached statistical significance (P = 0.07).

Conclusions: No significant association was found between HER-2/neu expression in DCIS and disease recurrence. However, HER-2/neu correlated with negative markers such as nuclear grading and comedo necrosis, and its role should therefore be investigated in larger studies.
 

[1] DCIS = ductal carcinoma in situ

Background: Sporadic abdominal desmoid tumors are rare and data on these tumors as a distinct disease entity are lacking. Previous abdominal surgery, trauma, pregnancy and estrogen intake are considered risk factors. Although desmoidsare benign, invasion and a high recurrence rate are common.

Objectives: To evaluate outcomes of surgery for this rare disease.

Methods: Since 1995, 16 patients with pathologically confirmed desmoid tumor were operated on in our center. All familial adenomatous polyposis patients were excluded. A retrospective analysis of data was performed.

Results: Of the 16 patients 12 (75%) were females. Mean age was 40.5 years (range 24-70). Thirteen patients were symptomatic and 3 were incidentally diagnosed. All patients presented with an isolated mass; 7 (50%) originated in the abdominal wall, 6 (37.5%) were retroperitoneal and 3 were (18.8%) mesenteric. All tumors except one were completely excised. Morbidity was low with no mortality. One patient was reoperated due to involved margins. None of the patients had recurrence within a median follow-up of 64 months (range 5-143).

Conclusions: The perception of sporadic abdominal desmoids as tumors with a high recurrence rate (20-70%) is probably incorrect. Adequate surgery with wide margins leads to a very low recurrence rate; cure is a legitimate goal.
 

The most troublesome complication of acute pericarditis is recurrent episodes of pericardial inflammation, which occur in 15–32% of cases. It was recently found that viral infection has a major role, but in many cases the cause is unknown. The optimal method for prevention has not been fully established; accepted modalities include non-steroidal anti-inflammatory drugs, corticosteroids, immunosuppressive agents, and pericardiectomy. Based on the proven efficacy of colchicine in familial Mediterranean fever, several small and large-scale international clinical trials have shown the beneficial effect of colchicine therapy in preventing recurrent pericarditis. Indeed, colchicines-treated patients consistently display significantly fewer recurrences, longer symptom-free periods, and even when attacks occur they are weaker and shorter in nature. It was also found that pretreatment with corticosteroids substantially attenuates the efficacy of colchicine, as evidenced by significantly more recurrences and longer therapy periods. Colchicine is a safe and effective modality for the treatment and prevention of recurrent pericarditis, especially as an adjunct to other modalities, since it provides a sustained benefit superior to all current modalities. The safety profile seems superior to other drugs such as corticosteroids and immunosuppressive drugs.