Recurrent reactive non-sexually related acute genital ulcers: a risk factor for Behcet's disease?

BACKGROUND AND AIM: Lipschutz ulcers (LU) are idiopathic genital lesions characterized by the sudden appearance of painful, usually symmetric vulvar ulcers, typically occurring in sexually inactive adolescents. LU is a diagnosis of exclusion. As these lesions heal spontaneously, in the absence of tissue scarring, the therapy is mainly symptomatic and focuses on pain relief. Recurrence of LU associated with oral ulcers describes the clinical picture of complex aphthosis, which belongs to Behçet's disease (BD) pathological spectrum. Our work aims to analyze the correct diagnostic approach to recurrent aphthous, focusing on the importance of a multidisciplinary assessment and immunogenetic investigation to identify the subjects at risk of progression towards BD.   Methods: We present the case of a 12-year-old non sexually active Italian girl who was diagnosed with LU. After 15 months, she presented recurrent reactive non sexually related acute genital ulcer associated with a history of oral aphthous. According to clinical features and anamnesis, complex aphthosis was diagnosed. For diagnostic purposes, she underwent an immunogenetic analysis that showed HLA-B51 positivity. RESULTS: In the absence of clinical and laboratory criteria to define the risk of progression of complex aphtosis towards BD, we think that besides a strict follow-up, in pediatric patients with a suggestive clinical history, it is crucial to adopt a multidisciplinary approach, comprehensive of HLA investigation, in order to guarantee an early diagnosis and a prompt therapeutic intervention. CONCLUSIONS: In children and adolescents with genital ulcers, it is essential to consider all the possible differential diagnoses to undertake a timely and correct course of treatment.

46, XY Disorders of Sexual Development: a case report and theoretical framework.

BACKGROUND AND AIM: Disorders of sexual differentiation (DSD) with karyotype 46,XY include gonadal developmental differences such as complete gonadal dysgenesis, partial gonadal dysgenesis, testicular regression and ovotesticular sexual differentiation disorder, differences in androgen synthesis or action, such as androgen synthesis deficiency, androgen action deficits, LH receptor deficiency, AMH synthesis or action deficits, and other conditions such as severe hypospadias, cloaca estrophy, etc. Methods: A 17 years-old girl came to our attention for hirsutism, clitoral hypertrophy, primary amenorrhea, and bilateral mammary hypoplasia. According to clinical features and anamnesis, the diagnosis of 46, XY DSD was made. For diagnostic purposes, she underwent an extensive genetic analysis, hormone dosage and instrumental examinations. After a clitoridoplasty and hormone replacement treatment, the patient performs appropriate multidisciplinary follow-up and regular psychotherapy. RESULTS: The clinical case reported falls, according to the recent classification developed by the Chicago Consensus, within the scope of DSD with karyotype 46, XY. About 160 cases of patients with 17ß-HSD3 deficiency, diagnosed at a mean age of 12 years, are described in the literature, most of them coming from Western Asia and Europe and only three cases from Eastern Asia. Clinically, about 30% of patients showed virilization, 20% clitoromegaly, ambiguous genitalia, inguinal/labial mass, 16% primary amenorrhea, and 5% absence of mammary development, features that are partly traced in the case described here. CONCLUSIONS: This case underscores the complexity of managing individuals with DSD. Having acquired the concept that irreversible surgery should be avoided, except in cases where failure to do so would determine health risks, the primary objective of the medical decision lies in meeting conditions aimed at harmonious sexual identification, especially regarding sexual activity and fertility, involving a team of experienced professionals (psychologists, pediatricians, surgeons, endocrinologists, radiologists), capable of promptly identifying suggestive clinical signs.

Effectiveness of Articulating Linear Stapler for Total and Partial Laparoscopic Splenectomy in Children.

Purpose: We performed a retrospective study to determine the effectiveness and feasibility of articulating linear stapler in laparoscopic total splenectomy (LTS) and laparoscopic partial splenectomy (LPS), focusing on technical laparoscopic skills that could help pediatric surgeons to avoid intra- and postoperative complications. Methods: Retrospective cohort study of children younger than 18 years who underwent laparoscopic spleen surgery between January 2008 and March 2020. Age, sex, indication for surgery, operative time (OT), intra- and postoperative complications, and postoperative length of hospital stay (LHS) were analyzed. Data from parenchymal resection and vessels sealing techniques were obtained. Results: Thirty patients, 19 LTS and 11 LPS, were included. The mean age of the patients was 10.9 years, and 16 patients were male and 14 were female. For hematologic diseases, LTS was the elective surgery, associated with cholecystectomy in 5 cases. LPS was the common procedure for splenic cysts. The stapler was used in LTS to close the hilum vessels and in LPS for parenchymal resection. No statistically significant differences in OT were observed comparing LTS and LPS. Two conversions occurred in LTS; none in LPS. The mean LHS was 6 days in both groups. No recurrence or major complications appeared in both groups at 1-12 years of follow-up. In particular for LPS, there are no relapse of cyst neither reduction in splenic function. Conclusions: This study shows the effectiveness, feasibility, and safety of mechanic stapler in splenic surgery both for hilum vessels sealing and for parenchymal resection. The use of this device can reduce risk of hemorrhagic recurrences or major surgical complications improving the safety of the operation.

Vesicoscopic Treatment of Symptomatic Congenital Bladder Diverticula in Children: A 7-Year Experience.

Introduction The objective of this study was to report on the use of vesicoscopy in the treatment of symptomatic congenital bladder diverticula (CBD) in children. Material and Methods In this study, 16 males, aged 4 to 12 years (median age, 6.25 years), were treated for symptomatic CBD; 3 patients presented double diverticulum and 13 presented single diverticulum. The presenting symptoms were recurrent urinary tract infection, hematuria, lower abdominal pain, and voiding dysfunctions as urgency, frequency alone, or in association. A first midline 5-mm trocar was introduced for a 0-degree telescope at the dome of the bladder, and two left and right 3- or 5-mm trocars were inserted through the anterolateral wall. The bladder was then insufflated with carbon dioxide to 10 to 12 mm Hg pressure. The diverticula were inverted into the bladder and the mucosa around the neck was circumcised by using scissors and hook. The defect was sutured and the bladder was drained. Vesicoureteral reflux (VUR) of third grade or higher was treated endoscopically. Results Mean operative time was 90 minutes for procedures. At 6-month follow-up, ultrasound and voiding cystourethrogram (VCUG) showed the disappearance of the diverticulum in 15 out of the 16 patients. The patient, with huge double diverticulum and fourth grade right VUR, presented recurrence of a small left diverticulum. Patients with voiding disorders presented a gradual improvement of their urgency. VUR disappeared at VCUG in all patients. Conclusion Vesicoscopic diverticulectomy resulted a safe and effective procedure and can be considered a valid alternative to the open or laparoscopic procedures. In our opinion, routine use of vesicoscopy could become the gold standard for the treatment of CBD in children.

The role of laparoscopy in recurrent right lower quadrant pain in children.

According to scientific literature, laparoscopy as aid in diagnosis and therapy for chronic pain in the right iliac quadrant shows a undeniable advantage thanks to its mini-invasiveness, the possibility of a methodical and thorough exploration of the entire abdominal cavity in those cases of recurrent pain, emotionally and socially debilitating, that do not find an answer in the usual etiological diagnostic clinical-instrumental. In those cases in which any significant organic pathology that justifies the recurring pain in the right iliac fossa is found during laparoscopic exploration, it has been seen that it is useful to perform appendectomy anyway, that leads to the disappearance of symptoms, which are probably due to inflammatory recurrent catarrhal phenomena of appendix in such patients, as it is demonstrated by the adhesions found at cecum-appendicular level. From January 2011 to December 2013, 24 children with chronic recurrent right lower quadrant pain were subjected to diagnostic laparoscopy. Ages varied from 11 to 18 years (mean, 14 years). There were 6 males and 18 females. Laparoscopic findings included macroscopical signs of acute appendicitis in 15 patients; cecal adhesions in 20 patients, kink of the appendix in 3. The abdominal pain completely resolved in all the patients following laparoscopy.

Single-incision laparoscopic surgery and conventional laparoscopic treatment of varicocele in adolescents: Comparison between two techniques.

BACKGROUND: Single-incision laparoscopic surgery (SILS) has gained great popularity in paediatric surgery due to its minimally invasive approach and improved cosmetic results. Notwithstanding, reports describing its adoption in children are still fragmentary and some perplexities have been raised by some surgeons. We reviewed our experience with the SILS Palomo varicocelectomy procedure (SIL-V) in children and adolescents, comparing this group with a similar series operated using conventional laparoscopic varicocelectomy (CL-V). PATIENTS AND METHODS: A total of 69 Palomo laparoscopic varicocelectomies were performed in patients aged 11-17 years from January 2011 to January 2013. Indications for surgery included grades II-III varicocele or ipsilateral testicular hypotrophy. The SIL-V procedure was performed in 44 patients with roticulating and conventional 5 mm instruments. Testicular vessels were isolated "en bloc," clipped and cut. Operating time, visual analogue scale and post-operative results were compared to a similar group of 25 patients operated with CL-V. RESULTS: No patient of the SIL-V group required conversion to conventional laparoscopy, none to open surgery. Mean operative time was 22 min (range: 19-28) in the SIL-V group, not significantly different compared with CL-V (mean 21 min, range: 18-25). All patients experienced a smooth recovery from surgery without any complications, and were discharged on day 1. No difficulties were found in the SIL-V group. The post-operative pain score was significantly better in SIL-V. CONCLUSION: The SIL-V procedure is safe and effective and allows a fast and efficient isolation of the vascular bundle. The use of conventional instruments is technically feasible in SIL-V.

"Spaghetti maneuver": a useful tool in pediatric laparoscopy - our experience.

AIMS: The laparoscopic "Spaghetti Maneuver" consists in holding an organ by its extremity with a grasper and rolling it up around the tool to keep the organ stable and facilitate its traction within a small space. We describe our experience with the "Spaghetti Maneuver" in some minimally invasive procedures. MATERIALS AND METHODS: We successfully adopted this technique in 13 patients (5F : 8M) aged between 6 and 14 years (average age, 10) on whom we performed 7 appendectomies, 2 ureteral reimplantation and 4 cholecystectomies. In all cases, after the first steps, the appendix, the gallbladder and the ureter were rolled around the grasper and easily isolated; hemostasis was thus induced and the organ was mobilized until removal during cholecystectomy and appendectomy, and before the reimplantation in case of ureteral reimplantation. RESULTS: We found that this technique facilitated significantly the acts of holding, isolating and removing, when necessary, the structures involved, which remained constantly within the visual field of the operator. This allowed a very ergonomic work setting, overcoming the problem of the "blind" zone, which represents a dangerous and invisible area out of the operator's control during laparoscopy. Moreover the isolation maneuvers resulted easier and reduced operating time. CONCLUSION: We think that this technique is easy to perform and very useful, because it facilitates the dissection of these organs, by harmonizing and stabilizing the force of traction exercised.