Granular Cell Tumor (Abrikossoff's Tumor) of the Vulva in Childhood.

Granular cell tumors (GCTs) (Abrikossoff's tumors) are rare neoplasms derived from Schwann cells. Immunohistochemistry remains the most useful instrument for diagnosing GCTs. Complete surgical excision has been demonstrated to be curative for benign lesions. However, long-term follow-up in these patients is strongly recommended.

Laparoscopic orchiopexy: short-term outcomes. Experience of a single centre.

BACKGROUND: Increased infertility and smaller volume accompany undescended testis. Timing of orchiopexy is still a matter of debate. We evaluated the growth of non-palpable testes after laparoscopic orchiopexy according to age at surgery, intraoperative findings and type of procedure. METHODS: Forty-one boys undergoing laparoscopy for nonpalpable testes were retrospectively reviewed and divided into two groups, ≤18 months and >18 months, according to their age at surgery. RESULTS: At follow-up, 14 testes in the younger group had normal size, while 3 atrophied either after single (2) or two stage procedure (1). Similarly, in older boys 11 testes grew normally, while 5 atrophied after both procedures. CONCLUSIONS: Most of the non-palpable testes grew normally after laparoscopic orchiopexy and the postoperative volume seemed independent from the surgical strategy. Both techniques led to a few cases of testicular hypotrophy. In our experience, the age at surgery did not affect the outcome in terms of testicular growth.

Pediatric schwannoma of the hypoglossal nerve: a case report and narrative literature overview.

Schwannomas are benign, slow-growing tumors originating from the Schwann cells of nerve sheaths. Extracranial schwannomas are rare, particularly in pediatric populations. Here, we report the case of a hypoglossal schwannoma in a 15-year-old male who experienced tongue paresthesia and fasciculations and difficulty swallowing two years before hospital admission. Magnetic resonance imaging showed an oval mass with sharp and regular limits of approximately 45 × 29 × 25 mm in the cranial portion of the right carotid adipose space, caudal to the right carotid and lateral foramen. The patient underwent surgery, and a histological examination confirmed a schwannoma of the hypoglossal nerve. Six months after surgery, the patient was symptom-free. The literature on schwannomas of the hypoglossal nerve is scarce, with only a few previously reported cases in the adult population. Despite their rarity, schwannomas should be considered in the differential diagnosis of masses located in the neck that present with lingual and occasionally auditory symptoms, even in pediatric patients. Surgical resection is recommended and has a low risk of long-term recurrence.

Informed consent in minor and intermediate pediatric elective surgery: results of an in-house questionnaire.

Background: The aim of this study was to evaluate the quality of our surgical informed consent and parents'/guardians' late recall of surgical procedures and risks of elective day surgery after pre-operative interview with surgeons. Methods: All parents/guardians of patients <18 years of age undergoing minor and intermediate elective procedures from January 15th to September 1st, 2022, were prospectively enrolled in the study. Before discharge, parents/guardians were asked to complete an in-house questionnaire regarding the duration of the consent procedure, duration of the interview, quality of the informative handouts, and their ability to recall the type of disease, type of surgical procedure, and surgical risks. Results: One hundred and two questionnaires were returned. In all cases, informed consent was obtained between 24 and 72 h prior to surgery. The following responses were collected: 98/102 (96%) parents/guardians reported that the duration of the consent process was adequate; 95/102 (93%) reported that the handouts were fully informative, and 7/102 (7%) reported that they were partially informative regarding explanation of the disease and surgical procedure; regarding complications, 93/102 (91%) perceived the handouts to be fully/partially informative, while 4/102 (4%) perceived the handouts to be poorly/non-informative, and 5/102 (5%) did not provide a response; 94/102 (92%) stated that they remembered the pathology, but only 87/94 (93%) recalled it correctly; 90/102 (88%) stated that they remembered the type of procedure, but only 76/90 (84%) recalled it correctly; and 53/102 (52%) stated that they remembered the surgical risks, but only 20/53 (38%) could recall more than one complication. Conclusions: Late recall of surgical complications by parents was poor despite the high perceived quality of the surgical risk handouts and medical interview. Implementation of expedient methods may improve overall comprehension and satisfaction of parents/guardians regarding the IC process. Further, more efforts should be made to develop standardized guidelines for an optimal IC process.

Robotic-assisted surgery in pediatrics: what is evidence-based?-a literature review.

Background and Objective: The use of robotic-assisted surgery (RAS) has increased more slowly in pediatrics than in the adult population. Despite the many advantages of robotic instruments, the da Vinci Surgical System (Intuitive Surgical, Sunnyvale, CA, USA) still presents some limitations for use in pediatric surgery. This study aims to examine evidence-based indications for RAS in the different fields of pediatric surgery according to the published literature. Methods: A database search (MEDLINE, Scopus, Web of Science) was performed to identify articles covering any aspect of RAS in the pediatric population. Using Boolean operators AND/OR, all possible combinations of the following search terms were used: robotic surgery, pediatrics, neonatal surgery, thoracic surgery, abdominal surgery, urologic surgery, hepatobiliary surgery, and surgical oncology. The selection criteria were limited to the English language, pediatric patients (under 18 years of age), and articles published after 2010. Key Content and Findings: A total of 239 abstracts were reviewed. Of these, 10 published articles met the purposes of our study with the highest level of evidence and therefore were analyzed. Notably, most of the articles included in this review reported evidence-based indications in urological surgery. Conclusions: According to this study, the exclusive indications for RAS in the pediatric population are pyeloplasty for ureteropelvic junction obstruction in older children and ureteral reimplantation according to the Lich-Gregoire technique in selected cases for the need to access the pelvis with a narrow anatomical and working space. All other indications for RAS in pediatric surgery are still under discussion to date, and cannot be supported by papers with a high level of evidence. However, RAS is certainly a promising technology. Further evidence is strongly encouraged in the future.

Management of pleural effusion and empyema in a third-level pediatric surgical center.

BACKGROUND: Pleural effusion and empyema are frequent complications of acute bacterial pneumonia in children. To date, evidence regarding the optimal treatment of this condition is limited in the literature. METHODS: Patients with pleural effusion and empyema admitted at our Department of Pediatric Surgery over the last ten years were enrolled in this retrospective study, and successively compared with the clinical charts of patients treated before the introduction of a new diagnostic and therapeutic algorithm. In particular, primary outcomes investigated between pre- and postalgorithm period were the use of diagnostic tools and antibiotics, the need for additional therapeutic approaches, complications, and the length of stay. RESULTS: After the introduction of the new algorithm there were a decrease in the use of chest radiography for re-evaluation (100% vs. 79%, P=0.003), a more focused use of computed tomography (68% vs. 15%, P=0.001), and a decrease in the use of a second computed tomography (18% vs. 3%, P=0.07); in favor of an increase in the use of the ultrasound scan (40% vs. 100%, P=0.001). There was also a shift from the use of chest tube drainage alone to the use of drainage for urokinase administration (50% vs. 92.3%, P=0.001), and a statistically significant decrease in the need for video-assisted thoracoscopic surgery (25% vs. 7.7%, P=0.001). CONCLUSIONS: The introduction of a diagnostic and therapeutic algorithm for the management of pediatric pleural effusion and empyema has notably resulted in a decrease in the utilization of radiography and computed tomography and an increase in the use of ultrasonography, reducing unnecessary radiation exposure in children and overall costs.

Laparoscopic fundoplication after oesophageal atresia repair.

Background: Esophageal atresia (EA) is a rare congenital malformation. A high incidence of GER unresponsive to medical management is noted with EA. Literature suggests that complications from GER can persist in adulthood. In paediatric age, laparoscopic treatment is a valid option even if recurrence rate is not negligible. Aims and Objectives: To evaluate our experience with gastro-esophageal reflux (GER) treatment after esophageal atresia (EA) repair. Materials and Methods: We retrospectively analysed 29 consecutive patients treated for EA at birth and studied for GER at our Institute in a period of 11 years. Results: 24/29 (82,7%) cases had symptoms of reflux, 17/29 (58,6%) cases were treated with laparoscopic fundoplication (LF). Three infants were younger than 6 months and had apparent life threatening events (ALTE) condition as principal indication for surgery. No intra-operative complications occurred. 3/17 LF had open surgical conversion due to technical problems. 2/17 cases required a second operation. At the last follow-up: (1) 6/17 (35,3%) of patients healed after the last operation, (2) 8/17 (47,1%) have GER improvement (four still in medical treatment), (3) 2/17 (11,8%) have persistent GER, (4) 1/17 (5,9%) died for causes not related to antireflux surgery. Conclusions: According to literature and to our retrospective analysis, LF for GER after EA repair is feasible, even if recurrence risk is not negligible. Infants less than 6 months old with associated conditions (malformations, gastrostomy/jejunostomy) seem to have a higher failure rate with a greater risk of conversion. Longer follow-up and multicenter experiences would guarantee an adequate surveillance for patients with EA.

Management of Primary Spontaneous Pneumothorax in a Third-Level Pediatric Surgical Center: A Retrospective Study.

Introduction: The management of primary spontaneous pneumothorax (PSP) in pediatrics remains controversial. The aim of this study was to investigate the risk of recurrence after non-surgical treatment vs. surgery, the difference in the length of stay (LOS) between various treatment options, and the role of computed tomography (CT) in the management of PSP. Materials and Methods: We retrospectively reviewed patients admitted to our Pediatric Surgery Unit for an episode of PSP between June 2009 and July 2020. Medical records including clinical presentation at admission, diagnostics, treatments, complications, and LOS were collected. Results: Twenty-three patients (22 males and 1 female) were included in this study. Median age was 15.65 (range 9-18). Chest X-rays were performed in all patients and showed 5 small (22%) and 18 large (78%) PSP. Chest drain was used for large PSP (≥2 cm) if the patient was clinically unstable. Eleven patients (48%) were managed non-operatively with observation alone and a recurrence rate of 18%, chest drain was used in 11 patients with a recurrence rate of 36%, and surgery was deemed necessary as a first treatment choice in one case. Six patients (27%) had an episode of relapse after non-operative management or chest drain placement. Following surgery, a relapse occurred in 2 of the 6 patients. Chest drain insertion was associated with a longer LOS than observation alone (6.36 vs. 2.4 days), and surgery resulted in a longer LOS than other types of treatment (P = 0.001). Conclusion: According to our experience, small PSP or clinically stable larger PSP can be treated conservatively with observation alone. Operative management should be taken into consideration in children with large symptomatic PSP, persistent air leak, and/or relapse after chest drain insertion.

Oxygen-enriched oleic matrix (NovoX) for wound healing in pediatric patients undergoing open surgical treatment for pilonidal disease: Preliminary experience.

Introduction: Pilonidal disease (PD) is a common infectious and inflammatory condition affecting the gluteal cleft and sacrococcygeal region. The optimal treatment for PD remains controversial. While the open technique reduces the number of relapses compared to minimally invasive approaches, it is associated with a longer healing time. Reactive oxygen species are a key part of the normal wound-healing process. Herein, we reported our preliminary experience using a new oxygen-enriched oil-based product called NovoX for wound healing after open surgery for PD. Materials and methods: We used a new oxygen-enriched product for wound healing in three pediatric patients undergoing open surgical repair for PD between December 2021 and April 2022. During postoperative follow-up, healing time and the aesthetic result were evaluated. Results: Our preliminary study included three patients with chronic PD. The average follow-up time was 5 weeks, corresponding to the end of the healing process and the resumption of normal daily activities. Only one mild complication occurred during the study period. No short-term side effects were reported. The cosmetic result was reported as satisfactory. Conclusion: NovoX is easy to apply, safe, and effective for treating pediatric patients undergoing open surgical treatment for PD, leading to slightly faster wound healing with good aesthetic outcomes.

Bilateral sactosalphinx and congenital adrenal hyperplasia: case report on two rare conditions in two virgin girls.

BACKGROUND: Sactosalpinx means a collection of fluid (serum, blood or pus) in the fallopian tube. CAH (Congenital Adrenal Hyperplasia) is a typical 46XX DSD (Disorder of Sex Development) due to a steroidogenic enzymatic defect. Both conditions are rare and can lead to reduced fertility rate. CASE PRESENTATION: We describe two post-menarche virgin girls with CAH who were hospitalized for acute abdomen due to laparoscopically confirmed sactosalpinx. Case 1 recovered after conservative management, case 2 after a second-look and bilateral salpingectomy. The first case consisted of right sactosalpinx and previous peritonitis reported; the second one of bilateral symptomatic pyosalpinx and previous vaginal stenosis. Recurrent abdominal pain persisted at follow-up in Case 1: post-operative MRI (Magnetic Resonance Imaging) showed bilateral hydrosapinx that disappeared at a following ultrasound scan control. Follow-up was uneventful 36 months after surgery in Case 2, except for the surgical revision of the vaginal introitus. CONCLUSIONS: CAH-sactosalpinx association is a very rare but not negligible event. We suggest a conservative approach for sactosalpinx if tubal and/or ovary torsion can be excluded. Pyosalpinx is more challenging to treat, but during pediatric age we suggest starting with a conservative approach, especially in patients with CAH who have a potential low fertility rate. Careful gynecological follow-up after menarche is recommended to rule out any further causes of infertility.

The Histopathology of the Appendix in Children at Interval Appendectomy.

Whilst most surgeons agree that conservative treatment of appendiceal abscess in children is an adequate treatment, the need for subsequent interval appendectomy is still controversial. We analyzed the histopathology in interval appendectomy in search of signs of inflammation. All patients admitted between 2010 and 2017 with appendiceal abscess and scheduled for interval appendectomy were reviewed. The specimens were evaluated for grade of inflammation, type and distribution of cellular infiltrate, presence of necrosis or hemorrhage and infiltrate in the serosa. Forty-two patients had appendiceal abscess and were treated conservatively. Seven underwent emergent appendectomy. Thirty-three out of 35 patients underwent elective interval appendectomy. Thirty-two specimens were revised. Carcinoid tumor or other malignant lesions were not found. All of them presented some amount of inflammation, grade 1 to 2 in 53%, grade 3 to 4 in 47%. Twenty-five percent of the specimens had signs of necrosis accompanied by hemorrhage and in more than the half (53%) the infiltrate extended to the serosa. Conclusions: Although the appendix was mostly found not macroscopically inflamed intraoperatively, histology confirmed a certain grade of inflammation even months after the conservative treatment. No correlation was found between histopathologic findings and lapse of time between abscess treatment and interval appendectomy.

Pyogenic granuloma arising within capillary malformations in children: A case report and literature review.

Pyogenic granuloma (PG) is an acquired benign vascular hyperplasia. Even though PG is not so rare, its etiology is still unclear. Assuredly, an association between vascular anomalies and PG is an extremely rare finding, and a proper management of these cases is not well standardized to date. Herein, we report our experience with a giant PG arising spontaneously within a port-wine stain of the thorax. Previous cases reported in the pediatric literature and strategies of treatment are also discussed.