Pediatria Medica e Chirurgica最新文献

Proximal and complex hypospadias repairs remain among the most technically demanding procedures in pediatric urology, particularly in patients with inadequate local tissue or previous failed reconstructions. This study reports a five-year institutional experience with a single-stage hybrid technique combining the Snodgrass Tubularized Incised Plate (TIP) repair and the Duckett transverse preputial island flap urethroplasty. Between 2019 and 2024, forty-two patients with severe hypospadias underwent hybrid Snodgrass- Duckett urethroplasty at our institution. Patients were categorized as: Group A (n = 12) with 46,XY Disorders of Sexual Development (DSD); Group B (n = 16) with primary proximal perineoscrotal hypospadias; and Group C (n = 14) with multiple failed prior repairs. The proximal urethra was reconstructed using a Tubularized Native Plate (TNP) and the distal segment with a tubularized preputial island flap (Duckett), joined via an oblique anastomosis. Penile curvature correction and ventral coverage were achieved using lateral flaps. Functional and cosmetic outcomes, as well as complications, were recorded prospectively. A glans-tip meatus was achieved in all patients. Cosmetic appearance was deemed satisfactory in all cases. Uroflowmetry was normal in 95.2% (40/42; 95% CI 86.6- 100). Complications occurred in seven patients (16.7%; 95% CI 5.6-27.7), including urethrocutaneous fistula (n = 4), diverticulum (n = 1), and early breakdown (n = 2), all successfully corrected. Median follow-up was 26 months (IQR 18-36). No urethral strictures or meatal stenosis developed during follow-up. The hybrid Snodgrass-Duckett urethroplasty represents a feasible single-stage alter- native for selected severe hypospadias cases, including reoperative and DSD patients, when local tissue is insufficient. The approach combines the vascular reliability of the preputial flap with the stability of the native urethral plate. While results are encouraging, the retrospective design, absence of a control group, and heterogeneity of the cohort limit generalizability. Prospective multicenter studies with standardized functional and cosmetic scoring are warranted.

Although rare, pool suction-drain injuries may result in potentially fatal consequences: risk of drowning due to hair or extremities entrapment, bowel evisceration, hypovolemic shock, and death. We present two case reports: the first patient was entrapped by a suction grate (placed at the bottom of a water park pool), resulting in an extensive circular lesion on his back; the second child was evaluated for foot entrapment by suction. Both patients were discharged after observation with a good prognosis and without complications. Accidents associated with suction systems are little known, for this reason it is important to emphasize primary prevention. While awaiting new technological tools, public awareness and careful supervision are fundamental. European standards and pool circulation systems must be respected, particularly the use of anti-vortex covers, automatic power cut-offs for filtration systems, dual- drain systems, poolside panic buttons, and supervision of swimmers.

Colostrum is a source of growth factors and nutrients aiding newborns in adaptation to extrauterine life. Its clinical use has been investigated as an immunological component to protect, especially preterm newborns, from early infectious complications. This article aims to investigate the current knowledge about the value of colostrum in enhancing mechanisms of intestinal adaptation in patients affected by Short Bowel Syndrome (SBS). A MEDLINE systematic search was conducted. Inclusion criteria were English language and post-operative colostrum administration in animals and humans undergoing bowel resection. From a total of 734, 10 full-text articles were included: 5 studies on animal models, 4 on humans affected by SBS, and 1 study on animal and paediatric populations. Intestinal adaptation was investigated through diverse clinical, morphological, and functional parameters. No clear benefits of colostrum were reported in both populations. Paucity of trials, limited study duration, and heterogeneous conditions led to poorly standardized results. Colostrum tolerability is an encouraging result, but the outcome of colostrum clinical use in short bowel has still to be determined. Further investigations are required to safely promote use of colostrum in nutritional programs. Standard parameters of intestinal adaptation would be required to evaluate the possible role of colostrum in the process.

The purpose of this paper was to define the specturm of management for Parapneumonial Pleural Effusion/Pleural Empyema (PE/PPE) in children in Italy. We conducted an online survey, distributed by the SIVI committee to 54 Italian pediatric surgery centers. A total of 23/54 (43%) Italian pediatric surgery centers responded. All responders (100%) required an anteroposterior chest radiograph (CXR) as the first imaging approach to suspect PPE, and chest Ultrasound (US) was routinely used in 96% of centers. A preoperative CT scan was routinely performed in 70% of centers. An etiological diagnosis was obtained in more than 80% of patients in 13% of centers, between 40% and 80% of cases in 61% of centers and in less than 40% of patients in 26% of centers. Empirical antimicrobial therapy with 2 antibiotics is the most commonly used therapeutic scheme (78% of centers) and targeted antibiotic therapy was used in 82% of enrolled centers. The majority of centers (57%) approached advanced stage pleural empyema with pleural drain placement and fibrinolysis (PDF); 26% of centers preferred to execute upfront Video-Assisted Thoracoscopic Debridement (VATD), and in 13% of centers, both fibrinolysis and VATD were used. In all cases (100%), urokinase was the fibrinolytic agent of choice. Broncho-Pleural Fistula (BPF) was treated conservatively with prolonged Pleural Drainage (PD) and antibiotics in 82% of centers, while the remaining 18% proposed early surgical treatment. 82% of centers proposed prolonged antimicrobial therapy for the treatment of Lung Abscess (LA), while 18% of centers preferred to execute upfront ultrasound-guided or thoracoscopic positioning of a pig-tail drain. As expected, we observed a lack of homogeneity in the treatment between the different centers: most of these have a preference for fibrinolysis over the use of primary VATD, with urokinase being the only fibrinolytic agent used in all centers. It would be desirable to involve as many centers as possible for the drafting of shared national guidelines in the treatment of PPE in children in Italy.

Nutcracker Syndrome (NCS) is characterized by impeded blood flow from the Left Renal Vein (LRV) into the inferior vena cava due to an abnormally narrow angle between the abdominal aorta and superior mesenteric artery. This syndrome is usually associated with hematuria, abdominal pain and orthostatic proteinuria. We described three patients diagnosed with NCS (mean age 16.3 years). The aortomesenteric angle, LRV diameter ratio and peak velocity ratio were assessed through doppler ultrasonography and CT angiography. A kidney model was printed out for surgical planning. An extravascular stent was designed based on the LRV's structure using computer software and printed in three dimensions with a precision setting of 20μm. Patients underwent laparoscopic placement of the extravascular stent. The mean duration of surgery was 180 minutes. There were no intraoperative complications. CT examinations revealed that pre- and postoperative AMA ranged from 18.7°±4.3° to 55.0°±4.4, respectively. No side effects were observed in the follow-up period (range 12-24 months). At present, treatment guidelines for NCS are unclear, and the different therapeutic principles need to be applied in a patient-specific manner. Our results confirm previous reports in literature concerning the efficacy and safety of ES in pediatric patients.

Pilomatricoma and trichilemmal cyst are two benign tumors of hair follicles that can affect children. We performed a retrospective study to histologically differentiate the two tumors to determine if this has clinical utility. For 5 years, we conducted a retrospective study on the patients who underwent surgery and evaluated some parameters (site, recurrence and scar outcome) categorizing them based on their histological type. We treated 71 patients, with an average age of 7.6 years. Most of the lesions were localized on the scalp, head and neck. Histological examination diagnosed 55 pilomatricomas and 16 trichilemmal cysts. Five patients with pilomatricoma developed an unaesthetic scar (4 in subscapular region), while the recurrence was present in 10 cases, of which 6 were trichilemmal cysts (37,5% of 16 cases). Both types of lesions healed successfully, however trichilemmal cysts are more susceptible to recurrence.

Cushing's Disease (CD), defined as hypercortisolism caused by excess Adrenocorticotropic Hormone (ACTH) secretion by a pituitary corticotroph adenoma, rarely presents in the pediatric age range. The aim is to describe a 12-year-old Albanian boy with a challenging pathway to diagnosis and treatment process for Cushing's disease. He presented with headaches, rapid weight gain during the last three years, stunting, increased body hair growth, and a typical Cushingoid appearance. After a consultation in the Pediatric Endocrinology Unit at the University Hospital Center, "Mother Teresa," in Tirana, the boy was pre-diagnosed with Cushing Syndrome, and hypophyseal adenoma was suspected due to suggestive laboratory tests, although non-consistent imaging results. An ectopic ACTH-dependent Cushing syndrome was suspected together with neuroendocrine neoplasia (carcinoid tumor) as a thoracic CT showed a nodular lesion with regular-lobulated sharp contours in the lower lobe of the right lobe resulted in pulmonary tuberculosis granuloma. Even imaging failed to identify the ACTHsecreting microadenoma; the decisive examination was an intervention to collect samples from the inferior petrosal sinus during the CRH test, which found a left-side ACTH-secreting focus. Left hemi-hypophysectomy was performed using gamma knife therapy, resulting in effective normalization of hypercortisolism, but with the side effect of growth hormone deficiency.

Phimosis is a pathological condition in children and is easy to diagnose. In particular cases it can be associated with curious malformations of the foreskin. Four adolescents (aged from 9 to 13 years) presented at our attention because of a phimosis with hooded foreskin. Symptoms complained were: balanitis, urinary infection, preputial urinary retention, or psychological problems. One of the cases was asymptomatic. No other malformations were present. The surgical treatment was to remove the hooded foreskin and treat the ventral part of the skin of the penis to avoid abnormal curvatures. This type of malformation should be recognized and treated even if asymptomatic because it can create psychological problems in adolescence. The surgical treatment must prevent penile curvatures.

Lymphatic Malformations (LMs) are benign congenital malformations of the lymphatic system that commonly involve the abdomen in children (mesentery of the small intestine and omentum). The management of these malformations is not unique. 7 children with different ages (range: newborn to 14 years), diagnosis was incidental in some cases, while in others for abdominal pain. All patients underwent abdominal ultrasound and Magnetic Resonance Imaging (MRI). Laparoscopy for diagnosis was useful in 4 cases. Treatment was: conservative in 1 child, laparoscopic excision in 3 patients, laparotomic excision in 3 cases. At follow up we observed recurrence in a case that required integrated treatment and bowel occlusion after excision in 1 case. This benign malformations may not cause any symptoms to patient. The goal of treatment is to maintain organ function, preserve aesthetic integrity and complications control. Management of these patients could be varied: the best approach should be evaluated on the basis of the patient.

Pediatric Colorectal Diseases (PCRD), mainly Anorectal Malformations (ARM) and Hirschsprung's Disease, are a major issue in Sub-Saharan Africa (SSA). Even with advances in healthcare facilities and global health initiatives, most of children living in low resources SSA lack of specialist pediatric surgical facilities. Improved healthcare access is needed to manage PCRD in these locations, to prevent and reduce missing or delayed diagnosis, early mismanagement by inexperienced health practitioners, and barriers to corrective therapy and long-term follow-up. A retrospective analysis of data from three SSA hospitals shows that international surgical outreach visits helped increase capacity. Along one hundred twentyfour weeks of staggered surgical outreach visits 174 ARM and 64 highly suspected HSCR cases were collected. The study evaluates 152 ARM and 59 HSCR patients who had not been treated before. Those who came after an unsuccessful treatment elsewhere were not included. Management, clinical course, complications and results are reported. Focus is on context-aware adaptive surgery and sustainable solutions to improve outcomes and quality of life for those children, discussing long-term follow-up options and results. The local context has a substantial impact on epidemiology, demographics, and presentation compared to high-resource countries. Intestinal stomas done at a primary health facility level presented at referral to our Hospital with 25% complication rate. Due to social, economical, and transit issues, only 108 ARM and 41 HSCR could finally receive a corrective treatment by the outreach visiting teams. Complications (9.1%) were controlled using adaptive solutions. Only one surgical fatality occurred. A limited proportion of patients (46% ARM, 31% HSCR) attended a regular follow up schedule for one year or more, and finding those lost in wide rural areas was difficult. Successful and comprehensive PCRD management in under-resourced SSA requires better training at the primary health level on early recognition and correct, first surgical approach, together with a referral network to specialist facilities for further treatment. Surgical short-term outreach trips can boost local capability in under-resourced areas. The research of adaptive and sustainable surgical solutions to reduce hospital stay and staged treatments time for PCRD must be emphasised. Nevertheless financial and logistical constraints still challenge post-discharge monitoring and follow-up, which remain crucial for long-term outcome.