Proteomics of urinary small extracellular vesicles in early diagnosis of kidney diseases in children-expectations and limitations.

The primary function of the kidneys is to maintain systemic homeostasis (disruption of renal structure and function results in multilevel impairment of body function). Kidney diseases are characterized by a chronic, progressive course and may result in the development of chronic kidney disease (CKD). Evaluation of the composition of the proteome of urinary small extracellular vesicles (sEVs) as a so-called liquid biopsy is a promising new research direction. Knowing the composition of sEV could allow localization of cellular changes in specific sections of the nephron or the interstitial tissue before fixed changes, detectable only at an advanced stage of the disease, occur. Research is currently underway on the role of sEVs in the diagnosis and monitoring of many disease entities. Reports in the literature on the subject include: diabetic nephropathy, focal glomerulosclerosis in the course of glomerulopathies, renal fibrosis of various etiologies. Studies on pediatric patients are still few, involving piloting if small groups of patients without validation studies. Here, we review the literature addressing the use of sEV for diagnosis of the most common urinary disorders in children. We evaluate the clinical utility and define limitations of markers present in sEV as potential liquid biopsy.

Open Fetal Surgery for Ventricular-Amniotic Valve Implantation in Aqueductal Stenosis-Dependent Severe Fetal Hydrocephalus: A Case Report with 7-Year Follow-Up.

INTRODUCTION: Fetal ventriculomegaly is one of the most commonly diagnosed central nervous system pathologies of the second trimester, occurring with a frequency of 0.3-0.5/1,000 births. Severe fetal ventriculomegaly (SVM) may necessitate intrauterine intervention. Most such interventions have been made percutaneously with ultrasound guidance insertion of a pigtail catheter, which sadly often became obstructed or migrated. CASE PRESENTATION: Our case report presents the possibility of ventriculo-amniotic valve implantation (VAVI) by classic hysterotomy in isolated severe fetal hydrocephalus (IVSM) due to aqueductal stenosis. The patient was operated on similarly to open fetal surgery MOMS criteria at 24+4/7 GA, with an initial lateral ventricular dimension of 22.5 mm. A female newborn was delivered by elective cesarean section at 31+1/7 GA due to PPROM (Apgar 10' 8 points, birth weight 1,600 g), required CPAP, and removal of the drainage system due to infection and narrow lateral ventricles. Evans index (EI) gradual increase and clinical symptoms of high-pressure hydrocephalus after 10 days required a ventricle-peritoneal shunt (VPS) implantation. The newborn was discharged home after 28 days with stabile hydrocephalus (EI: 0.59-0.6), in good clinical condition. The 7-year follow-up was complicated by epilepsy, VPS shunt infections, delay in motor and intellectual functions (mild to moderate), and symptoms of atypical autism, the phenotype possibly related to a variant in ZEB2 gene. CONCLUSION: Intrauterine VAVI is a one-step procedure that is effective in draining CFS. The limitations of the method remain complications due to preterm labor and infection of the drainage system.

Presence of tubuloreticular inclusions in ultrastructural studies of renal biopsies in children with lupus nephropathy - one-center preliminary study.

Introduction: Juvenile systemic lupus erythematosus (jSLE) is an autoimmune disease that develops as a result of multi-level immune dysregulation, including the interferon pathway. Nephropathy develops at an early stage and eventually affects 90% of patients. A renal biopsy allows one to classify lupus nephritis and determine the proper treatment. Biopsy assessment should be done not only in a light microscope but also in a transmission electron microscope (TEM). Its usage may reveal the presence of intracellular tubuloreticular inclusions (TRIs), considered as a morphological marker of interferon hyperactivity. Material and methods: Renal biopsies of 10 children with jSLE and nephropathy were analyzed in TEM. The location, structure, and size of TRIs were assessed. Demographic data, nephropathy manifestation, non-renal symptoms, and serological activity of lupus were analyzed. Results: All the patients were female with an average onset at 12.7 years of age and met SLE criteria. Nephropathy manifested with proteinuria (n = 10) and hematuria (n = 6). Glomerular filtration rate (GFR) was normal in all patients. In three children with early disease onset, it manifested with hematological disorders. TRIs were revealed in 7 biopsies, with the highest expression in the youngest children, with peripheral cytopenia, membranous glomerulonephritis, and lupus nephritis. Conclusions: Demonstration of TRIs in renal biopsies of children with juvenile systemic lupus may confirm the diagnosis of lupus nephritis and is a sign of involvement of the interferon pathway at the early stage of the disease.

The role of assessment of renal biopsy in electron microscopy in making a diagnosis of juvenile-onset systemic lupus erythematosus in a 16-year-old female patient with depression and proteinuria - a case report.

The course of juvenile-onset systemic lupus erythematosus may vary, from rapid multiorgan involvement to insidious development mimicking different medical conditions. Depressive disorder in adolescents poses considerable diagnostic difficulties due to the natural tendency to lowered mood in this age group. However, it may also be the manifestation of a systemic disease. We present a case of a 16-year-old female patient without any somatic symptoms in whom severe depression resistant to treatment was the preceding symptom of juvenile-onset systemic lupus erythematosus (jSLE). Because of isolated proteinuria and presence of antinuclear antibodies, renal biopsy was performed. Light microscopy showed only findings characteristic for membranous nephropathy. Examination on electron microscopy showed characteristic tubuloreticular inclusions (TRIs) which were crucial for making the diagnosis of systemic lupus erythematosus. The evaluation of renal biopsy specimens by electron microscopy could be a useful diagnostic step to confirm the diagnosis, especially in difficult cases where the criteria for SLE are not fully met. The association of mental symptoms with systemic lupus erythematosus and other autoimmune disorders is well documented. However, the increasing prevalence of depression in children and adolescents poses a risk of delaying the diagnosis of a systemic disease.

Anorexia nervosa and juvenile lupus erythematosus in a 16-year-old female patient - common disease origin or random coincidence?

Adolescence is a period in which eating disorders and juvenile systemic lupus erythematosus are typically diagnosed. The coexistence of both disorders prompts the search for a common aetiology. In this paper, we present a case of a 16-year-old girl with life-threatening anorexia nervosa followed by clinical and immunological manifestations of systemic lupus erythematosus. The severity of the symptoms of anorexia nervosa resulted in significant delay in proper diagnosis of the concomitant systemic disease which had already been active. The administration of immunosuppressive treatment resulted in decreased lupus activity and resolution of the symptoms of anorexia nervosa.Being affected by one severe and chronic disease does not preclude the coexistence of another disease of different aetiology. However, such coexistence may suggest a common pathophysiology. Many authors have indicated a possible link between anorexia nervosa and many autoimmune disorders. Currently, modern genetic techniques have confirmed a significant correlation between these disorders. This issue needs further investigation and may be helpful in arriving at the final diagnosis in similar cases.

[Prenatal myelomeningocele repair - a chance to improve the quality of life].

Spina bifida (myelomeningocele) is the most prevalent developmental defect of the nervous system. In different regions of the world it affects from 0.3 to 5 per 1000 of living neonates. It is known that the defect develops during the early fetal life however no explicit cause has been found yet. Recently it has been accepted that one of the major factors contributing to spina bifida is deficiency of folic acid in the mother's body before and during pregnancy. The first attempts to cure myelomeningocele were taken in ancient times. More extensive knowledge of aetiology and effects of spina bifida and development of new surgical techniques allowed for management not only of the defect itself but also the consequent complications. Prenatal spina bifida repair has been performed in the USA for 20 years now while European surgeons (Poland and Switzerland) have operated myelomeningocele for 12 years. Comparative analysis carried out to evaluate the patients operated for spina bifida prenatally and postnatally pointed that those after fetal repair demand the use of ventriculoperitoneal shunt for hydrocephalus twice less frequently. Thanks to shortened exposure of the spinal cord and the spinal nerves to the toxic effect of amniotic fluid and reduced exposure to mechanical injuries, prenatal myelomeningocele repair offers better opportunities to improve motor, urinary and alimentary functions, contributing then statistically to higher quality of lives of children with spina bifida.

Prenatal myelomeningocele repair improves urinary continence and reduces the risk of constipation.

AIMS: The aim of the study was to evaluate the lower urinary tract function, frequency UTI, the degree of social urine continence, and the occurrence of constipation in children who underwent prenatal or postnatal surgery for MMC. MATERIALS AND METHODS: Seventy-two patients with MMC were assessed and divided into the prenatal group (36 patients) and the postnatal one (36 patients). All children, regardless the time of operation, received the same treatment after birth. The urodynamic tests, USG, cystourethrography were performed in all patients along with evaluation of the UTI's, social urine continence, and presence of constipation. RESULTS: Urodynamic and imaging studies showed no differences between the test groups. Children from the prenatally operated group showed statistically significant lower number of urinary tract infections, better urine continence, and less frequent constipation. CONCLUSIONS: Prenatal MMC repair ensures statistically significant improvement of the degree of social urinary continence, reducing the risk of urinary infections and constipation. Time of MMC repair does not statistically influence the urodynamic tests results and the urodynamic parameters are not the prognostic elements to assess the social urinary continence possibility in patients with the neurogenic bladder.

Treatment of the dens fractures in children.

The anatomical and biomechanical features of the immature cervical spine make the upper segments at C1-3 especially susceptible to injury. MATERIAL AND METHOD: From 2000 to 2016, 10 patients (3 boys, 7 girls) with C2 dens fractures were treated. The average age of each child was 11 years (3-17). According to the Anderson classification, there were 6 patients with a type III fracture and 4 with a type II. 4 patients were treated conservatively using the Minerva cervical brace for 75 days (66-125) and 6 patients by means of the Halo-Vest for 79 days (64-87) and followed by the Schantz collar for 17 days (2-35). RESULTS: The follow up lasted 78 months (12-180). The NDI (Neck Disability Index) score was calculated for each patient, except for section 8 (driving the car). The scores ranged from 1/45 (2.22%) to 20/45 (44.44%). The mean score for 9 out of the 10 patients (one patient died) was 4.77 (10.61%). CONCLUSION: The C2 dens fracture is a rare injury in children. The classification system of dens fractures developed by Anderson is useful in choosing the mode of treatment of dens fractures.

Reconstructive Options for Pressure Ulcers in Pediatric Patients.

BACKGROUND: Pressure ulcers pose significant challenges in terms of treatment, often exhibiting a low success rate and a propensity for recurrence. Children with neurological impairments such as myelomeningocele and those with spinal injuries are particularly vulnerable to developing pressure ulcers. Despite advancements, achieving successful reconstruction remains a formidable task. Common sites prone to pressure ulcer formation include the sacral and ischial regions, as well as areas over bony prominences. Additionally, pressure ulcers attributable to medical devices facilitating ambulation are observed. While many pressure sores resolve spontaneously, conservative management may prove ineffective for some, especially in cases of stage 3 and 4 ulcers, necessitating surgical intervention. Various surgical techniques are employed for the treatment of decubitus ulcers, yet there exists no universally accepted gold standard for their management. This paper presents our institutional experience in this domain, highlighting differences in surgical approaches, treatment outcomes, complication rates, and long-term follow-up. METHODS: This study involved a retrospective analysis of medical records from 11 children, ranging in age from 10 to 17 years, who presented with extensive pressure ulcers that were unresponsive to conservative treatment measures. Data collection spanned from February 2017 to June 2022. The pressure ulcers affected various anatomical regions, including the ischial area (5/11 patients), sacral region (3/11 patients), lower limb (1/11 patients), elbow (1/11 patients), and perineal area (1/11 patients). Surgical intervention was the chosen approach for all cases, employing techniques such as reconstructive surgery utilizing perforator, pediculated flaps, and locoregional flaps. RESULTS: Eleven patients with sore ulcers (stage 3 and 4) were treated surgically. We present our experience of using surgical methods, including pedicled anterolateral flaps, pedicled gracilis musculocutaneous flaps, propeller flaps and locoregional flaps. In some cases, surgery was performed after 60 days of hospitalization or ten years after ulcer occurrence. We reviewed the length of hospital stay, surgical management and patient satisfaction. Patients were followed up to 5 years post-surgery. All flaps survived except for one flap where partial necrosis was observed. The recurrence rate was 9.01% (1/11). One patient underwent another surgery. The general outcome was satisfactory. CONCLUSIONS: Conclusions: Our findings underscore the efficacy of flap reconstruction surgical techniques in the management of pressure ulcers among pediatric patients. Based on our experience and the outcomes observed, we advocate for considering reconstructive surgery as a viable therapeutic option early in the treatment course, particularly for stage 3 and 4 ulcers. This approach not only addresses the immediate needs of patients but also holds promise for long-term wound healing and prevention of recurrence.

Laparoscopic Uterine Preservation and Vaginal Reconstruction in a 13-Year-Old Girl with Uterus Didelphys, Bilateral Vesicovaginal Fistulas, and Transverse Vaginal Septum.

BACKGROUND: Complex female genital tract malformations account for 1.2% of all female genitourinary malformations. Although exceedingly rare, they can cause severe gynecologic symptoms in young women and lead to fertility problems. CASE: We present the case of a 13-year-old girl with primary amenorrhea referred for cyclic abdominal lower pain and menouria. Detailed diagnostics revealed uterus didelphys, transverse vaginal septum, and bilateral vesicovaginal fistulas. Laparoscopic left hemi-hysterectomy and salpingectomy were performed. The vesicovaginal fistula on the right side was excised, and the proximal vagina was anastomosed with the distal dimple. Since the operation, the patient has been pain-free and menstruating regularly from the right uterus. SUMMARY AND CONCLUSION: Preservation of the uterus should be considered in any case of complex female genital tract malformation and, as successful laparoscopic treatment advocates, a minimally invasive approach is feasible.

Absence of a Hernia Sack in Patients Undergoing Prenatal Repair of Spina Bifida Increases the Risk of Developing Shunt-Dependent Hydrocephalus.

Spina bifida aperta (SBA), with (myelomeningocele) or without (myeloschisis) a hernia sack, is the most common congenital defect of the central nervous system. Prenatal surgical closure of SBA lowers the risk for developing shunt-dependent hydrocephalus, which offers a chance at improved motor, urinary, and gastrointestinal function. A total of 96 patients who had undergone open surgery prenatal repair for SBA were analyzed. The patients were divided into two groups: Group I-12 patients (12.5%)-without a hernia sack (myeloschisis) and Group II-84 patients (87.5%)-with a hernia sack (myelomeningocele). In this study, we demonstrated that prenatal SBA repair was statistically significantly less often associated with the need for ventriculoperitoneal shunting (p > 0.00001). The shunting was statistically significantly more often required in patients from Group I (p > 0.004). The absence of a hernia sack increases the risk for developing shunt-dependent hydrocephalus in patients after prenatal SBA repair. However, as prenatal SBA repair is associated with better motor, urinary, and gastrointestinal function, increased risk of developing shunt-dependent hydrocephalus in fetuses without a hernia sack should not be treated as a contraindication to prenatal intervention.

Neurologic Outcome Comparison between Fetal Open-, Endoscopic- and Neonatal-Intervention Techniques in Spina Bifida Aperta.

INTRODUCTION: In spina bifida aperta (SBA), fetal closure of the myelomeningocele (MMC) can have a neuroprotective effect and improve outcomes. In Europe, surgical MMC closure is offered by fetal-open (OSBAR), fetal-endoscopic (FSBAR), and neonatal (NSBAR) surgical techniques. Pediatric neurologists facing the challenging task of counseling the parents may therefore seek objective outcome comparisons. Until now, such data are hardly available. In SBA, we aimed to compare neurologic outcomes between OSBAR, FSBAR, and NSBAR intervention techniques. METHODS: We determined intervention-related complications, neuromuscular integrity, and neurologic outcome parameters after OSBAR (n = 17) and FSBAR (n = 13) interventions by age- and lesion-matched comparisons with NSBAR-controls. Neurological outcome parameters concerned: shunt dependency, segmental alterations in muscle ultrasound density (reflecting neuromuscular integrity), segmental motor-, sensory- and reflex conditions, and the likelihood of intervention-related gain in ambulation. RESULTS: Compared with NSBAR-controls, fetal intervention is associated with improved neuromuscular tissue integrity, segmental neurological outcomes, reduced shunt dependency, and a higher chance of acquiring ambulation in ≈20% of the operated children. Children with MMC-lesions with a cranial border at L3 revealed the most likely intervention-related motor function gain. The outcome comparison between OSBAR versus FSBAR interventions revealed no significant differences. CONCLUSION: In SBA, OSBAR- and FSBAR-techniques achieved similar neuroprotective results. A randomized controlled trial is helpful in revealing and compare ongoing effects by surgical learning curves.

In utero myelomeningocele repair reduces intensification of inflammatory changes in the dura mater and the skin.

Objective: Chemical and mechanical injury in myelomeningocele (MMC) during the fetal life results in functional disorders of multiple organs. Prenatal MMC repair reduces sequelae of spinal cord injury.Design: Histopathological evaluation of dura mater and skin specimens to assess the severity of inflammatory changes.Setting: Histopathological laboratory and operated patients.Participants: 45 cases (Group I)-intrauterine surgery due to MMC and 42 cases (Group II)-postnatal surgery.Outcome measures: Specimens of the skin and of the dura mater adjacent directly to the uncovered section of the spinal cord were collected for assessment. The specimens were histopathologically evaluated to assess the severity of inflammatory changes.Results: The analysis of the severity of inflammatory changes in the skin and the dura mater showed only small lymphocytic infiltration in 5 fetuses (Group I). Medium and large infiltration in the skin and the dura mater was found in all children who underwent postnatal surgery (Group II). Lymphocytic and granulocytic infiltration in the skin and the dura mater were statistically significantly more prevalent in children who underwent postnatal surgery compared to the group of children who underwent prenatal surgery (P < 0.000003).Conclusions: By reducing the time of exposure to damaging factors, prenatal MMC repair statistically reduces the risk of inflammatory changes in the exposed spinal cord and spinal nerves. Prenatal closure of spina bifida before 24 week of gestation does not reduce the severity of inflammatory changes in the exposed spinal cord.

Ovarian adenocarcinoma in 14-year-old girl.

Ovarian tumors have an uncommon occurrence in children and adolescent girls compared with adult women. The peak incidence of ovarian tumors is between 15-19 years of age. Malignant ovarian tumors in children and adolescent girls are extremely rare. A case of a 14-year-old girl with ovarian mucinous adenocarcinoma that has probably not been described before in the literature is presented below.

Fetoscopic Myelomeningocele Repair with Complete Release of the Tethered Spinal Cord Using a Three-Port Technique: Twelve-Month Follow-Up-A Case Report.

Open spina bifida is one of the most common congenital defects of the central nervous system. Open fetal surgery, which is one of the available therapeutic options, remains the gold standard for prenatal repairs. Fetoscopic closure may lower the number of maternal complications associated with open fetal surgery. Regardless of the approach, the outcome may be compromised by the development of tethered spinal cord (TSC) syndrome. At 24.2 weeks of gestation, a primipara was admitted due to fetal myelomeningocele and was deemed eligible for fetoscopic repair. Fetal surgery was performed at 25.0 weeks of gestation. It was the first complete untethering of the spinal cord and anatomic reconstruction (dura mater, spinal erectors, skin) achieved during a fetoscopic repair of spina bifida. Cesarean section due to placental abruption was performed at 31.1 weeks of gestation. VP shunting, with no need for revision, was performed at 5 weeks postdelivery due to progressing ventriculomegaly. No clinical or radiological signs of secondary tethering were observed. Neurological examination at 11 months postdelivery revealed cranial nerves without any signs of damage, axial hypotonia, decreased muscle tone in the lower extremities, and absent pathological reflexes. Motor development was slightly retarded. Complete untethering of the neural structures should always be performed, regardless of the surgical approach, as it is the only course of action that lowers the risk for developing secondary TSC.

Pulmonary function, exercise capacity and dyspnea in patients 7 ​years after Nuss surgery.

PURPOSE: Pectus excavatum is a frequent thoracic malformation increasingly treated with minimally invasive methods (MIRPE), which are performed for cardio-respiratory problems and in some centers also for esthetic considerations. Theoretically, MIRPE may increase thoracic elastic recoil, work of breathing and cause emphysema. The aim of the present study was to determine whether teenagers who underwent MIRPE may expect normal thoracic cage development, cardio-respiratory function, exercise capacity and asymptomatic functioning. MATERIAL AND METHODS: Fifty five patients (21.1 â€‹± â€‹3.0 years) who underwent MIRPE between 2000 and 2010 were assessed 6.8 (±2.4) years after surgery. Controls were matched for sex, age and height to the intervention participants. Spirometry, body plethysmography, diffusion capacity and the 6 â€‹min walking test (6MWT) were performed. Anteroposterior (AP) and transverse chest diameters were measured. RESULTS: Participants who underwent MIRPE had normal pulmonary function, and exercise capacity. After adjustment for potential confounders, the intervention group had lower mean BMI [-1.88 â€‹± â€‹0.56 (kg/m2); p â€‹= â€‹0.001] and chest AP diameter [-2.79 â€‹± â€‹0.57 (cm); p â€‹< â€‹0.001], but higher residual volume (RV%) [12.98 â€‹± â€‹5.31 (%); p â€‹= â€‹0.001], RV% total lung capacity (TLC) [5.56 â€‹± â€‹0.92 (%); p â€‹< â€‹0.001], forced expiratory volume in 1 â€‹s/forced vital capacity (FEV1/FVC) [2.64 â€‹± â€‹1.28 (%); p â€‹= â€‹0.039] and 6MWT distance [29.10 â€‹± â€‹13.02 (m); p â€‹= â€‹0.025]. CONCLUSIONS: Young adults who undergo MIRPE may expect normal pulmonary function and exercise capacity. Observed differences in air trapping require further assessment in terms of emphysema development risk.

Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome - one clinical center case series and the systematic review of 734 cases.

INTRODUCTION: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare female urogenital tract malformation. STUDY OBJECTIVE: To present 10 patients with OHVIRA treated at the clinical center. To perform a systematic review of OHVIRA case series related to the prevalence of anatomical variants, surgical interventions and endometriosis, and to compare them with our case series. MATERIALS AND METHODS: Medical records from 10 OHVIRA patients treated between 2016 and 2020 were retrospectively reviewed. For the systematic review, PubMed and Web of Science were used to search for relevant studies. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were strictly followed. RESULTS: The most common anatomical variant includes left obstructed hemivagina (50.7%) with isolated hematocolpos or hydrocolpos (55.9%), uterus didelphys (82.9%), and ipsilateral renal agenesis (92.2%). Vaginal septectomy was the most common surgical approach (86.5%). Hemivaginectomy (2.2%), hemihysterectomy (4.2%), or total hysterectomy (0.7%) were also performed in several patients. Some subjects required salpingectomy (3.3%) or oophorectomy (1.8%). 7.5% of patients, mainly infants, did not require surgery due to the spontaneous resolution of hydrocolpos. Endometriosis was fortuitously found in 13.6% of the selected cases who underwent laparoscopy or laparotomy. DISCUSSION: The most common variant of OHVIRA includes isolated hematocolpos and a thick vaginal septum between adjacent hemivaginas. Endometriosis was present in approximately 14% of OHVIRA patients, but this number is probably underestimated. Routine laparoscopy is not required. However, all patients need further monitoring due to a higher risk of endometriosis. Based on the analyzed studies and our case series, vaginal septectomy is a sufficient surgical technique to relieve symptoms and prevent possible complications in most OHVIRA patients.

Early Feminizing Genitoplasty in Girls with Congenital Adrenal Hyperplasia (CAH)-Analysis of Unified Surgical Management.

AIM: To analyze a single-centre experience in feminizing genitoplasty in virilized girls with congenital adrenal hyperplasia (CAH). METHODS: Review of medical records of all 46, XX CAH patients undergoing single stage feminizing genitoplasty between 2003 and 2018 was performed. RESULTS: A total of 31 girls aged from 4 months to 10 years were included in the study. The majority (n = 26/31, 84%) were operated before 2 years of age (median 8 months). External virilization was rated as Prader 3 (n = 7/31), Prader 4 (n = 21/31) and Prader 5 (n = 3/31). The urethrovaginal confluence location was low in 19 and high in 12 girls with a percentage distribution similar in Prader 4 and 5 (p > 0.05) but significantly different in Prader 3 (p = 0.017). The follow-up ranged from 12 months to 15 years. All parents assessed the cosmetic result as satisfactory. Perioperative complications occurred in two patients and included rectal injury (n = 1/31) and prolonged bleeding (n = 1/31). Three patients developed late complications including labial atheromas (n = 2/31) and vaginal stricture requiring surgical dilatation (n = 1/31). Low confluence did not decrease the risk of complications. CONCLUSIONS: Early feminizing genitoplasty in girls with congenital adrenal hyperplasia, irrespective of virilization severity, gives satisfactory cosmetic results and is characterized by low and acceptable surgical risk. Nevertheless, the most important determinant of the effectiveness of such management would be future patients' satisfaction.

Developmental changes in ACLs and semitendinosus tendons dimensions according to age in children.

PURPOSE: Managing anterior cruciate ligament (ACL) injuries in skeletally immature patients remains difficult. The main aim of this study was to retrospectively compile normative data on the cross-sectional area (CSA) of the semitendinosus tendon (ST) and the diameter of the ACL in children and young adults. METHODS: Knee magnetic resonance imaging (MRI) examinations were performed for a 2-year period in 132 patients (83 female and 49 male patients). The mean age was 14.9 years (8-18 years). Measurements of the ST CSA were performed on axial views in greyscale by two independent researchers. The ACL diameter was measured as well. RESULTS: The results show the CSA of the ST was related to age, and its growth was not linear. The highest growth rate of the CSA of the ST occurred at age 12-13 at the level of the femoral growth plate and at the level of the tibial plateau. The growth of the ACL diameter was linear until 18 years of age. CONCLUSIONS: ST growth (measured in CSA increments) is almost complete at the age of 13, even though the growth is not linear. ACL growth measured in diameter increments proceeds linearly from 8 to 18 years of age. MRI is a clinically useful tool for assessing hamstring tendon grafts preoperatively. LEVEL OF EVIDENCE: Level III, diagnostic studies.

Aggressive Angiomyxoma in an 11-Year-Old Boy - Diagnostic and Therapeutic Dilemmas: An Unusual Case Report and Review of the Literature.

Aggressive angiomyxoma (AAM) is a rare tumor with a high risk of local recurrence. Scrotal AAM mimics common pediatric pathologies including hernia or hydrocele. We present 11-year-old boy who underwent macroscopically radical excision of right scrotal AAM. The patient has been already followed up for 29 months utilizing US every 6 months and MRI every 2 years. Residual scrotal mass has been visualized in MRI 3 months after surgery however no further growth was reported. Long term follow up with reliable local imaging is mandatory.