Low-Grade Injury following Testicular Torsion: A Multicenter Study Confirming a Disturbing Possibility.

INTRODUCTION: There is an ongoing debate whether to perform orchiectomy or orchidopexy following testicular torsion (TT) in cases where the testis seems non-viable. The main problem is lack of objective criteria defining testicular viability. The aim of this study was to investigate the grade of injury in orchiectomy specimens obtained from cases of TT and its association with clinical findings. METHODS: This multicenter retrospective study involved double-blinded reassessment of the patient files and the pathological specimens using Mikuz classification to analyze the relation between clinical and pathological findings. RESULTS: A total of 289 patient charts from 14 centers were reviewed and 228 were included in this study. Twenty (8.8%) patients had grade 1 injury which refers to reversible injury. The clinical findings of these 20 patients were compared to 208 patients with higher grades of injury. As expected, there was statistically significant difference regarding duration of symptoms (p < 0.001); however, range was wide in both groups (as long as 96 h for grade 1 and as short as 7 h for higher grades). There was no statistically significant difference in any other variable including age (median 14 for both, p = 0.531), symptoms (pain: 19/20 vs. 189/202, p = 0.801; swelling: 13/19 vs. 168/197, p = 0.094), absence of blood flow in Doppler US (15/19 vs. 164/197, p = 0.635), or degree of torsion (median 720° for both, p = 0.172). CONCLUSION: Our study revealed necessity for better criteria to define viability of testis following TT. Histopathological injury appeared to be reversible even in some patients with more severe perioperative findings, late admission, or high degree of twisting. Our findings support the tendency for testicular fixation instead of orchiectomy as none of the clinical or perioperative findings could be attributed to high-grade injury.

Case Report: Severe McCune-Albright syndrome presenting with neonatal Cushing syndrome: navigating through clinical obstacles.

Background: Café-au-lait skin macules, Cushing syndrome (CS), hyperthyroidism, and liver and cardiac dysfunction are presenting features of neonatal McCune-Albright syndrome (MAS), CS being the rarest endocrine feature. Although spontaneous resolution of hypercortisolism has been reported, outcome is usually unfavorable. While a unified approach to diagnosis, treatment, and follow-up is lacking, herein successful treatment and long-term follow-up of a rare case is presented. Clinical case: An 11-day-old girl born small for gestational age presented with deterioration of well-being and weight loss. Large hyperpigmented macules on the trunk, hypertension, hyponatremia, hyperglycemia, and elevated liver enzymes were noted. ACTH-independent CS due to MAS was diagnosed. Although metyrapone (300 mg/m2/day) was started on the 25th day, complete remission could not be achieved despite increasing the dose up to 1,850 mg/m2/day. At 9 months, right total and left three-quarters adrenalectomy was performed. Cortisol decreased substantially, ACTH remained suppressed, rapid tapering of hydrocortisone to physiological dose was not tolerated, and supraphysiological doses were required for 2 months. GNAS analysis from the adrenal tissue showed a pathogenic heterozygous mutation. During 34 months of follow-up, in addition to CS due to MAS, fibrous dysplasia, hypophosphatemic rickets, and peripheral precocious puberty were detected. She is still regularly screened for other endocrinopathies. Conclusion: Neonatal CS due to MAS is extremely rare. Although there is no specific guideline for diagnosis, treatment, or follow-up, addressing side effects and identifying treatment outcomes will improve quality of life and survival.

Favourable outcomes of conservatively managed acute abdomen associated with multisystem inflammatory syndrome in children during the COVID-19 pandemic.

BACKGROUNDS: Abdominal pain is one of the most common symptoms of multisystem inflammatory syndrome in children (MIS-C). Abdominal pain can vary from mild to severe and may present as acute abdomen. Severe abdominal pain in patients with MIS-C should be differentiated from surgical causes of acute abdomen to prevent unnecessary surgery. METHODS: The diagnosis of MIS-C was based on WHO and CDC recommended criteria. Records of children treated for MIS-C between September 2020 and January 2021 were reviewed retrospectively. RESULTS: In a short time, we encountered seven patients who were diagnosed with MIS-C and showed acute abdomen findings. There were four male and three female patients. The median age was 9 years. Fever, abdominal pain and distension were present in all. The median duration of symptoms was 4 days. Five patients had general abdominal tenderness mimicking acute abdomen. Three patients had right lower quadrant tenderness mimicking acute appendicitis. After the initiation of immunomodulatory therapy and antibiotics, the physical examination findings were improved step by step in all. The median time to initiate oral feeding was 2 days. The median length of hospitalization time was 8 days. CONCLUSION: Serial abdominal examinations performed by the same surgeon enabled us to follow these patients conservatively and thus avoid unnecessary surgical intervention.

Lung ultrasound findings in children with foreign body aspiration.

OBJECTIVES: Foreign body aspiration (FBA) has a wide clinical spectrum, patients may be asymptomatic or present with cardiopulmonary arrest. Radiological imaging methods are used in addition to history and physical examination findings for certain diagnosis. Lung ultrasonography (LUS), whose usage area is increasing year by year, can be included in these. This study aimed to investigate ultrasonography findings in FBA and hypothesized that LUS may have a standard place in FBA diagnosis. METHODS: Patients who administered to the pediatric emergency department between August 2019-August 2021, considered according to the clinical findings and physical examination possible FBA, and who undergone rigid bronchoscopy by pediatric surgery were included in the study. RESULTS: Thirty-two patients were included in the study. According to the results of bronchoscopy, FBA was detected in 25 patients. The most common finding in radiography was hyperinflation on one side, while LUS findings were confluent-B lines in eight patients, barcode-sign in five patients, pleural line abnormalities in two patients, and pleural consolidation in two patients. CONCLUSION: This study is the first study aiming to evaluate LUS findings in FBA to the best of our knowledge. The B-lines, barcode sign, pleural line abnormalities and consolidation are seen findings in LUS of the patients with FBA. Although it is far from replacing chest radiography in these patients, it is possible to say that it has an equivalent value with chest radiography.

Early postoperative complications in pediatric abdominal solid tumor surgery according to Clavian-Dindo classification.

PURPOSE: Postoperative period after abdominal solid tumor surgery is critical regarding complications. This study aimed to detect incidence and treatment of complications. METHODS: Single center retrospective study including years 2010-2019 for early postoperative complications were documented and graded according to Clavian-Dindo classification. RESULTS: The overall complication rate was 10% and they were chylous leak (n = 8, 2%), collection in surgical field (n = 8, 2%), acute renal failure (n = 7, 1.7%), hemorrhage (n = 5, 1.3%), intestinal obstruction (n = 4, 1%), surgical site infection (n = 3, 0.7%), thromboembolism (n = 3, 0.7%) and peripheric neuronal event (n = 2, 0.5%). Distribution of complications according to Clavian-Dindo classification was as follows: 2 grade I, 23 grade II, 9 grade IIIb, 5 grade IVa and 1 grade V. While age and percentage of benign or malignant disease were not different in groups with or without complications (p = 0.11, p = 0.24), males had more complications than female patients (p = 0.008). Having more than one surgery aiming tumor resection was associated with increased postoperative complications (p = 0.002). Incidence of complications were highest after extragonadal germ cell tumor (35%) and hepatic tumors (29%). CONCLUSIONS: Postoperative complications of abdominal tumor surgery are diverse in children. They are related with prolonged hospital stay and need for medical or surgical interventions. Number of surgeries, organ of origin and tumor type have an influence on risk of complications.

An unusual complication of tumor surgery: chylous leakage.

PURPOSE: This study was designed to evaluate the effectiveness of conservative treatment for chylous leak after tumor surgery and to propose a management algorithm. METHODS: The data of patients with postoperative chylous leak after tumor surgery in our institution between 2010 and 2019 were retrospectively reviewed. In this study, 469 laparotomies, 89 thoracotomies, and 57 cervical excisions were performed for tumor surgery in our institution. RESULTS: Twelve patients with a median age of 4 (IQR, 3-8) years had postoperative chylous leak. All patients received total parenteral nutrition for a median of 13 days. Five patients had intravenous somatostatin for a median of 14 days (IQR, 9-16) to decrease chyle production. Eventually, chylous leak ceased in all patients with conservative treatment and surgical drains were removed after no leak was observed with enteral feeding. CONCLUSIONS: The incidence of chylous leak in childhood tumor surgery is approximately 2%. Extended tumor resection and lymph node dissection lead to the injury of the delicate structures that drain chyle. Conservative treatment with total parenteral nutrition and somatostatin seems to be effective. In particular, somatostatin may be used in resistant cases. Conservative treatment can take up to 1 month. The algorithm consists of how to manage postoperative chylous leak in childhood.

Reconstruction of congenital rectovestibular fistula in an adult. Case report.

Anorectal malformations are common congenital anomalies but diagnosis and treatment in adulthood are quite rare. Treatment during adulthood may be challenging due to anatomic and physiologic changes. Posterior sagittal anorectoplasty may provide good cosmetic and functional results even in adult patients. KEY WORDS: Congenital malformations, Rectovaginal fistula, Rectovestibular fistula.

Extraovarian juvenile granulosa cell tumor in a prepubertal child: novel location of a rare tumor.

Juvenile granulosa cell tumor (JGCT) is the most common type of sex cord stromal tumor arising from gonadal structures of children and young adults. We present a 3.5-year-old girl with JGCT located in retroperitoneum without ovarian involvement. Extragonadal occurrences of other sex cord stromal tumors have been rarely reported, but this is the first case of JGCT in an extragonadal location. We speculate the possible underlying mechanism of sex cord stromal tumor formation in extragonadal locations. Furthermore, clinical presentation, differential diagnosis and management of this tumor in childhood are discussed.

The effect of hypothermia in a rat testicular torsion/detorsion model.

BACKGROUND: Testicular torsion is an emergent condition. The protective effect of medical hypothermia in ischemia/reperfusion injury is well defined. OBJECTIVES: To evaluate the late results of hypothermia through a rat testicular torsion/detorsion model compatible with human testicular torsion. STUDY DESIGN: Rats were divided into 5 groups (n = 7): (1)Sham (S) group, (2)T/D group: right testis was torted for 1-h, (3)T/D + H30 group: hypothermia at 4 °C was applied for 30 min before detorsion, (4)T/D + H90 group: hypothermia at 4 °C was applied for a total of 90 min (30 min before and 1-h after detorsion), (5)H group: hypothermia at 4 °C was applied to right testis for 90 min. Testicular diameters at preoperative period and 8th postoperative week were measured. Biochemically, MPO, NO, 3-NT and 4-HNE in testicular tissue and serum levels of NO, PGF 2α, 3-NT, 8-OHdG and 4-HNE were studied. Histopathologic examination and TUNEL assay were also performed. RESULTS: Biochemical and macroscopical parameters of both T/D + H30 and T/D + H90 groups were statistically different from group T/D with respect to protective effects of hypothermia. Johnsen's score was also statistically different in group T/D + H90. DISCUSSION: Hypothermia can easily be applied with ice bags both in perioperative period. This is the first study evaluating the effect of hypothermia applied postoperatively. Tissue level of protein oxidation marker (3-NT) and serum levels of DNA damage (8-OHdG), lipid peroxidation (4-HNE), protein oxidation (3-NT) and oxidative stress (PGF-2α) markers were measured for the first time. CONCLUSIONS: Hypothermia has been shown to be macroscopically, biochemically and histopathologically beneficial in the long-term experimental testicular torsion model.

Approach to pheochromocytoma and paraganglioma in children and adolescents: A retrospective clinical study from a tertiary care center.

AIM: Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors in childhood. They are catecholamine secreting tumors and present with signs or symptoms related to their excess. Most common signs and symptoms are hypertension, headache and diaphoresis. The management of children usually depend on experience of adulthood. This study is conducted to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care center. MATERIAL AND METHODS: We reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively. RESULTS: There were 18 children operated for PCC and PGL in the study period. The female to male ratio was 1:1. The median age at diagnosis was 13 (IQR, 9-15) years. The most common presenting symptoms were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumors of two patients with VHL were detected during routine follow-up. Three patients had multifocal disease. Medical preparation for surgery was carried out in all patients. Antihypertensive treatments were administered preoperatively. Since the patients are at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction, high salt diet was recommended. Intravenous normal saline at a rate of 3000 ml/m2 body surface area per day was started for intravascular volume expansion preoperatively. The mean duration for preoperative medication to achieve normal blood pressure was 22 days (range, 16-30). Twenty-five tumors were excised in eighteen patients. One patient who had bone metastases on diagnosis and is on I131MIBG therapy. The median follow-up time was 5.6 years (range, 1 months - 21 years). Five patients reached adulthood during the study period. Four of these had recurrent metastases (n = 2) and new tumors (pancreatic neuroendocrine tumor, n = 1 and pancreatic neuroendocrine tumor and renal cell carcinoma, n = 1) after the age of 18. CONCLUSION: Multidisciplinary approach is necessary to achieve safe surgical treatment and surveillance of PCC and PGL. Detection of associated familial cancer susceptibility syndromes and long-term follow-up is essential to detect late recurrences and new tumors.

Common pediatric surgical diseases of refugee children: health around warzone.

PURPOSE: We aim to evaluate the sociodemographic and medical features of child-aged refugee patients and identify their health-related problems. METHODS: Refugee children admitted to pediatric surgery department of a teaching hospital during the years 2012-2017 were included. Patients' files were reviewed retrospectively for sociodemographic and medical features. RESULTS: A total of 254 patients with the mean age of 4.6 ± 4.15 years (0-16 years) were treated. Male-to-female ratio was 1.7. Most common diagnosis were inguino-scrotal pathologies (n = 50, 19.7%) followed by foreign body ingestion (n = 37, 14.6%) and corrosive esophagitis (n = 22, 8.7%). The cause of admission was a potentially preventable trauma in 24.4% of cases. Comorbid medical conditions were present in 49 patients (19.3%). Anemia was detected in 23.2% of cases. Weight according to age and gender were < 3 percentile in 29.1% of patients. Difficulties in communication, lack of former medical history and advanced presentation of disease were the challenges faced by caregivers. CONCLUSION: The primary diagnoses for admission of refugee children were different from the routine practice and a significant part were from preventable causes. Comorbidities were common potentially having a negative influence on treatment processes. This can be a result of unfavorable living conditions and lack of medical care during migration.

Clinical Presentation, Prognostic Factors, and Outcome in Neutropenic Enteropathy of Childhood Leukemia.

Leukemia patients are at risk for neutropenic enteropathy (NEP) because of the effects of intensified chemotherapy. Medical records of 18 patients having 20 episodes of NEP were reviewed retrospectively. Primary diagnosis was acute lymphoblastic leukemia in 12 and myeloblastic leukemia in 6 cases. According to prognosis, 3 patients were in the standard-risk group, 6 in the moderate-risk group, and 9 in the high-risk group. Ultrasonography detected increased bowel wall thickness in 6 patients. Abdominal x-ray revealed air-fluid levels (n=8), pneumatosis intestinalis, pneumoperitoneum (n=1), and portal venous gas (n=1). All patients received medical treatment, and 1 with unrelieved hematochezia required resection of the cecum. Two cases with appendicitis and another 1 with pneumoperitoneum responded to antibiotics and recovered without surgery. The mortality rate was 30% and related to sepsis-induced complications. The presence of hypokalemia, hypoalbuminemia, metabolic acidosis, and admission to the intensive care unit were more common in patients with mortality (P=0.01). In conclusion, NEP should be kept in mind as a treatable but potentially lethal complication of childhood leukemia. Radiologic findings should be interpreted in conjunction with clinical picture. A conservative approach should be used in all cases but surgery can be considered in some situations.

Posterior sagittal anorectoplasty in vestibular fistula: with or without colostomy.

PURPOSE: The aim of this study is to compare the results and complications of one- and three-stage repairs in females with vestibular fistula (VF) and make contribution to the discussion of whether the disadvantages outweigh the protective effect of a colostomy from wound infection and wound dehiscence following posterior sagittal anorectoplasty (PSARP). METHODS: Patients with a diagnosis of VF who underwent PSARP between October 2009 and November 2015 were retrospectively reviewed. The patients were divided into two groups: Group 1-patients treated by one-stage procedure (n = 30); Group 2-patients treated by three-stage procedure (n = 16). RESULTS: There were no statistically significant differences between the groups with respect to wound infection, recurrence of fistula and rectal mucosal prolapse. Minor wound dehiscence occurred slightly more common in Group 1, even if p value is not significant. No wound dehiscence has been observed since we switched to the protocol of keeping the child nil per oral for 5 postoperative days and loperamide (0.1 mg/kg) administration for 7 postoperative days. The mean time before resuming oral intake was 2.87 ± 1.7 and 1.19 ± 0.4 days in Group 1 and Group 2, respectively (p = 0.001). None developed major wound disruption or anal stenosis in either group. There were no statistical differences between the groups in terms of voluntary bowel movements, soiling and constipation. CONCLUSIONS: PSARP performed without a protective colostomy in patients with VF has low morbidity, good continence rates and obvious advantages for both the patients and their parents.

Congenital anterior urethrocutaneous fistula: 3 new cases and review of literature.

OBJECTIVE: Congenital anterior urethrocutaneous fistula, is a rare anomaly characterised by fistulisation of penile urethra to skin and presence of a concomitant normal or hypospadiac external urethral meatus. It may be seen as an isolated anomaly or may accompany genitourinary or anorectal malformations. We aim to present 3 new cases and define the common properties of patients stated in literature. METHOD: Information of 3 patients aged 2, 3 and 6 with this diagnosis were reviewed retrospectively and features of 51 patients in 25 articles with literature search. RESULTS: From the patients we operated, 2 had midpenile and 1 had subcoronal fistula. Urethral meatus was at tip of glans in all with 1 stenotic meatus. Two-layered primary repair was performed in 3 patients and deep ventral incision on urethral plate with meatotomy were added to fistula repair in one with stenotic meatus. Fistula recurred in this patient but resolved spontaneously after dilatations. In literature, most common fistula site was subcoronal in 27 (52.9%). Hypospadias was in 11.8% and associated genitourinary anomaly was detected in 21.5% of patients. Fistula recurrence ratio was 7.8% using different surgical techniques. CONCLUSION: Congenital anterior urethrocutaneous fistula is frequently located in subcoronal level and usually a normal urethra distal to it. Physical examination is important to detect additional anomalies. Success rates are high with primary repair techniques.

The comparison of dextranomer/hyaluronic acid and polyacrylate-polyalcohol copolymers in endoscopic treatment of vesicoureteral reflux.

BACKGROUND: Dextranomer/hyaluronic acid (Dx/Ha;Dexell®) and polyacrylate-polyalcohol copolymer (PPC;Vantris®) are the popular tissue-augmenting substances using for the endoscopic injections of vesicoureteral reflux (VUR). The aim of the study is to evaluate and compare Dx/Ha and PPC in terms of effectiveness, injection techniques and complications with special emphasis on vesicoureteral junction obstruction (VUJO). METHODS: A total of 95 patients who underwent endoscopic VUR treatment between 2009 and 2015 were retrospectively reviewed. The patients were divided into two groups: group 1: Patients underwent endoscopic treatment with PPC (n=50 patients, 70 renal refluxing units) group 2: Patients underwent endoscopic treatment with Dx/Ha (n=45 patients, 74 renal refluxing units). RESULTS: The overall resolution rates based on the number of renal refluxing units studied was 88.6% and 70.3% in group 1 and group 2, respectively. Resolution rates were significantly better in group 1 compared to group 2. VUJO requiring ureteral reimplantation or stent insertion developed in 7 patients in group 1. No VUJO was observed in group 2. VUJO in group 1 was markedly higher than that in group 2. CONCLUSIONS: Endoscopic treatment of VUR with PPC promises better resolution rates but higher VUJO rates compared to Dx/Ha.

Castleman disease: an unusual diagnosis of a portal mass in an 8-year-old boy.

An 8-year-old boy presented with a mass located in the portal hilum and hepatosplenomegaly, and the presumed initial diagnosis was lymphoma. The pathology result was Castleman disease of hyaline vascular type. Castleman disease is an unusual diagnosis that should be kept in mind in the differential diagnosis of portal masses. In the case of solitary lesions, total excision can be curative.