This study aims to evaluate the current surgical approach to adrenal masses in the pediatric age group. We retrospectively analyzed cases that underwent surgery for adrenal masses between 2007 and 2017. Patients were assessed regarding age, sex, primary diagnosis, image defined risk factors (IDRF), surgical treatment method, complications, duration of hospital stay, and follow-up. We examined 50 patients who underwent surgery for adrenal mass (mean age: 4.8 years; range: 5 days-14 years). For IDRF assessment, Ultrasonography was used in 42, Computed Tomography in 36, and Magnetic Resonance Imaging in 36 patients. Lesions were present on the right in 25, left in 21, and bilateral in 4 patients. Histopathological findings were neuroblastoma (n = 29), ganglioneuroma (n = 6), adrenal cortex tumor (n = 5), ganglioneuroblastoma (n = 4), pheochromocytoma (n = 3), cyst (n = 1), and adrenal hematoma (n = 2). Laparotomy was performed on 37 patients, and laparoscopy on 13 patients. None of the cases had any operative complications. The crucial factors determining the surgical approach to adrenal masses in pediatric cases are the histopathology of the mass, volume, and IDRF. Minimally invasive procedures could be reliably performed in appropriate cases.
5 September 2015 in Journal of Clinical Research in Pediatric Endocrinology
Testotoxicosis is a rare disorder which presents as isosexual peripheral precocious puberty in males. Despite the pattern of autosomal dominant inheritance, sporadic cases also may occur. Due to activating mutation in luteinizing hormone (LH))/choriogonadotropin receptor (LHCGR) gene, early virilization and advancement in bone age are common with increased serum testosterone levels above adult ranges, despite low LH and follicular-stimulating hormone (FSH) levels. There are different treatment regimens, such as combination of bicalutamide (antiandrogen agent) and a third-generation aromatase inhibitor, that are reported to be well-tolerated and successful in slowing bone age advancement and preventing progression of virilization. We report here two patients who presented with peripheral precocious puberty and an activating mutation in the LHCGR gene: one with a family history and previously determined mutation and the other without family history and with a novel mutation (c.830G>T). Combination of bicalutamide+anastrozole was ineffective in slowing pubertal progression and bone age. Short-term results were better with ketoconazole.
To evaluate the outcome and characteristics of patients who were operated for sacrococcygeal pilonidal sinus (SPS) treatment using primary repair or secondary healing technique. Forty-seven patients (female: 25, male: 22) diagnosed with pilonidal sinus operated between 2009 and 2015 were retrospectively analyzed. The cases were evaluated for age, gender, body mass index (BMI), surgical technique, hospital stay, recovery time and recurrence. Mainly two types of surgical techniques were applied: primary repair and secondary healing. Mean age of the patients was 15.6 (±1.2) years. Excision and primary repair was performed in 36, excision and secondary healing was in 11. There was no statistically significant difference between the groups regarding length of hospital stay and duration of postoperative pain (p > 0.05). There was a statistically significant difference between groups regarding recovery time (p < 0.05). Recurrence was detected in a total of 11 (23.4%) patients. We did not find any significant difference concerning length of hospitalization, duration of postoperative pain and recurrence rate. However we determined that the primary repair group recovery time is significantly shorter compared to our secondary healing group. Therefore we recommended primary repair in the treatment of primary repair PS in children.
23 September 2014 in Turkish Archives of Pediatrics
Afibrinogenemia is a rare bleeding disorder which is observed with an incidence of 1:1 000 000. It is an autosomal recessive disease and occurs as a result of mutation in one of the three genes which code the three polypeptide chains of fibrinogen. Basic clinical findings include spontaneous bleeding, bleeding after minor trauma or due to surgery. Splenic rupture in afibrinogenemia has been reported only in 6 cases so far. In this article, we present a 15-year old congenital afibrinogenemia patient with spontaneous splenic rupture.