Laparoscopy is a definitive diagnostic method for auto-amputated ovary in infants.

PURPOSE: Antenatal auto-amputation of the ovary is an extremely rare event, and its diagnosis is difficult. We aimed to retrospectively review the cases with antenatal auto-amputation, where the diagnosis was made based on detection of free-floating cyst during surgery. METHODS: Patients diagnosed with auto-amputated ovary during the surgery between 2012 and 2021 were included in the study. The data were reviewed retrospectively. Clinical, radiological, surgical, and histopathological findings were recorded. RESULTS: Eight patients underwent surgery for an abdominal cystic mass. The age range of patients who were operated was from 21 days to 9 months. None of the patients had symptoms, except one patient who had a large cyst and was vomiting. Prenatal ultrasound examination indicated an intra-abdominal cyst in all patients, but auto-amputated ovary diagnosis was not made. Differential postnatal diagnoses included an ovarian cyst, ovarian teratoma, tuba-ovarian torsion, mesenteric lymphatic malformation, and intestinal duplication cyst. Only one patient had an auto-amputated ovary suspicion in computed tomography. Laparoscopic exploration (n: 7) or laparotomy (n: 1) was performed. Histopathologic examination was necrosis and calcification (n: 6), necrosis (n: 1), and serous cystadenoma and necrosis (n: 1). CONCLUSION: We suggest that laparoscopy should be used for diagnosis and treatment of antenatal intra-abdominal cysts that persist postnatally because of diagnostic dilemmas. We recommend in patients diagnosed with auto-amputated ovary that the other ovary should be carefully monitored and followed up in terms of ovarian cyst, due to the possible risk of torsion.

Late effects in patients with sacrococcygeal teratoma: A single center series.

INTRODUCTION: The aim of this study is to evaluate late side effects that affect quality of life in children with sacrococcygeal teratoma (SCT). PATIENTS AND METHODS: The patients with SCT were evaluated retrospectively. The data were expressed by percentage and the subgroups were compared statistically. RESULTS: A total of 40 children with SCT were identified with median age 12 days (range: 1 day-14.6 years), 27 of whom were analyzed in this study with urodynamic data available for 24 and anal manometric evaluations for 20. Chronic constipation with need for laxative was reported in (7/27) 25.9%, fecal incontinence was present in (1/27) 3.7%, and urodynamic abnormalities were reported in (16/24) 66%. Among those with urodynamic abnormalities, low bladder capacity, dyssyergia and neurogenic bladder were observed in (21/24) 87.5% and anticholinergic treatment was applied. Urinary incontinence was present in (2/27) 7%, with clean intermittent catheterization utilized in (7/27) 25.9%. While defecation was observed more in the patients with Altman types II, III, and IV, micturation problems were observed more in the patients with Altman types II and IV. It was found that urodynamic dysfunctions were more frequent in the patients with increased number of operations. DISCUSSION: Although the rate of symptomatic patients was low, abnormalities determined by radiological and urodynamic evaluations were high.

Complete Penile Duplication with Structurally Normal Penises: A Case Report

BACKGROUND: Diphallia is a very rare anomaly and seen once in every 5.5 million live births. True diphallia with normal penile structures is extremely rare. Surgical management for patients with complete penile duplication without any penile or urethral pathology is challenging. CASE REPORT: A 4-year-old boy presented with diphallia. Initial physical examination revealed first physical examination revealed complete penile duplication, urine flow from both penises, meconium flow from right urethra, and anal atresia. Further evaluations showed double colon and rectum, double bladder, and large recto-vesical fistula. Two cavernous bodies and one spongious body were detected in each penile body. Surgical treatment plan consisted of right total penectomy and end-to-side urethra-urethrostomy. No postoperative complications and no voiding dysfunction were detected during the 18 months follow-up. CONCLUSION: Penile duplication is a rare anomaly, which presents differently in each patient. Because of this, the treatment should be individualized and end-to-side urethra-urethrostomy may be an alternative to removing posterior urethra. This approach eliminates the risk of damaging prostate gland and sphincter.

Pediatric patients with pheochromocytoma: Experience of a tertiary health center.

BACKGROUND: The aim of this retrospective study was to investigate pheochromocytoma (pheo), which is a rare endocrine tumor in the pediatric population. METHODS: The medical records of five children with pheo were studied. The age, gender, clinical presentation, family history, physical findings, coexisting pathology, laboratory evaluation, surgical treatment, and postoperative course were investigated. RESULTS: The patients were four girls and one boy with a mean age of 13.2 years (range, 9.57-15.95 years). None of the patients had paroxysmal hypertension and one had normal blood pressure. No malign pheo was identified. Mean height and weight standard deviation scores (SDS), body mass index (BMI), and BMI SDS were -0.24, 0.04, 20.9 kg/m(2), and 0.20 at the time of diagnosis, and 0.03, 0.43, 23.8 kg/m(2) and 0.49 1 year after operation, respectively. BMI increased significantly after operation. Three patients had normal epinephrine and metanephrine, but elevated norepinephrine and normetanephrine on 24 h urine. Vanillylmandelic acid on 24 h urine sample was elevated in all patients. Ultrasonography failed to visualize tumors in two patients with bilateral pheo. One patient had postoperative severe hypotension. Insulin resistance associated with severe acanthosis nigricans observed in one patient regressed postoperatively. CONCLUSIONS: Pheo in children may present with different symptoms and findings. Decreased catecholamine in the postoperative period may lead to weight gain.

Hyperprostaglandin E syndrome: use of indomethacin and steroid, and death due to necrotizing enterocolitis and sepsis.

Hyperprostaglandin E syndrome (HPS) is the antenatal variant of Bartter syndrome and characterized by polyhydramnios and preterm delivery in the antenatal period and salt-wasting, isosthenuric or hyposthenuric polyuria, hypercalciuria and nephrocalcinosis in the postnatal period. We report a one-month-old infant with HPS with a 15-year-old sister with Bartter syndrome. The infant's birth weight was 2750 g and she had severe dehydration on the 2nd day of life. She had hypercalcemia, hyponatremia, hypokalemia, metabolic alkalosis and elevated plasma renin and aldosterone levels. We instituted indomethacin therapy accompanied by steroid therapy for hypercalcemia. However, the patient developed abdominal distention on the 30th day, which was due to diffuse pneumatosis in sigmoid colon revealed by a subsequent surgical intervention. Following surgery, the patient developed fever, electrolyte abnormalities and subsequently sepsis. The patient died due to sepsis 10 days after surgery. We conclude that indomethacin and steroid therapy must be used cautiously in infants with HPS.

Hepatic P-glycoprotein changes with total parenteral nutrition administration.

BACKGROUND: The mechanism(s) responsible for the development of parenteral nutrition-associated liver disease (PNALD) is unknown. Recently, a number of bile canalicular transport proteins have been identified that transport bile components out of hepatocytes. One group of these genes, multidrug resistance 1 (mdr1) and mdr2, encode P-glycoproteins. Mice lacking mdr2 expression develop liver disease that appears similar to PNALD. This study investigated the alteration in the expression of these transport proteins during the administration of total parenteral nutrition (TPN). METHODS: Mice received either physiologic saline and standard chow or TPN. Mice were sacrificed on day 7, and hepatic DNA and RNA content, mRNA expression, and levels of mdr1 and mdr2 proteins were measured. RESULTS: TPN administration led to a significant (p < .05) decline in mdr2 mRNA expression and an increase in mdr1 mRNA expression. Mdr2 protein expression declined by 66% in the TPN-treated group, and mdr1 protein expression significantly increased by 58%. Histology and biochemical parameters showed no evidence of liver injury. Serum bile acid levels were elevated in the TPN group, suggesting the development of early cholestasis. CONCLUSIONS: The decline in mdr2 and rise in mdr1 mRNA and protein expression with TPN administration occurred before the development of liver injury but during an early state of cholestasis. This suggests that alterations in mdr gene expression may be a causative factor in the development of PNALD.

Diagnosis of orotracheal aspiration using radionuclide salivagram.

There are two main Nuclear Medicine techniques, the gastroesophageal reflux scintigraphy with late lung imaging and the nuclear salivagram, for diagnosis of pulmonary aspiration. Each of the techniques can document the two different, antegrade and retrograde, routes of pulmonary aspiration. In this report, we presented a patient with recurrent respiratory problems and emphasized the importance of concomitant use of the two techniques in the radionuclide diagnosis of aspiration.

Interferon-gamma expression by intraepithelial lymphocytes results in a loss of epithelial barrier function in a mouse model of total parenteral nutrition.

OBJECTIVE: To determine the etiology of the loss of epithelial barrier function observed with the administration of total parenteral nutrition (TPN) in a mouse model. SUMMARY BACKGROUND DATA: Removal of enteral nutrition with the administration of TPN is associated with a loss of intestinal epithelial barrier function. The etiology of this barrier loss is not clear. Because intraepithelial lymphocytes (IELs) produce a number of cytokines that may alter epithelial permeability, the authors investigated IEL cytokine expression in a mouse model of TPN. METHODS: Adult C57BL/6 mice received TPN or enteral diet for 7 days. IELs were subsequently harvested and the mRNA expression of cytokines was measured. Epithelial barrier function was assessed in vitro with 51Cr-EDTA in Ussing chambers and was expressed as the permeability coefficient (Papp). RESULTS: IEL mRNA expression of interferon-gamma (IFN-gamma) rose from 0.14 +/- 0.07 in the control (enterally fed) group to 0.44 +/- 0.11 attomoles/microL in the TPN group (P <.05). Transforming growth factor-beta1 declined slightly but not significantly, from 0.75 +/- 0.35 to 0.55 +/- 0.18 attomoles/microL in the control and TPN groups, respectively. Epithelial barrier function declined significantly with TPN compared to controls. To assess the relevance of IFN-gamma changes, permeability in IFN-gamma knockout mice was studied. Barrier function was significantly higher in IFN-gamma knockout mice on TPN compared to C57BL/6 mice that received TPN. CONCLUSIONS: IEL cytokine expression changes significantly with TPN administration. The partial correction with IFN-gamma knockout mice suggests that an upregulation of IFN-gamma expression is one mechanism responsible for the loss of the epithelial barrier associated with TPN.

Total parenteral nutrition-associated changes in mouse intestinal intraepithelial lymphocytes.

Intraepithelial lymphocytes (IEL) play a major role in mucosal defense mechanisms against intraluminal foreign antigens. To address the role luminal nutrients have on the phenotype and function of the IEL, we administered total parenteral nutrition (TPN) to mice, with the absence of enteral intake. We hypothesized that administration of TPN would result in changes in the phenotype and function of the IEL. For this, we utilized a mouse model of TPN. A significant decline in the CD4+ IEL population occurred with TPN. Additionally, the CD8+,CD44+ IEL subset showed a 65% decline (P < 0.05), and the CD4+,CD44+ subset declined by 55% with TPN (P < 0.05). The CD8alphabeta+ population (a marker of thymic-dependence) also declined by 92% (P < 0.01) with TPN. IEL in the TPN group showed a significantly lower degree of in vitro proliferation. In conclusion, the IEL showed significant phenotypic changes with TPN including the loss of the thymic-derived population. Functionally, the IEL showed a significant decline in proliferation. Such changes demonstrate the important role luminal nutrients have on IEL phenotype and function.