Pituitary adenoma in monozigotic twins with Cri du Chat syndome: A rare case report

Pituitary adenomas are rare tumours of pediatric population. In etiology, genetic factors are more common than they are in adults. Because of the rarity of the cases, there are only a few large case studies in the literature. Pituitary tumours in children are often related with syndromes like MEN type 1, Carney Complex and Mc Cune Albright, but there is no case in the literature associated with Cri Du Chat syndrome. Statisticlly, it has been reported that, pediatric tumours occur more often in twins, in the pediatric population. Main treatment for prolactinomas is medical intervention with dopamine agonists, as in adults. Surgery is prefered when the tumour is resistant to medical treatment or shows mass effects around sellae. In that situation, as in adults, both transcranial and transsphenoidal approach is possible

Statistical analysis of associated vertebra and costal anomalies in spina bifida patients

Objective: Spina bifida is one of the most severe birth defects and can happen as a result of disrupted primary neurulation. Congenital vertebra and costa anomalies are more frequently seen with spina bifida, and associated anomalies significantly affect the prognosis of affected children. In this study, we aimed to determine the incidence of scoliosis, costal anomalies, and vertebral deformations seen at the time of diagnosis and to statistically evaluate their concomitancies. Methods: Gender and mean ages of the patients were determined. The spina bifida patients were examined for deformation anomalies, butterfly vertebra, hemivertebra, wedge vertebra, costal anomalies and scoliosis. The relationships between these anomalies were evaluated. Results: 94 patients with a mean age of 11,5 months examined. The incidence of scoliosis was 21.8% among female infants and 17.9% among males. Rates of scoliosis with vertebra anomalies (hemivertebra, wedge vertebra) and costal anomalies did not differ significantly (P > 0.05). Wedge vertebra were the most frequent vertebra anomaly type with 38.2% ratio. Costal anomalies were detected in 25.5% of females and 20.5% of male infants. Hemivertebra and wedge vertebra were seen significantly more frequently in this group. Gender distribution did not differ between with and without any vertebra types. Conclusion: Congenital vertebra and costa anomalies are more frequently seen with spina bifida. We believe that these anomalies and relationship with spina bifida may demonstrate differences among different ethnic groups or locations. More detailed multi-centered studies performed on this issue will aid in the determination of etiologies, genetics, and treatment principles of these congenital anomalies

Factors Affecting the Outcome in Traumatic Subarachnoid Hemorrhage

Objective: To define risk factors affecting the outcome in traumatic subarachnoid hemorrhage.Material and Methods: Forty-four patients with traumatic subarachnoid hemorrhage were evaluated retrospectively. They were divided into three groups according to their age: elderly (≥65 years), adult (16- 64 years), and children (<16 years). The clinical picture on admission was evaluated using the Glasgow Coma Scale. The patients were also divided into three groups according to their coma grading on admission: mild injury (Glasgow Coma Scale score 13-15), moderate injury (8-12), and severe injury (3-7). The amount of subarachnoid blood shown in computerized tomography was evaluated according to the Fisher index, and additional tomography findings were recorded. At last follow-up, presence of headache and neurological deficits as well as return to work or school were investigated, and the last clinical picture was evaluated with the Glasgow Outcome Scale.Results: There were 11 children, 23 adults and 10 elderly patients. Twelve patients died between 1-49 days after trauma; the others were followed for a mean of 14.6 months (from 10 to 30 months). In the children group, Glasgow Coma Scale score was significantly higher (p=0.004), subarachnoid blood amount was significantly lesser, and Glasgow Outcome Scale score was significantly better compared to the other groups. For all groups, higher trauma severity on admission was associated with higher Fisher index (p=0.016). Most important factors affecting clinical results were severity of head injury on admission (p=0.0001), Fisher index (p=0.003), and presence of additional findings on computerized tomography (p=0.0001).Conclusion: Traumatic subarachnoid hemorrhage usually has a good clinical outcome in children; however, in elderly patients, the outcome is worse, and there are usually additional intracranial traumatic lesions. Most important factors affecting outcome are blood amount on first computerized tomography, head trauma severity, and presence of additional intracranial traumatic lesions

Secrets of anesthesia in fetoscopic surgery

WOS: 000371604200004Certain life-threatening congenital malformations have the opportunity to be treated with minimally invasive fetal surgery. In recent years fetoscopic surgery had a triggered interest. During the fetoscopic surgery all interventions effecting uteroplasental blood flow and eventually fetal oxygenation, may occur as complications like cardiac depression, maternal hypotension or pulmonary edema. Liaise with the increase in cardiac output and heart rate, the pregnant patient may display increased sensitivity to muscle relaxants and inhalational anesthetics. Due to incomplete myelination and synaptic activation, the fetus becomes more sensitive to volatile agents and analgesics. A goal directed therapy is necessary for both maternal and fetal well-being. According to goal directed therapy, perioperative fluid, vasopressor and inotropic agent titration is recommended to be used taking into account the systemic and pulmonary vascular hemodynamics of patients as well as the pulmonary vascular permeability and fluid content. Perioperative anesthesia management with hemodynamic monitorization, airway management and postoperative pain therapy are key features that make up the secrets of anesthesia. The patient's postoperative suffering from pain also leads to fetal and maternal stress by causing uterine contractions. Thus, appropriate treatment of postoperative pain should be provided using intravenous or epidural patient-controlled analgesia. One of the most important issues in the postoperative period is to prevent patient's premature contraction and not to trigger a premature birth. (C) 2015 Elsevier Ltd. All rights reserved

Hemimetameric shift in spina bifida: three case reports

WOS: 000398041500023PubMed ID: 27822764Purpose Hemivertebrae is the most frequent reason of congenital scoliosis, and hemimetameric shift is a finding, which is characterized by two hemivertebraes located contralateral, with at least one normal vertebra between them. Embryologically, hemivertebrae is caused by delay in somite movements and as a result, a total vertebral shift occurs because of mismatches in the following segments. Hemimetameric shift accompanying spina bifida is described as extremely rare. There are only two case series of hemimetameric shift in literature but no spina bifida patient was reported in these series. Methods We report three cases of hemimetameric shift with spina bifida with their detailed clinical and radiological evaluations. Results Case 1 is a 3-year-old congenital scoliosis patient with tethered cord. She has mild scoliosis with a very demonstrative hemimetameric shift. Case 3 is an infant with multiplelevel hemivertebrae anomalies and hemimetameric shift who had myelomeningocele closure and ventriculoperitoneal shunt installation neonatally. Case 3 is a 9-year-old male who had operated for myelomeningocele in the neonatal period. He had evident scoliosis with cervicothoracic hemimetameric shift and he is the only patient we operated for prominent scoliosis. Conclusions The present classification of hemimetameric shift was described by Kawakami et al. in 2009. It is based on development of anterior and posterior hemivertebrae segments but we think it is not suitable for patients with posterior fusion defects such as spina bifida patients. Spina bifida patients with scoliosis and hemimetameric shift are a rare but important patient group to evaluate in terms of understanding scoliosis patients with neural tube defects

Open Fetal Surgery in Turkey

WOS: 000456677600010PubMed ID: 30300900

Haemodynamic monitoring for foetal surgery: open versus foetoscopic repair of myelomeningocele

WOS: 000454642400011PubMed ID: 30615799

Shunt revision rates in myelomeningocele patients in the first year of life: a retrospective study of 52 patients

WOS: 000429793400017PubMed ID: 29159427Shunt placement indications are stringent and require confirmation of clinical and radiological evidence of hydrocephalus (HC). The aim of this study was to determine the rate of shunting and discuss the outcome in the first year of life in patients with myelomeningocele (MMC) on the basis of review of the literature. All patients who underwent postnatal repair of MMC at our institution between March 2014 and March 2015 were evaluated. Patients were only included if they underwent both MMC repair and ventriculoperitoneal (VP) shunt insertion at our institution and were followed up for at least 12 months. The mean ages for repair of MMC, MMC levels, timing of VP shunt placement, shunt revisions, and causes of shunt revisions were documented. Fifty-two patients with MMC were included in this study. The average gestational age at birth was 38 weeks. The level of MMC was thoracolumbar in 13 cases, 11 times lumbar, 21 times lumbosacral, and 7 times sacral. Thirty-one patients (59.61%) suffered from hydrocephalus and required placement of a shunt. When we evaluate the lesion levels of patients who require shunting, 13 cases were thoracolumbar, 6 cases were lumbar, and 11 cases were lumbosacral. None of the sacral cases needed VP shunt. Seven patients (13.4%) had shunt revision within the first year of life. The cause of shunt revision was wound problem in one patient (1.9%), underdrainage in two patients (3.8%), infection in three patients (5.7%), and mechanical obstruction in another one patient (1.9%). MMC closure and management of the associated HC are one of the most basic, but never simple, legs of the pediatric neurosurgery around the world. As clinicians and neurosurgeons, we are obligated to analyze recent evidences and evaluate present approaches to achieve optimization in this subject until further technologies or approaches became more advantageous for our patients