Quality of Life and Hormonal Impairment in Pediatric Patients With Craniopharyngiomas.

INTRODUCTION: Craniopharyngiomas (CP) are tumors in the sellar region that, despite a high survival rate, are associated with significant morbidity, including hypothalamic, hormonal, and visual dysfunction. This study aimed to assess the quality of life (QoL) in pediatric patients with CP and to evaluate its relationship with various factors, with a focus on the impact of endocrine dysfunction. METHODS: In this observational cross-sectional study, patients with CP aged between 0 and 18 years, currently followed up in a tertiary hospital by a multidisciplinary team, were included. QoL was assessed using the validated PEDS-QL4.0 questionnaire, which was administered to parents. This tool estimates Global QoL (QoL-G), further divided into Physical (QoL-P) and Psychosocial (QoL-PS) dimensions, including Emotional (QoL-Em), Social (QoL-S), and School (QoL-Sc) aspects. In Portugal, the estimated average QoL-G is 79.8, QoL-P is 83.5, and QoL-PS is 78.2. Variables studied included gender, current and diagnostic age, follow-up time, presence of hydrocephalus, hypothalamic involvement, type of resection (total or subtotal), radiotherapy, visual impairment, hormonal deficits, and therapy. RESULTS: The study included 11 patients with a median age of 15.2 years (interquartile ratio (IQR), 9.7-17.9 years) and a mean age at diagnosis of 9.3±4.1 years. Of these patients, 54.5% were male, and 36.4% were obese. Subtotal resection was performed in 72.7% of cases. Hydrocephalus was present in 54.5% of the patients, hypothalamic involvement in 63.7%, radiotherapy was received by 81.8%, and visual impairment was noted in 54.5%. All patients presented with at least one hormonal deficit. The average QoL-G was 69.9±22.5, with QoL-P at 66.9±30.0 and QoL-PS at 70.9±21.4. A worse QoL-S was associated with female gender (p=0.030) and subtotal resection (p=0.048). Worse QoL-G, QoL-P, QoL-Em, and QoL-PS were linked to hypothalamic involvement (p values 0.008, 0.025, 0.015, and 0.009, respectively). Irradiated patients had worse QoL-G (p=0.006). Treatment with sexual hormones enhanced QoL-Global (p=0.035) and QoL-Emotional (p=0.020), while treatment for adrenal insufficiency and diabetes insipidus improved QoL-Emotional (p=0.021 and p=0.013). No significant associations with visual deficit or obesity were found. CONCLUSIONS: Pediatric patients with CP appear to have poorer QoL-G, QoL-P, and QoL-PS compared to the healthy Portuguese population. However, the small sample size limits statistically significant associations with many of these variables. Predictors of worse QoL include female gender, hypothalamic involvement, subtotal resection, and radiotherapy. The results may be biased due to the small sample size, questionnaire administration to parents, and possible inadequacy of the questionnaire for the studied population. There is a need for a more suitable tool to enable a more precise assessment of QoL in these patients.

Neonatal Skull Depression: The Role of Cranial Ultrasound.

Nontraumatic congenital neonatal skull depression is a rare condition resulting from external forces shaping the fetal skull. Typically, newborns are asymptomatic, and, usually, the condition resolves in a few months with no need for intervention. However, many newborns undergo a CT scan, an ionizing technique, to check for fractures or intracranial lesions. We report a case of congenital skull depression without neurological deficits, managed conservatively through clinical monitoring and ultrasound.

Modified Technique to Protect the Superior Sagittal Sinus in Cranial Vault Reconstruction for Scaphocephaly / Técnica modificada para proteger o seio sagital superior em reconstrução da calota craniana por escafocefalia

Scaphocephaly remains the most prevalent form of isolated single-suture synostosis. In most Pediatric Neurosurgery Centers, the treatment consists of extensive calvarial reshaping operations which are performed in relatively late ages. We described a modified open technique of cranial vault reconstruction for scaphocephaly and we report the anesthetic approach and how it influences our results and enables the performance of this surgery at earlier ages. A total of 47 patients with an average age of 11 2.72 months underwent to the same surgical technique. Blood transfusion during the surgical intervention was needed in 25 patients (53%), and 22 patients (46.8%) did not need blood transfusion. None of the 14 patients who used tranexamic acid (TXA) needed blood transfusions. We reported a statistically significant association between the use of TXA and blood transfusion (p < 0.0001), with a decreased need for blood transfusion with the use of TXA. Besides sagittal suturectomy, we create new parasagittal sutures by replacing the prematurely fused one, and 1.5 cm of the anterior segment of bone of those 2 rectangular structures are cut before fixation, to enable the immediate shortening of the anteroposterior (AP) diameter of the skull. Finally, we put a mesh plate (SonicWeld Rx, KLS Martin Group, Tuttlingen, Baden-Wuerttemberg, Germany) over the superior sagittal sinus (SSS), which is fixed anteriorly and posteriorly with pins (SonicPins Rx, KLS Martin Group). This procedure enables an immediate reduction of the AP diameter of the skull, with no further therapies needed, and promotes a wide decompression and remodeling of the skull. With an experienced surgical team and an anesthesiologic protocol, we were able to perform this surgery at early ages, requiring no blood transfusions.

A escafocefalia continua sendo a forma mais prevalente de sinostose isolada de sutura única. Na maioria dos Centros de Neurocirurgia Pediátrica, o tratamento consiste em extensas operações de remodelação calvária, realizadas em idades relativamente tardias. Descrevemos uma técnica aberta modificada de reconstrução da calota craniana para escafocefalia e relatamos a abordagem anestésica e como ela influencia nossos resultados e permite a realização desta cirurgia em idades mais precoces. Um total de 47 pacientes com idademédia de 11 2,72meses foram submetidos à mesma técnica cirúrgica. A transfusão sanguínea durante a intervenção cirúrgica foi necessária em 25 pacientes (53%) e 22 pacientes (46,8%) não necessitaram de transfusão sanguínea. Nenhum dos 14 pacientes que usaram ácido tranexâmico (TXA) precisou de transfusões de sangue. Relatamos uma associação estatisticamente significativa entre o uso de TXA e transfusão de sangue (p < 0,0001), com diminuição da necessidade de transfusão de sangue com o uso de TXA. Além da suturectomia sagital, criamos novas suturas parassagitais substituindo a fundida prematuramente, e 1,5 cm do segmento anterior do osso dessas duas estruturas retangulares são cortados antes da fixação, para permitir o encurtamento imediato do diâmetro ântero-posterior (AP) do crânio. Por fim, colocamos uma placa de tela (SonicWeld Rx, KLS Martin Group, Tuttlingen, Baden-Wuerttemberg, Alemanha) sobre o seio sagital superior (SSS), a qual é fixada anterior e posteriormente com pinos (SonicPins Rx, KLS Martin Group). Este procedimento permite uma redução imediata do diâmetro AP do crânio, sem necessidade de terapias adicionais, e promove ampla descompressão e remodelação do crânio. Com uma equipe cirúrgica experiente e um protocolo anestesiológico, conseguimos realizar esta cirurgia em idades precoces, sem necessidade de transfusões sanguíneas.

Incidental versus symptomatic nonfunctioning pituitary adenomas: Are they different?

BACKGROUND: Nonfunctioning pituitary adenomas (NFPAs) constitute one of the most common tumours in the sellar region and are often discovered only when associated with compressive symptoms. With the frequent use of brain imaging, there has been an increase in the prevalence of incidentally discovered NFPAs. AIM: We aim to determine the prevalence of incidental diagnosis with NPAs observed over a decade and compare the analytical, clinical and treatment differences between those who were diagnosed either incidentally or symptomatically. We also intend to evaluate the pathology differences between both groups. METHODS: We retrospectively analysed patients aged ≥18 years with an apparent NFPA, defined as a pituitary lesion compatible with pituitary adenoma which is not associated with the clinical or biochemical evidence of a hormone-secreting tumour. Inclusion criteria included normal prolactin level for lesions <9 mm or a prolactin level <100 ng/mL for lesions ≥10 mm in maximal tumour diameter. RESULTS: We included 119 patients [53.8% males; mean age: 56.8 years (SD = 16.7)]. Diagnosis was incidental in 47.1% of patients, and many patients had unappreciated signs and symptoms of pituitary disease. In the symptomatic and incidental groups, 66.7% and 41.1% of patients had hypopituitarism, respectively (p = .005). Only 20.4% of patients incidentally diagnosed had microadenoma (p = .060). Hypopituitarism was present in 18.8% of those patients with microadenomas. Most tumours were macroadenomas (87.4%). Half of those patients diagnosed incidentally were submitted to surgery, compared with 75.8% of those who were diagnosed symptomatically (p = .004). CONCLUSIONS: Nonfunctioning pituitary adenomas are commonly diagnosed incidentally, with many manifesting symptoms on examination. NFPAs incidentally diagnosed are more commonly macroadenomas and less frequently associated with hypopituitarism than symptomatic. Accordingly, if there was a greater level of knowledge and more suspicion about these pathologies, it might be possible to discover them earlier.

Real Spinal Cord Injury Without Radiographic Abnormality (SCIWORA) in Pediatrics: A Clinical Case Report and Literature Review.

Spinal cord injury without radiological abnormality (SCIWORA) was first reported in 1974. The term was used to define "clinical symptoms of traumatic myelopathy without signs of fracture or spine instability on X-ray or CT scan." With the emergence of MRI, the gold standard method to identify spinal cord injuries, about two-thirds of former SCIWORA cases were found to have pathological findings, and, as such, the term has taken on an ambiguous meaning in the literature. We describe the clinical case of a 17-year-old boy who was admitted to the emergency department of a tertiary hospital after a fall during a soccer game. He suffered spinal and cranioencephalic trauma. A few minutes later, the boy began to show decreased strength in the right upper limb and lower limbs, as well as changes in sensation in the right hemibody. On objective examination, the boy presented a Glasgow Coma Scale score of 15 and the American Spinal Injury Association Impairment Scale D, with partial improvement of initial symptoms of monoparesis of the right lower limb. There were no other changes, specifically at the sensory level. The patient underwent a CT and MRI of the spine that showed no fractures, instability, or appreciable medullary signal changes. Electromyography was normal. Based on the clinical history and imaging findings, real SCIWORA was diagnosed. The patient was admitted to an inpatient rehabilitation program. At a follow-up visit two months later, a complete reversal of signs and symptoms was confirmed. The prognosis of this pathology depends on the extent of the spinal cord injury, as evidenced by MRI. Although neurological improvement when severe deficit is present at initial presentation is unlikely, most patients with incomplete neurological damage show good recovery. The absence of visible changes on MRI is associated with a better prognosis.

Primary Central Nervous System Lymphoma Mimicking the Presentation of Pituitary Apoplexy: A Case Report.

Pituitary lymphoma is one of the rare variants of primary central nervous system lymphoma (PCNSL), mostly arising due to the metastatic spread of systemic lymphoma. We report the case of a 69-year-old woman who initially presented to her family physician with a headache but without any other symptoms. The MRI scan revealed a sellar mass consistent with a pituitary macroadenoma. When the patient was referred to our hospital, two weeks later, the symptoms had progressed, comprising complete right-sided ophthalmoplegia and ptosis, with left-sided amaurosis. A repeat MRI revealed an increased size of the sellar mass, consistent with pituitary apoplexy. A right pterional craniotomy with partial resection of the mass was performed and an intraoperative frozen section biopsy was carried out. The final pathology revealed diffuse large B-cell lymphoma. A systemic follow-up including a whole-body CT, bone marrow aspiration, and cerebrospinal fluid studies ruled out any systemic manifestation, and the patient was HIV-negative. The patient underwent treatment with methotrexate, cytarabine, thiotepa, and rituximab for PCNSL. Although rare, PCNSL can mimic pituitary apoplexy, which needs to be considered if conservative therapy or surgery is to be offered to a patient with a radiological and clinical diagnosis of pituitary apoplexy.

Pituitary metastasis unveiling a lung adenocarcinoma.

SUMMARY: Pituitary metastasis (PM) can be the initial presentation of an otherwise unknown malignancy. As PM has no clinical or radiological pathognomonic features, diagnosis is challenging. The authors describe the case of a symptomatic PM that revealed a primary lung adenocarcinoma. A 62-year-old woman with multiple sclerosis and no history of malignancy, incidentally presented with a diffusely enlarged and homogeneously enhancing pituitary gland associated with stalk enlargement. Clinical and biochemical evaluation revealed anterior hypopituitarism and diabetes insipidus. Hypophysitis was considered the most likely diagnosis. However, rapid visual deterioration and pituitary growth raised the suspicion of metastatic involvement. A search for systemic malignancy was performed, and CT revealed a lung mass, which proved to be a lung adenocarcinoma. Accordingly, the patient was started on immunotherapy. Resection of the pituitary lesion was performed, and histopathology analysis revealed metastatic lung adenocarcinoma. Following surgery, the patient underwent radiotherapy. More than 2 years after PM detection, the patient shows a clinically relevant response to antineoplastic therapy and no evidence of PM recurrence. LEARNING POINTS: Although rare, metastatic involvement of the pituitary gland has been reported with increasing frequency during the last decades. Pituitary metastasis can be the initial presentation of an otherwise unknown malignancy and should be considered in the differential diagnosis of pituitary lesions, irrespective of a history of malignancy. The sudden onset and rapid progression of visual or endocrine dysfunction from a pituitary lesion should strongly raise the suspicion of metastatic disease. MRI features of pituitary metastasis can overlap with those of other pituitary lesions, including hypophysitis; however, rapid pituitary growth is highly suggestive of metastatic disease. Survival after pituitary metastasis detection has improved over time, encouraging individualized interventions directed to metastasis to improve quality of life and increase survival.

Cushing's syndrome: Consequences of late diagnosis after bariatric surgery.

Prior to bariatric surgery, endocrine causes of obesity must be excluded. The diagnosis of osteoporosis in a male requires the study of secondary causes of this condition. The diagnostic delay of Cushing's syndrome may have irreversible consequences.

Acromegaly with congenital generalized lipodystrophy - two rare insulin resistance conditions in one patient: a case report.

BACKGROUND: Lipodystrophies are a group of diseases which are characterized by abnormal adipose tissue deposition and are frequently associated with metabolic changes. Congenital generalized lipodystrophy is an autosomal recessive syndrome, with a prevalence < 1:10 million. Acromegaly is a rare disease, secondary to the chronic hypersecretion of growth hormone and insulin-like growth factor-1, with characteristic metabolic and somatic effects. "Acromegaloidism" is a term used for patients who manifest clinical features of acromegaly, but do not present a demonstrable hormone growth hypersecretion. The extreme shortage of subcutaneous adipose tissues and muscle hypertrophy confer an acromegaloid-like appearance in these patients. CASE PRESENTATION: We describe a case of a patient with the rare combination of Berardinelli-Seip congenital lipodystrophy and acromegaly; our patient is a 63-year-old white man, who was referred to an endocrinology consultation for suspected lipodystrophy. He had lipoatrophy of upper and lower limbs, trunk, and buttocks, with muscular prominence, acromegaloid facial appearance, large extremities, and soft tissue tumescence. In addition, he had dyslipidemia and prediabetes. His fat mass ratio (% trunk fat mass/% lower limbs fat mass) was 1.02 by densitometry and he also had hepatomegaly, with mild steatosis (from an abdominal ultrasound), and left ventricular hypertrophy (from an electrocardiogram). His first oral glucose tolerance test had growth hormone nadir of 0.92 ng/mL, and the second test, 10 months afterwards, registered growth hormone nadir of 0.64 ng/mL (growth hormone nadir < 0.3 ng/mL excludes acromegaly). Pituitary magnetic resonance imaging identified an area of hypocaptation of contrast product in relation to a pituitary adenoma and he was subsequently submitted to transsphenoidal surgical resection of the mass. A pathological evaluation showed pituitary adenoma with extensive expression of growth hormone and adrenocorticotropic hormone, as well as a rare expression of follicle-stimulating hormone and prolactin. A genetic study revealed an exon 3/exon 4 deletion of the AGPAT2 gene in homozygosity. CONCLUSIONS: Congenital generalized lipodystrophy is a rare disease which occurs with acromegaloid features. As far as we know, we have described the first case of genetic lipodystrophy associated with true acromegaly. Although this is a rare association, the presence of congenital generalized lipodystrophy should not exclude the possibility of simultaneous acromegaly.

Giant prolactinoma, germline BRCA1 mutation, and depression: a case report.

BACKGROUND: Giant prolactinomas are very rare pituitary tumors that may exhibit an aggressive behavior and present with a life-threatening condition. CASE PRESENTATION: A 25-year-old white woman was admitted to our hospital with a headache, psychomotor retardation, reduced vision, and loss of autonomy in daily activities. Her past medical history was significant for having oligomenorrhea and a depressive syndrome since her mother's death. She also had a breast cancer gene 1 (BRCA1) mutation and a family history of breast cancer. She had marked hyperprolactinemia (7615 ng/dL), central hypocortisolism, growth hormone deficiency, and a giant pituitary tumor (52 × 30 × 33 mm) which was shown in magnetic resonance imaging with obstructive hydrocephalus, requiring emergency surgery. Treatment with cabergoline led to a 99.8% reduction in serum prolactin levels and significant tumor shrinkage. Her depressive symptoms progressively improved and psychiatric drugs were withdrawn after 3 months of cabergoline treatment. Currently, she is being followed in Endocrinology, Neurosurgery, and Neurophthalmology out-patient clinics and in a breast cancer unit. Careful monitoring, support, and follow-up will be essential throughout this patient's life. CONCLUSIONS: This case is a rare presentation of a giant prolactinoma in a young woman, who presented a life-threatening event. She also had an unexpected association between diseases or symptoms that may have contributed to the delay in diagnosis. Given the concomitant presence of a giant prolactinoma, a BRCA1 mutation, and depressive symptoms, a possible association was hypothesized. The breast cancer risk in a BRCA1 mutation carrier and the possible interference of hyperprolactinemia and life events were also discussed. However this hypothesis requires further investigation.

A challenging coexistence of central diabetes insipidus and cerebral salt wasting syndrome: a case report.

BACKGROUND: Combined central diabetes insipidus and cerebral salt wasting syndrome is a rare clinical finding. However, when this happens, mortality is high due to delayed diagnosis and/or inadequate treatment. CASE PRESENTATION: A 42-year-old white man was referred to neurosurgery due to a non-functional pituitary macroadenoma. He underwent a partial resection of the tumor on July 2, 2015. On the day following surgery he presented polyuria with sodium 149 mEq/L, plasma osmolality 301 mOsm/kg, and urine osmolality 293 mOsm/kg. He started nasal desmopressin 0.05 mg/day with good response. He was already on dexamethasone 4 mg and levothyroxine 75 mcg due to hypopituitarism after surgery. On July 9 he became confused. Cerebral computed tomography was performed with no significant changes. His natremia dropped to 128 mEq/L with development of polyuria despite maintenance of desmopressin dose. His hemoglobin and hematocrit rose from 9.1 g/L to 11.6 g/L and 27.5 to 32.5, respectively. His thyroid function was normal and he was on hydrocortisone 30 mg/day. At 12 p.m. 150 mg/hydrocortisone infusion was initiated, but sodium did not increase. Plasma and urine osmolality were 264 mOsm/kg and 679 mOsm/kg, respectively. At 4 p.m. hydrocortisone was increased and hypertonic saline replacement started. Two hours later he was dehydrated with polyuria and vomiting, and natremia of 124 mEq/L. Hyponatremia was very resistant to treatment despite hypertonic saline replacement, hence desmopressin was suspended. The following day, urine spot analysis showed that natriuresis was 63 mEq/L with serum sodium 132 mEq/L. This was interpreted as a cerebral salt wasting syndrome and control was achieved with aggressive hypertonic saline replacements and fludrocortisone 0.1 mg/three times a day. CONCLUSIONS: We present a rare case of a patient with diabetes insipidus and cerebral salt wasting syndrome, who was successfully treated. Hyponatremia in a patient with diabetes insipidus may erroneously be interpreted as inadequate diabetes insipidus control or as syndrome of inappropriate antidiuretic hormone secretion, leading to therapeutic errors. Thus, all clinical and analytical data should be evaluated together for early and correct diagnosis.

Prolactin-Producing Pituitary Carcinoma, Hypopituitarism, and Graves' Disease-Report of a Challenging Case and Literature Review.

INTRODUCTION: The diagnosis of pituitary carcinoma is very rare, requires the evidence of metastatic disease, and has a poor overall survival. Malignant prolactinoma frequently requires dopamine agonist therapy, pituitary surgery, radiotherapy, and even chemotherapy. CASE DESCRIPTION: A 19-year-old female presented with galactorrhea, primary amenorrhea, and left hemianopsia. Complementary study detected hyperprolactinemia and a pituitary macroadenoma with cavernous sinus invasion and suprasellar growth. She was treated with cabergoline and bromocriptine without clinical or analytical improvement. Resection of the pituitary lesion was programmed and a non-contiguous lesion of the nasal mucosa was detected during the approach. This metastasis led to the diagnosis of prolactin-producing pituitary carcinoma. After partial resection, the patient was submitted to radiotherapy for residual disease with persistent symptoms. She developed growth hormone deficiency, central hypothyroidism, hypogonadism, and permanent diabetes insipidus. Six years later she was admitted for the suspicion of secondary adrenal insufficiency and thyrotoxicosis. Physical findings, laboratory data, thyroid ultrasound, and scintigraphy achieved the diagnosis of Graves' disease and hypocortisolism. She was treated with hydrocortisone and methimazole, but central hypothyroidism recurred after antithyroid drug withdrawal. Nine years after the diagnosis of a pituitary carcinoma, she maintains treatment with bromocriptine, has a locally stable disease, with no metastases. CONCLUSION: This report highlights an unusual presentation of a prolactin-producing pituitary carcinoma in a young female. The patient had multiple hormone deficiencies due to a pituitary lesion and treatments. The posterior development of hyperthyroidism and adrenal insufficiency brought an additional difficulty to the approach.

Endoscopic Third Ventriculostomy for the Treatment of Hydrocephalus in a Pediatric Population with Myelomeningocele.

BACKGROUND: Hydrocephalus develops in up to 90% of patients born with myelomeningocele. Although endoscopic third ventriculostomy (ETV) is currently considered the preferred treatment for obstructive hydrocephalus, its results have been inconsistent in patients with myelomeningocele. This study focuses on clinical and radiologic outcomes of ETV in children with hydrocephalus related to myelomeningocele. METHODS: Medical records of 18 pediatric patients with myelomeningocele treated with ETV from 1998 to 2015 at the Centro Hospitalar São João (Porto, Portugal) were reviewed retrospectively. Patients' caregivers were contacted to evaluate their clinical manifestations before and after surgery regarding signs and symptoms of hydrocephalus and Chiari malformation. Control neuroradiologic imaging of 9 patients was obtained and analyzed. Success of ETV was defined by clinical resolution and radiologic confirmation. RESULTS: ETV was successful in 8 of 18 cases (44.4%). Groups of patients were compared according to age at the time of surgery, with a 40% (2/5) success rate in newborns and a 50% success rate (3/6) in children older than 1 year. Eight patients underwent ETV as a first option, with a 37.5% success rate. Ten patients underwent the procedure after previous ventriculoperitoneal shunt (VPS), 5 for malfunction and 5 for VPS infection with 60% and 40% success rates, respectively. Early postoperative complications occurred in 2 patients. CONCLUSIONS: ETV can be performed in patients with myelomeningocele and hydrocephalus with success rates of almost 50%. Prior VPS or VPS malfunction or infection do not contraindicate ETV. If possible, the procedure should be delayed until the patient is at least 1 month old.

Endoscopic re-opening of third ventriculostomy: Case series and review of literature.

OBJECTIVES: Endoscopic third ventriculostomy (ETV) emerged as an effective alternative to shunting devices in patients with obstructive hydrocephalus. When ETV fails, neurosurgeons must choose between applying a shunting device or performing a repeat ETV (re-ETV) and attempt a shunt independent outcome. In this series, clinical, surgical and follow-up data from six patients who underwent a second ETV were reviewed. PATIENTS AND METHODS: Between January 2005 and June 2015, six patients underwent re-ETV, with four being children. Causes of obstructive hydrocephalus included idiopathic aqueduct stenosis, congenital aqueduct stenosis, neonatal intraventricular haemorrage, hypothalamic glioma and post-meningitis aqueductal stenosis. Success of the procedure was defined by clinical improvement and shunt independence. RESULTS: Overall success rate of this series was 83.3%, with re-ETV being effective in five of the six patients. The single case of re-ETV failure was observed in the pediatric population and was due to late stoma obstruction by tumoral growth, with a ventriculo-peritoneal shunt (VPS) being placed 6 months after re-ETV. In this series, no mortality and no major permanent morbidity were observed following re-ETV. CONCLUSION: Repeat ETV is a safe and effective procedure and should be an option for treatment of recurrent obstructive hydrocephalus if stoma closure or obstruction is present. Younger age and the presence of a previous VPS should not discourage this procedure.

Combined endonasal and neurosurgical resection of a congenital teratoma with pharyngeal, intracranial and orbital extension: Case report, surgical technique and review of the literature.

OBJECTIVES: This study reports a patient with a large teratoma involving the oropharynx, the nasopharynx and the left orbit, with intracranial extension. This case represents one of the first reported instances of such an association. A literature review reporting head and neck teratomas with intracranial involvement is also presented. CASE REPORT: The authors describe a case of a neonate presenting with a huge teratoma causing respiratory distress due to upper airway obstruction. The child was operated on at 3 months of age with a combined neurosurgical and endonasal endoscopic-assisted approach. After more than 1-year follow-up, the child has no recurrence and no complications of surgery. DISCUSSION: This type of teratoma is very rare and surgical morbidity is common. The diagnosis may be apparent before birth, which will facilitate the planning of respiratory management. The combined neurosurgical and otolaryngologic approach for a neonatal teratoma has not previously been described. Using an endoscopic-assisted approach for intranasal tumors removal in neonates and infants is a very rare surgical challenge. CONCLUSION: Large nasopharyngeal teratomas of infancy with sinonasal and intracranial extension may be managed using a combined endoscopic-assisted endonasal and neurosurgical procedure.

Decompressive craniectomy for massive internal carotid artery infarction after pediatric penetrating neck trauma.

Pediatric penetrating carotid arterial trauma is a rare unreported cause of malignant cerebral infarction. Despite increasing evidence of benefit of decompressive hemicraniectomy (DCH) in pediatric malignant stroke, indications and predictors of outcome remain controversial. We report a 4-year-old boy with penetrating zone II neck trauma with laceration of the right internal carotid artery who developed malignant cerebral infarction requiring DCH. Impressive neurological recovery and excellent functional outcome was observed with good psychomotor development and quality of life. To our knowledge, this is the first reported case of pediatric malignant ICA infarction due to penetrating arterial trauma with good neurologic outcome after DCH.

Pituitary incidentalomas: analysis of a neuroradiological cohort.

PURPOSE: Most pituitary lesions are detected during the investigation of symptoms associated with hormonal dysfunction and vision abnormalities. When the lesion is identified in an image performed for reasons not related to the tumor, the term incidentaloma applies. Our aim was to describe the diagnosis behind pituitary incidentalomas, patient characteristics and their follow up. METHODS: We searched for the terms "pituitary", "hypophysis" and "incidentaloma" in the requisitions and reports of all CTs and MRIs performed between 1st September 2008 and 30th October 2013. We retrieved demographic data as well as information regarding presentation and follow-up. RESULTS: We detected 71 pituitary incidentalomas, 3 in children/adolescents. In adult patients, mean age was 51.6 ± 18.46 years and 42 were female (61.8 %). The most frequent reason for imaging was headache (33.8 %). The image that first detected the incidentaloma was CT scan in 63.2 and 17.6 % patients presented symptoms that could have led to earlier diagnosis. Pituitary adenoma is the most prevalent lesion (n 48; 70.6 %), followed by Rathke's cleft cyst (n 9; 13.2 %). Hormonal evaluation revealed hypopituitarism in 14 patients and hypersecretion in 6: 5 prolactinomas and 1 somatotroph adenoma. Twenty-one (28.8 %) patients underwent surgery and there was no malignancy. CONCLUSIONS: In concordance with available literature, adenomas are the most frequent incidentally found pituitary lesions. Hormonal dysfunction is quite prevalent, including symptomatic presentations, which suggests that there seems to be a low sensitivity for the diagnosis of pituitary disease.

Endoscopic third ventriculostomy in the management of hydrocephalus: Outcome analysis of 168 consecutive procedures.

BACKGROUND: Endoscopic third ventriculostomy (ETV) is the treatment of choice for obstructive hydrocephalus, but the outcome is still controversial in terms of age and aetiology. METHODS: Between 1998 and 2011, 168 consecutive procedures were performed in 164 patients, primarily children (56%<18 years of age and 35%<2 years of age). The causes of obstructive hydrocephalus included tumoural pathology, Chiari malformation, congenital obstruction of the aqueduct, post-infectious and post-haemorrhagic membranes, and ventriculo-peritoneal shunt (VPS) malfunctions. Successful ETV was defined by the resolution of symptoms and the avoidance of a shunt. RESULTS: ETV was successful in 75.6% of patients, but 19% of the patients required VPS in the first month after ETV, and 5.4% required a VPS more than one month after ETV. Four patients were ultimately submitted for second ETVs. In this series, no major permanent morbidity or mortality was observed. CONCLUSIONS: ETV is a safe procedure and an effective treatment for obstructive hydrocephalus even following the dysfunction of previous VPSs and in children younger than two years.

Trans-lamina terminalis approach for third ventricle and suprasellar tumours.

BACKGROUND: The trans-lamina terminalis (TLT) approach to the suprasellar region and third ventricle is complex, with risks of visual and hormonal deficits. However, the postoperative deficits might not be directly related to opening of the lamina terminalis but to the close relationship of tumours with vital neural and vascular structures. The analysis of results using this approach was the objective of this study. MATERIAL AND METHODS: The TLT approach was used in 29 patients (18 craniopharyngiomas, 5 astrocytomas, 5 germinomas and 1 ganglioglioma). The extent of tumour removal, mortality and morbidity (especially visual or hormonal deficits) were studied. RESULTS: Complete tumour removal was achieved in 15 patients, subtotal extensive removal (more than 90%) in 9 cases and partial removal in 5 cases. Panhypopituitarism developed in 22 patients. Total tumour removal was associated with the development of endocrinological disturbances. There was worsening or the onset of new visual field defects in 4 cases. Postoperative endocrine and visual deficits were in the range generally described regarding surgery for tumours in this region. CONCLUSION: The TLT approach allows for extensive removal of third ventricle and suprasellar tumours, without increased risks of visual and hormonal deficits, compared to those described regarding surgery for lesions in this region.