Gonadectomy in conditions affecting sex development: a registry-based cohort study

Objectives To determine trends in clinical practice for individuals with DSD requiring gonadectomy. Design Retrospective cohort study. Methods Information regarding age at gonadectomy according to diagnosis; reported sex; time of presentation to specialist centre; and location of centre from cases reported to the International DSD Registry and who were over 16 years old in January 2019. Results Data regarding gonadectomy were available in 668 (88%) individuals from 44 centres. Of these, 248 (37%) (median age (range) 24 (17, 75) years) were male and 420 (63%) (median age (range) 26 (16, 86) years) were female. Gonadectomy was reported from 36 centres in 351/668 cases (53%). Females were more likely to undergo gonadectomy (n = 311, P < 0.0001). The indication for gonadectomy was reported in 268 (76%). The most common indication was mitigation of tumour risk in 172 (64%). Variations in the practice of gonadectomy were observed; of the 351 cases from 36 centres, 17 (5%) at 9 centres had undergone gonadectomy before their first presentation to the specialist centre. Median age at gonadectomy of cases from high-income countries and low-/middle-income countries (LMIC) was 13.0 years (0.1, 68) years and 16.5 years (1, 28), respectively (P < 0.0001) with the likelihood of long-term retention of gonads being higher in LMIC countries. Conclusions The likelihood of gonadectomy depends on the underlying diagnosis, sex of rearing and the geographical setting. Clinical benchmarks, which can be studied across all forms of DSD will allow a better understanding of the variation in the practice of gonadectomy

Possible spontaneous "birth" of a hydatid cyst into the lateral ventricle

EVLIYAOGLU, CETIN AYHAN/0000-0002-1774-3084WOS: 000228728700016PubMed: 15580516Introduction: Intraventricular hydatid cyst is an extremely rare entity. We report a solitary hydatid cyst in a lateral ventricle. Case report: A 7-year-old girl had a free floating intraventricular cyst, diagnosed by computerized tomography examination inside the enlarged left lateral ventricle of an associated Dandy Walker malformation. The patient underwent surgery and the cyst was removed. Conclusion: To our knowledge, this is the first case report in which the natural developmental phases of an intraventricular hydatid cyst have been observed

Proximally situated osseous septum in complex spina bifida - Case report

EVLIYAOGLU, CETIN AYHAN/0000-0002-1774-3084WOS: 000227173800014PubMed: 16206741In Type I split cord malformation (SCM) lesions, the osteocartilaginous spur and its dural sleeve are almost always located in the caudal extent of the median cleft. The authors present a case of lumbar myelomeningocele associated with a thoracic Type I SCM in which an osseous septum is uniquely situated in the proximal extent of the median cleft. Split cord malformations are cord-tethering lesions, which may be associated with a myelomeningocele that causes additional tethering, even in the opposite direction; therefore, both lesions should be treated. In view of this unique case, however, the surgical approach used for tethering lesions in such cases of complex spina bifida should perhaps be tailored. Whenever feasible, meticulous preoperative examination of a patient with spina bifida, including entire craniospinal magnetic resonance imaging followed with perioperative dynamic evaluation, is important for the effectiveness and safety of the surgery

Proximally situated osseous septum in complex spina bifida: Case report

PubMed: 16206741In Type I split cord malformation (SCM) lesions, the osteocartilaginous spur and its dural sleeve are almost always located in the caudal extent of the median cleft. The authors present a case of lumbar myelomeningocele associated with a thoracic Type I SCM in which an osseous septum is uniquely situated in the proximal extent of the median cleft. Split cord malformations are cord-tethering lesions, which may be associated with a myelomeningocele that causes additional tethering, even in the opposite direction; therefore, both lesions should be treated. In view of this unique case, however, the surgical approach used for tethering lesions in such cases of complex spina bifida should perhaps be tailored. Whenever feasible, meticulous preoperative examination of a patient with spina bifida, including entire craniospinal magnetic resonance imaging followed with perioperative dynamic evaluation, is important for the effectiveness and safety of the surgery

Primary cranial vault lymphoma presenting as a traumatic subdural hematoma

EVLIYAOGLU, CETIN AYHAN/0000-0002-1774-3084WOS: 000235798800006PubMed: 16518748Objective and Importance: The authors present a rare case of a primary cranial vault lymphoma, mimicking a subacute subdural hematoma after head trauma. Clinical Presentation: A 78-year-old woman was admitted to the hospital with progressive left hemiparesis, headache and a gradually increasing soft lump over the right parietal region 1 week after head trauma due to falling from a high place. A computed tomography (CT) scan demonstrated a hyperdense parieto-occipital subdural and subgaleal lesion together with marked midline shift and white matter edema. Intervention: Considering her trauma story and clinical progression, the patient underwent an emergency operation with the diagnosis of subdural hematoma. However, the lesion was found to be of a tumoros nature and the histopathological diagnosis was high-grade malignant non-Hodgkin's lymphoma. Conclusion: The authors emphasize the rarity of primary cranial vault lymphoma and its importance in the differential diagnosis of cranial vault mass lesions extending either intra- or extracranially. This case may be considered as another example where magnetic resonance imaging (MRI) is the ultimate definitive test in the emergency ward whenever CT demonstrates any findings which are unclear

Diagnostic value of bed-side ultrasonographic evaluation by anterior approach in spine disorders?: a preliminary report

EVLIYAOGLU, CETIN AYHAN/0000-0002-1774-3084WOS: 000188255800003PubMed: 14603386A preliminary investigation was planned so as to evaluate the effectiveness of ultrasonography for bedside evaluation of patients with suspected spinal pathology. Its application by anterior approach in 37 cases showed that herniated disc material, bone, epidural fat, scar tissue, tumoral masses and their relation to the interspace and dural sac could be visualized. A short description of the sonographic appearance of normal and pathological structures was also given in comparison to Computed Tomography or Magnetic Resonance images. Ultrasonographic examination was inadequate in five of the patients due to obesity, meteorism or diminution of the intervertebral disc space. Advantages of this technique are the availability almost in every hospital, the ease of obtaining images at various angles, avoiding the use of x-rays and the low price. The disadvantages are its relatively poor definition as compared to Computed Tomography or Magnetic Resonance Imaging and its inability to see through bone

Pott's puffy tumor of the vertex years after trauma in a diabetic patient: case report

EVLIYAOGLU, CETIN AYHAN/0000-0002-1774-3084WOS: 000228456700010PubMed: 15756412Pott's puffy tumor is a rare clinical entity characterized by subperiosteal abscess associated with osteomyelitis. It is usually seen as a complication of frontal sinusitis or trauma. This is the unique report of a Pott's puffy tumor located over the vertex of a type 1 diabetic patient with an unusual latency of 14 years following injury. A 27-year old man presented with pain and a soft swelling on his vertex. Magnetic resonance imaging demonstrated subperiosteal abscess in the vertex region associated with dural thickening and perisinusal irregularities of epidural space. Further history revealed that he had a trauma to the same location when he was 13 years old. Considering possible complications due to proximity of the lesion to the sagittal sinus, we retrained from aggressive surgical interventions. We treated our patient with a simple surgical abscess drainage followed by prolonged use of antibiotics and achieved complete therapy. The cellular and humoral elements of the immune system may be disrupted in diabetic patients resulting in such atypical courses and complications of infections. We want to emphasize both importance of the prompt diagnosis of Pott's puffy tumor as intracranial invasion may cause severe neurologic problems, and importance of a surgical intervention tailored for the individual lesion

Pott's puffy tumor of the vertex years after trauma in a diabetic patient: case report

EVLIYAOGLU, CETIN AYHAN/0000-0002-1774-3084WOS: 000228456700010PubMed: 15756412Pott's puffy tumor is a rare clinical entity characterized by subperiosteal abscess associated with osteomyelitis. It is usually seen as a complication of frontal sinusitis or trauma. This is the unique report of a Pott's puffy tumor located over the vertex of a type 1 diabetic patient with an unusual latency of 14 years following injury. A 27-year old man presented with pain and a soft swelling on his vertex. Magnetic resonance imaging demonstrated subperiosteal abscess in the vertex region associated with dural thickening and perisinusal irregularities of epidural space. Further history revealed that he had a trauma to the same location when he was 13 years old. Considering possible complications due to proximity of the lesion to the sagittal sinus, we retrained from aggressive surgical interventions. We treated our patient with a simple surgical abscess drainage followed by prolonged use of antibiotics and achieved complete therapy. The cellular and humoral elements of the immune system may be disrupted in diabetic patients resulting in such atypical courses and complications of infections. We want to emphasize both importance of the prompt diagnosis of Pott's puffy tumor as intracranial invasion may cause severe neurologic problems, and importance of a surgical intervention tailored for the individual lesion

Extremely delayed renal cell carcinoma metastasis mimicking convexity meningioma (2)

PubMed: 19112550Cerebral extra-axial metastasis mimicking meningioma which satisfy several criteria for a diagnosis of meningioma, but which have proved instead to be metastatic carcinoma and extremely delayed cerebral metastasis from renal cell carcinoma form the focus of the presentation. 68-year-old-woman who had been operated for renal cell carcinoma 20 years previously is presented with new symptoms of intracranial mass. A large extra-axial mass of the convexity which destroyed calvarium and dura was excised with Simpson Grade I removal, revealed metastatic carcinoma. Imaging characteristics can not always discern between meningioma and metastatic tumours. A meticulous clinical evaluation and histopathological diagnosis is essential in patients with intracranial mass even they resemble both primary and metastatic tumours