Iniencephaly and long-term survival: a rare case report

Introduction Iniencephaly, a neural tube defect involving occiput and inion and combined with rachischisis of the cervical, thoracic spine, and retroflexion position of the head is a very rare congenital abnormality of the fetus–newborn with a 0.1–10 of 10,000 prevalence. This abnormality’s prognosis is thought to be dismal. This abnormality can be associated with other abnormalities such as anencephaly, encephalocele, hydrocephalus, cyclopia, absence of the mandible, cleft lip and palate, cardiovascular disorders, diaphragmatic hernia, renal abnormalities, overgrowth of the arms compared to the legs, and club food and gastrointestinal atresia. Discussion Most of the patients are dead born, and the others die in a few hours. There are only six previously documented long-term survivors. In our case, our patient with iniencephalic signs and findings is still living. She is 2 years old now. We think that this patient presents a mild form of iniencephaly.     

Orbital hydatid cysts: Report of four cases

Hydatid cysts of central nervous system are rare and comprise only 2% to 3% of all hydatid cysts reported. Orbital localization is very uncommon and has been reported less than 1 % of all hydatid diseases. The primary treatment of hydatid disease is surgical. The most important complication of the surgical treatment is secondary hydatidosis due to spillage of the cyst contents. Because of the difficulties of the orbital localization, total extirpation of the cysts without rupture is almost impossible. Preventing spontaneous rupture of the cysts during surgery and postoperative antihelmintic treatment should be taken into consideration in these cases.This study includes four cases who underwent surgery for orbital hydatid cysts. Radiological characteristics, operative technique and postoperative medical therapy are discussed.     

Intramedullary epidermoid cyst of the cervical spinal cord associated with an extraspinal neuroenteric cyst: case report.

A 4-year-old girl presented with an intramedullary epidermoid cyst of the cervical spinal cord. The clinical, radiological, and surgical features and a brief critical review of the literature are included in this report. The cyst contents were removed totally in two operations. The child had a coexisting neuroenteric cyst in the posterior mediastinum. To our knowledge, this coexistence has not been previously reported. Contemporary imaging modes and prospects of the surgical treatment are discussed.     

Evaluation of fractionated total body irradiation and dose rate on cataractogenesis in bone marrow transplantation

PURPOSE: To assess dose rate effect on cataractogenesis in allogeneic or autologous bone marrow transplanted patients conditioned with fractionated total-body irradiation (FTBI). MATERIALS AND METHODS: Between 1987 and 2001, a total of 105 patients have received TBI conditioning for Bone Marrow Transplantation (BMT) for hematological malignancies at Gulhane Military Medical School. 12 Gy FTBI was applied in 6 fractions over 3 consecutive days with a Co60 teletherapy machine. 46 patients who have survived and were followed up after more than one year were evaluated for cataractogenesis in relation to dose rate. Conditioning therapy included only cyclophosphamide (60 mg/kg/day for two days) + TBI with no steroid and veno-occlusive disease prophylaxis. RESULTS: The median follow-up is 32 months. Posterior subcapsular cataract developed in 5 eyes of three patients out of 46 patients. The 5-year and 10-year estimated cataract incidence in the high-dose rate (> 0.04 Gy/min) group was 29% and 43% respectively while no cataracts occurred in the low-dose rate (< or = 0.04 Gy/min) group. Cataract development in the high-dose rate group versus low-dose rate group was statistically significant (p < 0.039). CONCLUSION: Cataract is a late side effect of TBI. Low-dose rate fractionated TBI is a reliable conditioning program in BMT with effective lens sparing to avoid cataractogenesis.     

Comparing the Approach to Radical Prostatectomy Using the Multiport da Vinci Xi and da Vinci SP Robots: A Propensity Score Analysis of Perioperative Outcomes

BackgroundUse of the single-port da Vinci SP robotic platform for various urological procedures has been described by several groups. However, the comparative performance of the SP robot in relation to earlier models such as the da Vinci Xi is still unclear.ObjectiveTo compare intraoperative and short-term postoperative outcomes between the da Vinci Xi and SP robots for patients undergoing radical prostatectomy (RP) in a referral center.Design, setting, and participantsData were prospectively collected for patients undergoing RP from June 2019 to April 2020 in a single center. The da Vinci SP was used for 71 patients and the da Vinci Xi for 875 patients. After propensity score (PS) matching, two groups of 71 patients were selected for the comparative study.InterventionRP via a transperitoneal approach using the same technique steps and anatomy access with both robot consoles.Outcome measurements and statistical analysisA PS analysis was performed using the covariates age, body mass index, Charlson comorbidity index, Sexual Health Inventory for Men score, American Urological Association symptom score, prostate size, prostate-specific antigen levels, Gleason score, D’Amico risk group, and degree of nerve-sparing. Intraoperative performance and short-term functional (continence and potency) and oncological outcomes were compared between the groups.Results and limitationsMedian follow-up was 4.4 mo (interquartile range [IQR] 1.6–7.2) for the SP group and 3.2 mo (IQR 1.6–4.8) for the Xi group (p = 0.2). The median total operative time and median console time were both significantly higher in the SP group, with median differences of 14 min (95% confidence interval [CI] 9–19) and 5 min (95% CI 0–5), respectively. The proportion of patients with blood loss of >100 ml was significantly lower in the SP group (difference of 27%, 95% CI 12–42%). No intra- or postoperative complications were reported in either group. There were no significant differences in pain scores at 6, 12, and 18 h or in positive surgical margin rates between the groups. The SP group had a significantly higher percentage of extraprostatic extension than the Xi group (difference of 16%, 95% CI 4.6–27%). None of the patients experienced biochemical recurrence during follow-up. The difference in continence rates at 45 d between the SP and Xi groups was 11% (95% CI ?5.6% to 28%) and the difference in potency rates at 45 d was ?7.3% (95% CI ?21% to 6.2%). The short-term follow-up for comparison of functional and oncological outcomes is a limitation.ConclusionsDespite differences in trocar placement and technology between the two da Vinci consoles, the SP has satisfactory intraoperative performance compared to the Xi. SP surgery can be performed safely and effectively during the initial learning phase. However, longer-term follow-up is needed to provide further evidence on the impact of SP implementation on functional and oncological outcomes.Patient summaryWe compared intraoperative and short-term postoperative outcomes for patients who underwent radical prostatectomy using two different robots, the da Vinci Xi and the single-port da Vinci SP. We found that operative time was longer for the Single Port console. Studies with long-term follow-up are needed to compare the functional and oncological outcomes.     

Spindle cell melanoma coexisting with chronic lymphocytic leukemia/small lymphocytic lymphoma: a rare collision tumor in multiple sites

A strong association has been reported between chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL) and malignant melanoma (MM). In rare cases of MM, lymphoid malignancies may be detected incidentally during sentinel lymph node biopsies. In this case, we found a unique collision of MM and CLL infiltration in the skin. An 88-year-old male patient presented with a mass on the nasal root. Histopathological examination of the skin biopsy specimen revealed a deeply infiltrative, atypical spindle cell proliferation in the background of a collagenous stroma. Accompanying this lesion, there were foci of monotonous lymphoid cell populations involving skin appendages. In the immunohistochemical studies, the spindle cells were diffusely positive for S100, and focally positive for Melan-A and HMB45; the lymphoid cells were positive for CD20, CD5, and Bcl-2 and negative for CD3, Bcl-6, CD10, and Cyclin D1. Histopathological and immunohistochemical findings were consistent with diagnoses of spindle cell melanoma and CLL. Interestingly, these two tumors together in their same morphological appearance were confirmed in a subsequent liver biopsy. Active skin surveillance of patients with CLL may be important to detect MM at an early stage that correlates with a better prognosis.     

Prognostic significance of 68Ga-Pentixafor PET/CT in multiple myeloma recurrence: a comparison to 18F-FDG PET/CT and laboratory results

Annals of Nuclear Medicine - This study investigates the prognostic value of 68Ga-Pentixafor PET/CT using PET-derived quantitative in multiple myeloma (MM) patients with suspected recurrence in...