Transcervical Tongue Base Reduction with Hyoepiglottoplasty: Long-Term Results

Aims To evaluate the long term results of tongue base reduction with hyoepiglottoplasty as a surgical option in the treatment of severe obstructive sleep apnea. Material and Method Severe obstructive sleep apnea patients diagnosed as upper airway narrowing at the tongue base level were treated with transcervical tongue base reduction with hyoepiglottoplasty. Seven years after single stage multilevel surgery, the patients were reevaluated clinically, radiologically and polysomnographic records were taken. Preoperative, early postoperative and long-term postoperative parameters were compared to determine the success rate of the surgical technique. Results In the postoperative long-term follow-up Epworth sleepiness scale (ESS) scores were reduced to 4 and 6 respectively 2 months after surgery despite the initial values of 17 and 15. BMI were decreased from 29.7 and 27.9 kg/m² respectively to 26 and 24 kg/m². The apnea/hypopnea index (AHI) were reduced to 14.1 and 16.2 respectively from 68.6 and 83.83. O₂ nadir was 55 and 66% respectively and improved to 86 and 89%. Flexible nasopharyngoscopy revealed competent airway in both retropalatal and retroglossal level. Bed partners scored snoring as 2/10 and 4/10 corresponding to very mild and moderate. Daytime somnolence and witnessed apneic periods were completely disappeared in both patients. Conclusion Open tongue base resection with hyoepiglottoplasty is effective among all other surgical corrections of sleep apnea even after 7 years postoperatively.     

Dyskeratosis congenita: clinical report and review of the literature

Abstract:  Dyskeratosis congenita (DKC) is an inherited disorder that usually presents in males, consisting of the triad of leukoplakia of the mucous membranes, nails dystrophy and skin pigmentation. Oral and dental abnormalities may also be present. Most cases are X-linked autosomal dominant, but recessive forms have also been reported. This study describes herein a case in which the classic triad of signs was present, along with the development of leukoplakia in the buccal mucosa. Our patient, a 25-year-old man, presented with several characteristic systemic features of this condition, together with the following oral features: hypodontia, delayed dental eruption, short blunt roots, extensive caries, gingival inflammation and bleeding, loss of alveolar bone and buccal mucosa with leukoplakia and irregular ulcers. The patient was given full preventive care. The primary teeth were extracted under local anaesthesia. After establishing optimal oral health, oral hygiene instructions were given to the patient and he was rehabilitated with fixed and removable partial denture. Prosthetic treatments were carried out after establishing optimal oral health. This treatment option appears beneficial in this patient, resulting in rehabilitation of occlusion and less mechanical irritation to the oral mucosa.     

Outcome and prognostic factors for children with supratentorial primitive neuroectodermal tumors treated with carboplatin during radiotherapy: A report from the Children's Oncology Group

Background

Supratentorial PNETs (sPNET) are uncommon embryonal malignancies of the central nervous system whose prognosis has historically been poor. We evaluated the outcome and prognostic factors of children with sPNET treated prospectively on a Children's Oncology Group trial.

Procedure

Following surgery, patients received craniospinal radiotherapy with concurrent carboplatin followed by six months of maintenance chemotherapy with cyclophosphamide and vincristine.

Results

Five‐year overall survival (OS) and progression‐free survival (PFS) for all patients was 58 ± 7% and 48 ± 7%. For patients with pineoblastoma (n = 23), five‐year OS and PFS was 81 ± 9% and 62 ± 11%. Extent of resection but not M‐stage was prognostic. Five‐year OS and PFS for 37 patients with non‐pineal tumors (NPsPNET) was 44 ± 8% and 39 ± 8%, significantly worse than for PB (P = 0.055 and 0.009 respectively). Extent of resection and major radiotherapy deviations were prognostic. Five year OS was 59 +/? 11.4% for those undergoing complete resection versus 10.4 +/? 7% for those who did not (P = 0.017). Central pathologic review called 14 (38%) “classic” sPNET, 8 (22%) “undifferentiated” and 13 (35%) “malignant gliomas.” There was no significant difference between the subgroups, although survival distributions approached significance when the combined “classic” and “undifferentiated” group was compared to the “malignant gliomas.”

Conclusions

Carboplatin during RT followed by 6 months of non‐intensive chemotherapy is a feasible treatment strategy for patients with sPNET. Aggressive surgical resection should be attempted if feasible. The classification of supratentorial small cell malignancies can be difficult. Pediatr Blood Cancer 2015;62:776–783. © 2015 Wiley Periodicals, Inc.

     

放射状角膜切开术在轻中度圆锥角膜病例中视觉康复的疗效

目的：探讨放射状角膜切开术在轻中度圆锥角膜治疗中光学和视力康复的效果。
　　方法：回顾性分析应用放射状角膜切开术治疗圆锥角膜的病例22例31眼并进行了至少12 mo的随访。测量并分析术前术后裸眼视力,最佳矫正视力,自动屈光计值,角膜曲率,角膜不规则指数以及并发症。
　　结果：在最后一次随访中,平均裸眼视力( logMAR)由0.86±0.34显著提升至0.30±0.29(P<0.0001),平均最佳矫正视力由0.47±0.21提升至0.17±0.23(P<0.0001)。平均角膜曲率由48.69±3.68 D 降低至44.33±3.09 D ( P<0.0001)。自动屈光计测得平均等效球镜值由-5.61±2.85D显著提升至-2.29±1.95D(P<0.0001)。在整个随访过程中,中央角膜厚度和3mm,5mm区域的角膜不规则指数均无变化。术中和术后没有观察到严重并发症。
　　结论：在本组病例中,放射状角膜切开术是轻中度圆锥角膜视觉康复的有效治疗方法。     

A Novel Approach to Improving Fat Delivery in Neonatal Enteral Feeding

Continuous infusion systems used for enteral nutrition support in the neonatal intensive care unit deliver as little as 60% of the fat in human milk to the neonate. This study determined the effect of mixing common feedings for preterm infants in the feeding bag and tubing on fat losses during enteral feeding. Laboratory models were developed to assess the contribution of various mixing techniques to delivered fat content. Fat content was measured periodically during feeding and compared to baseline measurements. A multistage approach incorporating a feeding bag inverter and a tubing circulation loop delivered >90% of milk fat when used in conjunction with a commercial continuous infusion system. With unfortified human milk, this approach delivered 91.9% ± 1.5% of fat content over a one hour feed, significantly greater (p < 0.01) than 77.5% ± 2.2% delivered by continuous infusion controls (Mean ± SEM). With fortified human milk, this approach delivered 92.1% ± 2.4% of fat content, significantly greater (p < 0.01) than 79.4% ± 1.0% delivered by a non-adapted infusion system (Mean ± SEM). Mixing human milk during continuous infusion improves fat delivery, which may improve nutrition and growth outcomes in low birth weight neonates.     

Blastic Plasmacytoid Dendritic Cell Neoplasm: Single Center Experience on a Rare Hematological Malignancy

PurposeBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and poor prognostic hematological malignancy. There is still no standard treatment established for BPDCN patients. We aim to summarize the main clinical, biological features and treatment of 9 BPDCN patients.MethodsNine patients with BPDCN who had been diagnosed between July 2008 and December 2018 in Ankara University School of Medicine, were retrospectively evaluated.ResultsAll patients (n = 9) were male, median age was 64 (21–80). Five patients (55.6%) had bone marrow infiltration, 5 patients (55.6%) cutaneous lesions, 6 patients (66.7%) lymph node involvement, 2 patients (22.2%) central nervous system involvement and 2 patients (22.2%) spleen involvement at time of diagnosis. Complex karyotype was observed in 2 patients. CHOP was given to 5 patients (55.6%), hyper-CVAD to 2 patients (22.2%), fludarabine, cyclophosphamide and mitoxantrone to 1 patient (11.1%) and cyclophosphamide, etoposide, methylprednisolone to 1 patient (11.1%) as first line chemotherapy. Four patients (44.4%) underwent allogeneic hematopoietic stem cell transplantation (AHSCT) in complete remission (CR) 1. Venetoclax was given to a transplant ineligible patient who had skin and lymph node involvement, with the off-label use. The median follow-up time was 15.9 months (3–48.6 months). Estimated median overall survival was 15.9 + 1.6 (95% CI 12.7–19.1) months.ConclusionIntensive induction therapies followed by AHSCT in CR seems to be best approaches for patients with BPDCN. Thus, more effective treatment strategies particularly targeted therapies should be warranted to improve the survival of patients with this rare disease.