ALX-Related Frontonasal Dysplasias: Clinical Characteristics and Surgical Management.

AIM: The term frontonasal dysplasia (FND) represents a spectrum of anomalies and its genetics have not been well defined. Recently, the critical role of the aristaless-like homeobox (ALX) gene family on the craniofacial development has been discovered. In the present study, we aimed to propose a systematic surgical treatment plan for the ALX-related FNDs according to the genotypic classification as well as demonstrating their clinical characteristics to help surgeons diagnose the underlying pathology accurately. DESIGN: Single-institution retrospective. SETTING: Tertiary health care. PATIENTS AND METHODS: Eighty-nine FND cases were evaluated. Eight of them had ALX1-related FND3, 3 had ALX3-related FND1, and 2 had ALX4-related FND2. Phenotype characteristics of ALX-related FNDs were evaluated, and relevant surgical interventions were assessed. RESULTS: The ALX1-related FND3 phenotype is striking due to the involvement of the eyes in addition to the presence of hypertelorism, facial clefts, and nasal deformities. A widened philtrum and prominent philtral columns are remarkable features of the ALX3-related FND1, whereas the ALX4-related FND2 has more severe deformities: severe hypertelorism, brachycephaly, large parietal bone defects, broad nasal dorsum, and alopecia. Facial bipartition, box osteotomies, eyelid coloboma repair, cleft lip and palate repair, nasal reconstruction, and fronto-orbital advancement can be performed in ALX-related FNDs based on the characteristics of each subtype. CONCLUSIONS: This genetic classification system will help surgeon diagnose patients with FND with unique features and draw a roadmap for their treatment with a better surgical perspective.

A diagnostic dilemma: A rare case of an ovarian carcinoma with signet ring cells hidden behind a substantial dermatomyositis.

Dermatomyositis (DM) is a malignancy-associated inflammatory connective tissue disease which involves muscles and skin. It accompanies many cancer types. Herein, we aimed to present a 42-year-old patient with primary signet ring cell ovarian carcinoma which has not been seen hitherto. Presentation with DM induces rapid and aggressive progression and emphasizes the importance of more comprehensive malignancy screening in these patients.

Preoperative neutrophil-to-lymphocyte ratio and plateletto- lymphocyte ratio as new prognostic factors for patients with colorectal cancer.

PURPOSE: The objective of this study was to preoperatively evaluate blood platelet to lymphocyte ratio (PLR) and neutrophil to lymphocyte ratio (NLR) for their prognostic value in patients with colorectal cancer (CRC). METHODS: We retrospectively reviewed 347 patients who underwent colorectal surgery for CRC in the Istanbul Education and Research Hospital and the Antalya Education and Research Hospital. The prognostic value of preoperative PLR, NLR, and other clinical and laboratory parameters was assessed with univariate and multivariate analysis. RESULTS: Median overall survival (OS) was 61.8 months [95% CI for hazard ratio (HR) 46.24-77.14]. Significant parameters in univariate analysis, which were the preoperative levels of carcinoembryonic antigen (CEA) (p=0.055), albumin (p=0.003), hemoglobin (p=0.012), PLR (p=0.004), and NLR (p=0.054) were assessed by multivariate analysis which showed that only albumin retained its significance (p=0.008). Median OS was 70.1 vs 44.8 months with PLR ? 180 vs PLR > 180 (log rank; p=0.005). Median OS was "Not reached" (NR) vs 43.5 months with NLR ? 3 vs NLR > 3 (log rank; p=0.012). CONCLUSIONS: This study showed that preoperative levels of CEA, albumin, PLR, and NLR have significant prognostic value for patients with CRC.

Surgical rehabilitation of nasoalveolar complex in patients with alveolar clefts.

OBJECTIVE: The objective of this study was to review the outcome of surgical rehabilitation of nasoalveolar complex in patients with alveolar clefts. STUDY DESIGN: Twenty-seven patients (13 female, 14 male) with 4 bilateral and 23 unilateral alveolar clefts who were treated in our clinic during the period between 2002 and 2009 were included in the study. RESULTS: All the patients had oronasal fistulas, and all of them were closed successfully except one. Recurrence of the oronasal fistula was seen in 1 patient. Alar base was supported by onlay cortical bone in most of the patients. Eleven of the canines at the cleft site erupted after the operation in to the grafted area. Seventy-six percent (n = 16) of the 21 patients could be assigned to the successful groups 1 and 2, whereas 24% (n = 5) were assigned to the unfavorable group. There was not any insufficient result. CONCLUSIONS: Bone graft placed along the piriform margin and alar wings during alveolar bone grafting improves the results of nasal correction. Late grafting should be performed at least to support the alar base for nasal symmetry.

Auricular teratoma: report of a case and review of rare sites.

We report a case of auricular teratoma in a 2-month-old child. We could find no other report of a case of auricular teratoma, although there are a few reports of teratomas in various other parts of the ear.

New experimental composite flap model in rats: gluteus maximus-tensor fascia lata osteomuscle flap.

Experimental animal models need to be developed for studies of composite flaps that have often recently been used for defects of both bone and soft tissues. A consistent anatomy, simple surgical technique, and reliable blood flow are essential for the success of experimental flap studies. Here we propose a gluteus maximus-tensor fascia lata osteomuscle flap in rats as a model of these qualities. Gluteus maximus and tensor fascia lata muscles and the adjacent iliac bone segment were combined as a lateral circumflex femoral artery-based flap. To test the reliability of this composite flap, three types of composite tissues were harvested and replaced: osteomusculocutaneous flap, osteomuscle flap, and osteomuscle composite graft. The osteomusculocutaneous flap was elevated by including a skin island over the gluteal region. The osteomuscle graft was formed by deliberately dividing the vascular pedicle of the osteomuscle flap. Direct observation revealed complete necrosis of the skin islands in all osteomusculocutaneous flaps. Microangiography of the flap demonstrated that both muscles and the attached bone were supplied by the pedicle. Dye studies with nitro blue tetrazolium (NBT) and India ink demonstrated dye uptake in both muscle and bone components in osteomuscle flaps. Histological examinations also demonstrated the viability of both tissues only in the flap group. Bone scintigraphy performed in flaps on postoperative day 7 demonstrated radionuclide uptake, confirming perfusion of the bony segment. The gluteus maximus-tensor fascia lata osteomuscle flap is a reliable and simple model for composite flap studies that offers the following advantages: 1) it is a new composite flap which includes bone, 2) it can be dissected easily with the naked eye, without using the microscope, 3) it has a long pedicle for flap displacement, and 4) it is a small animal model.