Multidisciplinary surgical approach for cerebrospinal fluid leak in children with complex head trauma

Abstract Introduction Post-traumatic cerebrospinal fluid leak from the anterior cranial fossa in children may be isolated or combined with severe facial and calvarial injury. Untreated leak may result with meningitis, hydrocephalus, and abnormal neurocognitive development. Patients and methods We present nine children, ages 4-16 years, with complicated craniofacial injury treated by a combined subcranial and intracranial approach. A continuous lumbar drainage was kept for several days, and prophylactic antibiotics and anti-convulsive medications were routinely given. A multidisciplinary approach including discussion before surgery about other surgical options (endoscopic extracranial and intracranial alone) were performed. Results None of the operated children had episodes of meningitis/leak after the combined approach, suggesting that appropriate sealing of the base of the skull has been achieved. There was no mortality, and the long-term follow-up showed good developmental and cosmetic results. Most of the children had significant brain contusions prior to surgery; however, these did not progress as minimal retraction was enabled by the extensive subcranial and intracranial approach. Conclusions Child's age, anatomy of the bone, extent of cranial injury, and clinical parameters should be seriously considered when choosing the technical methods as for sealing base of skull and reconstruction of facial/cranial bones. Young age does not seem to be a contraindication to the combined approach, thus, we recommend considering it in extensive base of skull fractures when concomitant cranial, maxillofacial, and orbital fractures coexist, as alternative options may not suffice in these cases

Craniofacial surgery for nonmelanoma skin malignancy: Report of an international collaborative study

AbstractBackground.This study examined the efficacy of craniofacial surgery (CFS) in treating locally advanced nonmelanoma skin cancer (NMSC).Methods.One hundred twenty patients who underwent CFS for NMSC were identified from 17 participating institutions. Patient, tumor, and treatment information was analyzed for prognostic impact on survival.Results.Surgical margins were negative in 74%, close in 3%, and involved in 23% of patients. Complications occurred in 35% of patients, half of which were local wound problems. Operative mortality was 4%. Median follow‐up interval after CFS was 27 months. The 5‐year overall survival (OS), disease‐specific survival (DSS), and recurrence‐free survival (RFS) rates were 64%, 75%, and 60%, respectively. Squamous cell histology, brain invasion, and positive resection margins independently predicted worse OS, DSS, and RFS.Conclusion.CFS is an effective treatment for patients with NMSC invading the skull base. Histology, extent of disease, and resection margins are the most significant predictors of outcome. © 2007 Wiley Periodicals, Inc. Head Neck, 200

The gene structure and expression of human ABHD1: overlapping polyadenylation signal sequence with Sec12

BACKGROUND: Overlapping sense/antisense genes orientated in a tail-to-tail manner, often involving only the 3'UTRs, form the majority of gene pairs in mammalian genomes and can lead to the formation of double-stranded RNA that triggers the destruction of homologous mRNAs. Overlapping polyadenylation signal sequences have not been described previously. RESULTS: An instance of gene overlap has been found involving a shared single functional polyadenylation site. The genes involved are the human alpha/beta hydrolase domain containing gene 1 (ABHD1) and Sec12 genes. The nine exon human ABHD1 gene is located on chromosome 2p23.3 and encodes a 405-residue protein containing a catalytic triad analogous to that present in serine proteases. The Sec12 protein promotes efficient guanine nucleotide exchange on the Sar1 GTPase in the ER. Their sequences overlap for 42 bp in the 3'UTR in an antisense manner. Analysis by 3' RACE identified a single functional polyadenylation site, ATTAAA, within the 3'UTR of ABHD1 and a single polyadenylation signal, AATAAA, within the 3'UTR of Sec12. These polyadenylation signals overlap, sharing three bp. They are also conserved in mouse and rat. ABHD1 was expressed in all tissues and cells examined, but levels of ABHD1 varied greatly, being high in skeletal muscle and testis and low in spleen and fibroblasts. CONCLUSIONS: Mammalian ABHD1 and Sec12 genes contain a conserved 42 bp overlap in their 3'UTR, and share a conserved TTTATTAAA/TTTAATAAA sequence that serves as a polyadenylation signal for both genes. No inverse correlation between the respective levels of ABHD1 and Sec12 RNA was found to indicate that any RNA interference occurred

Surgery versus Watchful Waiting in Patients with Craniofacial Fibrous Dysplasia – a Meta-Analysis

Fibrous dysplasia (FD) is a benign bone tumor which most commonly involves the craniofacial skeleton. The most devastating consequence of craniofacial FD (CFD) is loss of vision due to optic nerve compression (ONC). Radiological evidence of ONC is common, however the management of this condition is not well established. Our objective was to compare the long-term outcome of patients with optic nerve compression (ONC) due to craniofacial fibrous dysplasia (CFD) who either underwent surgery or were managed expectantly.We performed a meta-analysis of 27 studies along with analysis of the records of a cohort of patients enrolled in National Institutes of Health (NIH) protocol 98-D-0145, entitled Screening and Natural History of Fibrous Dysplasia, with a diagnosis of CFD. The study group consisted of 241 patients; 122 were enrolled in the NIH study and 119 were extracted from cases published in the literature. The median follow-up period was 54 months (range, 6-228 months). A total of 368 optic nerves were investigated. All clinically impaired optic nerves (n = 86, 23.3%) underwent therapeutic decompression. Of the 282 clinically intact nerves, 41 (15%) were surgically decompressed and 241 (85%) were followed expectantly. Improvement in visual function was reported in fifty-eight (67.4%) of the clinically impaired nerves after surgery. In the intact nerves group, long-term stable vision was achieved in 31/45 (75.6%) of the operated nerves, compared to 229/241 (95.1%) of the non-operated ones (p = 0.0003). Surgery in asymptomatic patients was associated with visual deterioration (RR 4.89; 95% CI 2.26-10.59).Most patients with CFD will remain asymptomatic during long-term follow-up. Expectant management is recommended in asymptomatic patients even in the presence of radiological evidence of ONC

Surgical Approaches to Resection of Anterior Skull Base and Paranasal Sinuses Tumors

Malignant tumours of the sinonasal tract comprise approximately 3% of the malignancies that arise in the upper aerodigestive tract. Approximately 10% of tumours that arise in the sinonasal tract originate in the ethmoid and/or frontal sinuses, and are likely to involve the anterior cranial base. The route of spread of tumours originating in the anterior skull base and paranasal sinuses is determined by the complex anatomy of the craniomaxillofacial compartments. These tumours may invade laterally into the orbit and middle fossa, inferiorly into the maxillary antrum and palate, posteriorly into the nasopharynx and pterygopalatine fossa, and superiorly into the cavernous sinus and brain. Recent improvements in endoscopic technology now allow the resection of the majority of benign neoplasms and some early malignant tumours with minor dural involvement. For advanced-stage malignant tumours and benign tumours with frontal bone involvement, the classical open approaches remain viable surgical techniques. In this paper, we review the open surgical resection approaches used for resections in the craniomaxillofacial area