Basal encephalocele: surgical strategy and functional outcomes in the Tokyo experience

OBJECTIVEThe basal encephalocele (BEC) is the rarest form of encephalocele, with an incidence of about 1/35,000 live births. The incidence of its subtype, sphenoidal BEC, is even lower at about 1/700,000 live births. The aim of this study was to propose the optimal surgical approach to repairing BEC, with special attention to the reconstruction of the skull base bone defect.METHODSFourteen consecutive pediatric patients with BEC who underwent surgical repair between March 2004 and March 2020 (10 boys and 4 girls, age 25 days to 7 years, median age 4 months) were enrolled. The follow-up period of the surviving patients ranged from 53 to192 months (mean 119.8 months). The patient demographics, BEC subtypes, preoperative clinical condition, radiographic findings, surgical procedures, and postoperative course were retrospectively analyzed.RESULTSThere were 4, 8, and 2 cases of sphenoidal BEC, sphenoethmoidal BEC, and ethmoidal BEC, respectively. The size of the bone defect was small in 3 patients, medium in 7, and large in 4 patients. All the patients with sphenoethmoidal and ethmoidal BEC showed associated congenital anomalies other than cleft palate. In total, 25 operations were performed. Two patients underwent multiple operations, whereas the remaining 9 patients received only 1 operation. The transoral transpalatal approach was the initial procedure used in all 14 patients. The transfrontobasal approach was applied as an additional procedure in 2 patients and as part of a 1-stage combined operation in 2 patients. Autograft bone alone was used for skull base reconstruction in 17 early operations. A titanium mesh/plate was used in the remaining 8 operations without any perioperative complications. All BECs were successfully repaired. Three patients died during the clinical course due to causes unrelated to their surgery. All but one of the surviving patients started growth hormone replacement therapy before school age.CONCLUSIONSBased on the authors’ limited experience, the key to successful BEC repair involves circumferential dissection of the BEC and a firm reconstruction of the skull base bone defect with a titanium plate/mesh. The transoral transpalatal approach is a promising, reliable procedure that may be used in the initial operation. When a cleft palate is absent, transnasal endoscopic repair is recommended. The transfrontobasal approach should be reserved for cases with a huge BEC and other anomalies. Long-term prognosis is apparently favorable in survivors.

Assisted resection of lateral extension of juvenile nasopharyngeal angiofibroma using a sphenopalatine fossa dissector

BackgroundJuvenile nasopharyngeal angiofibroma often attaches firmly to the adjoining bony region around the sphenopalatine foramina–sphenopalatine fossa–pterygomaxillary fissure. This can result in hourglass-shaped constriction and predispose to incomplete resection (residual disease) with a transpalatal approach. This paper describes attempts to address this ‘inaccessible’ area with a novel instrument, used since 2012.MethodsMeasurements of the sphenopalatine foramen, nasal septum, posterior nasopharyngeal wall and hard palate were undertaken in 20 skulls and 10 computed tomography scans (lateral extension). A device was designed (in terms of angulation and length) following several trials with malleable wire. A search of patents was also undertaken. Recurrence rates were compared in cases of device use and non-use.ResultsThe novelty of the sphenopalatine fossa dissector was established and the device was patented. This device has significantly improved our 17.59 per cent recurrence rate of the past 4 decades; of 63 cases over 3 years, there were only 3 recurrences and 2 residual disease cases. Findings of our previous studies with or without the device are compared.ConclusionExisting evidence supports the incorporation of this inexpensive instrument in the armamentarium for resecting lateral extension of juvenile nasopharyngeal angiofibroma during a transpalatal approach.

In defence of transpalatal, transpalatal-circumaxillary (transpterygopalatine) and transpalatal-circumaxillary-sublabial approaches to lateral extensions of juvenile nasopharyngeal angiofibroma

Background:Juvenile nasopharyngeal angiofibroma often presents with lateral extensions. In countries with limited resources, selection of a cost-effective and least morbid surgical approach for complete excision is challenging.Methods:Sixty-three patients with juvenile nasopharyngeal angiofibroma, with lateral extensions, underwent transpalatal, transpalatal-circumaxillary (transpterygopalatine) or transpalatal-circumaxillary-sublabial approaches for resection. Clinico-radiological characteristics, tumour volume and intra-operative bleeding were recorded.Results:The transpalatal approach was suitable for extensions involving medial part of pterygopalatine fossa; transpalatal-circumaxillary for extensions involving complete pterygopalatine fossa, with or without partial infratemporal fossa; and transpalatal-circumaxillary-sublabial for extensions involving complete infratemporal fossa, even cheek or temporal fossa up to zygomatic arch. Haemorrhage was greatest with the transpalatal-circumaxillary-sublabial approach, followed by transpalatal approach and transpalatal-circumaxillary approach (1212, 950 and 777 ml respectively). Tumour size (volume) was greatest with the transpalatal-circumaxillary approach, followed by transpalatal-circumaxillary-sublabial approach and transpalatal approach (40, 34 and 29 mm3). There was recurrence in three cases and residual disease in two cases. Long-term morbidity included small palatal perforation (n = 1), trismus (n = 1) and atrophic rhinitis (n = 2).Conclusion:These modified techniques, performed with endoscopic assistance under hypotensive anaesthesia, without embolisation, offer a superior option over other open procedures with regard to morbidity and recurrences.

Surgical treatment of transsphenoidal encephaloceles: transpalatal versus combined transpalatal and transcranial approach

Object Transsphenoidal encephalocele (TSEC) is a rare developmental anomaly with herniation of neural elements including pituitary gland or optic apparatus, through a defect of sphenoid bone. To prevent obstructive dyspnea, endocrine dysfunction, or visual disturbance, surgical correction is usually performed. However, the optimal operative approach has not been determined yet. The authors present their surgical experiences with transpalatal, transcranial, or combined approach. Methods The authors retrospectively analyzed surgical results in patients with TSEC. The charts were reviewed for demographic data, clinical presentation, surgical therapy, and clinical outcomes. Results Seven consecutive patients with TSEC were surgically treated between March 2004 and February 2012. The mean age was 23.9 months (range 2–69 months). Four patients with TSEC who had a bone defect within the sphenoid bone were successfully treated using a transpalatal approach. Two patients with an extensive sphenoethmoidal bone defect that was treated either by a transpalatal or transcranial approach experienced several relapses with a prolapse of the encephalocele. One patient with an extensive sphenoethmoidal bone defect was successfully treated by combined transpalatal and transcranial approach without relapse for 3.6 years. Conclusions The combined transpalatal and transcranial approach is useful for TSEC with an extensive sphenoethmoidal bone defect to dissect the sac and expose the bone defect circumferentially. If dissection is not sufficient, relapse with a prolapse of the encephalocele can ensue. The transpalatal approach is sufficient for TSEC in which a bone defect is limited within the sphenoid bone.

Choanal Atresia- A Clinical Study and Review of Literature

Choanal atresia is a developmental anomaly where there is failure of communication of the nasal cavity to the nasopharynx. The atresia can be membranous or bony in nature, but may be mixed as is seen in most of the cases. Bilateral choanal atresia is an emergency condition and newborns can have severe respiratory distress and cyanosis at birth. Failure to pass an 8 F catherter through the nasal cavity more than 5.5 cm from the alar rim points to the presence of choanal atresia. In emergency, bilateral choanal atresia is managed with an oropharyngeal airway. Flexible nasal endoscopy and CT scan can confirm the diagnosis of choanal atresia. Surgery is the definitive treatment which can be done either by transnasal or transpalatal approach. The transnasal endoscopic route is currently the preferred procedure and can be performed as a minimally invasive procedure. This has less complications and a high success rate. We studied four cases of unilateral choanal atresia presented to our hospital, with respect to the etiological factors, clinical features and management.