照井 慶太

Congenital esophageal stenosis (CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic review to assess outcomes of the treatment for CES, especially the role of endoscopic modalities. A total of 144 literatures were screened and reviewed. CES was categorized in fibromuscular thickening, tracheobronchial remnants (TBR) and membranous web, and the frequency was 54%, 30% and 16%, respectively. Therapeutic option includes surgery and dilatation, and surgery tends to be reserved for ineffective dilatation. An essential point is that dilatation for TBR type of CES has low success rate and high rate of perforation. TBR can be distinguished by using endoscopic ultrasonography (EUS). Overall success rate of dilatation for CES with or without case selection by using EUS was 90% and 29%, respectively. Overall rate of perforation with or without case selection was 7% and 24%, respectively. By case selection using EUS, high success rate with low rate of perforation could be achieved. In conclusion, endoscopic dilatation has been established as a primary therapy for CES except TBR type. Repetitive dilatation with gradual step-up might be one of safe ways to minimize the risk of perforation.

The aim of this paper is to investigate the clinical courses of patients with biliary atresia (BA) during neonatal period. We examined 19 patients with BA, who underwent blood tests including direct bilirubin (D-Bil) within 20 days of age, in 3 tertiary hospitals in Japan. The first blood sample was collected at 8.4±6.5 days of age. The acholic stool was observed within 2 weeks of age in 16 cases (84.2%). Decrease of T-Bil was observed in all the subjects, with a range of reduction of 6.5±3.3 mg/dL, from 10.4±7.5 to 29.8±9.1 days of age. Decrease of D-Bil was also observed in 17 out of 19 cases (89%), with a range of reduction of 1.1±1.0 mg/dL, from 15.5±8.0 to 24.9±9.6 days of age. A significant decrease of D-Bil was observed in 2 cases of biliary atresia splenic malformation syndrome. We therefore conclude that clinicians treating icteric infants should not exclude a diagnosis of BA even if the level of D-Bil has a declining tendency.

Microvascular decompression is now a standard surgical technique for the treatment of trigeminal neuralgia. However, it is occasionally difficult to expose the trigeminal nerves because of the high anatomical variety of vascular or bony structures in the posterior fossa. We reported the case of a 59-year-old woman with trigeminal neuralgia whose site of neurovascular compression could not be observed in microvascular decompression. On approaching the trigeminal nerve, the suprameatal tubercle was so prominent that it prevented adequate visualization of the nerve tract. After drilling out the tubercle concealing the trigeminal nerve behind it, we exposed the nerve entirely and subsequently decompressed it from the superior cerebellar artery. Retrospectively, the suprameatal tubercle was found 3mm high above the posterior surface of the petrous bone. Then, we analyzed the height of suprameatal tubercles in 106 patients who underwent three-dimensional CT of the skull. Mean values of the suprameatal tubercles were 1.4-1.7mm in height, and 5.2% of them were higher than 3mm. The result suggested the high morphological variety of the petrous bone. We emphasize the importance of presurgical evaluation of the petrous bone in trigeminal neuralgia, because the neurovascular compression site may not be exposed sufficiently by the suprameatal tubercle in approximately 5% of the patients.

Cerebral aneurysms arising from fenestration of the horizontal (A(1)) segment in the anterior cerebral artery (ACA) are very rare. In this paper, we report our case, discuss radiological features, and review previous cases. A 70-year-old male was referred to our hospital presenting with memory disturbance. His unruptured cerebral aneurysm in the A(1) segment was incidentally found by magnetic resonance angiography (MRA). Three dimensional computed tomographic angiography (3D-CTA) demonstrated this aneurysm arising from fenestration of the A(1) segment. Surgical neck clipping was performed via the pterional approach, while sacrificing one pair of the A(1) segment. The patient's post operative course was uneventful. Only 14 cases with an aneurysm arising from fenestration of the A(1) segment have been reported previously. In the present case, 3D-CTA was very useful for finding out where the aneurysm arose from, and we also had to be careful about perforating arteries from the A(1) segment during the surgery.