Synovial chondromatosis of the temporomandibular joint with intracranial extension

A 52-year-old man presented with an extremely rare case of synovial chondromatosis in the temporomandibular joint (TMJ) with extension into the middle cranial fossa manifesting as swelling and exacerbation of pain. He had a long history of right TMJ disorders. Computed tomography and magnetic resonance imaging showed a mass in the right TMJ with extension into the intracranial part through the destroyed temporal skull base. The preoperative diagnosis was chondrosarcoma or osteosarcoma. The patient underwent surgery via combined trans-zygomatic temporal skull base and pre-auricular approaches and the mass was totally removed. Histological examination found an enormous number of closely packed loose bodies of various sizes, consisting of hyaline chondrocytes. The histological diagnosis was synovial chondromatosis. This rare lesion is difficult to discriminate from chondrosarcoma, so total removal is essential for correct diagnosis and cure.     

A pitfall of AirWay Scope--an experience of distinctive airway edema after palatal laceration caused by irWay Scope

Pentax AirWay Scope (AWS) is a new videolaryngoscope which allows indirect visualization of the vocal cords and provides a better laryngeal view compared with the conventional Macintosh laryngoscope. We report a female patient who developed distinctive upper airway edema after palatal laceration at the time of insertion of the AWS. She was scheduled for tympanoplasty. After anesthesia induction, there was technical difficulties in tracheal intubation with Macintosh laryngoscope (her Cormack grade was rated as 3), and we used the AWS, but could not obtain an appropriate view on the monitor. When the device was removed, we detected bleeding derived from the right palatal laceration. Following the aspiration of the blood, we could barely achieve tracheal intubation with a flexible fiberscope without hypoxemic episode. The upper airway including the arytenoid and vocal cords were distinctively edematous after the operation. Then, the tracheal tube was left for three days after the operation, to secure the airway until the reduction of airway edema. On the postoperative day 4, following the fibroscopic confirmation that the airway edema was reduced, the patient was extubated successfully. AWS should be used more carefully, especially at the time of insertion along the palate.     

Significance of ductal plate malformation in the postoperative clinical course of biliary atresia

Purpose

Ductal plate malformation (DPM) is one of the etiologic theories for the development of biliary atresia (BA). In this study, we investigated the significance of DPM in the postoperative clinical course of BA, especially as a predictive factor of jaundice clearance.

Methods

Between 1988 and 2005, 31 patients with uncorrectable BA underwent hepatoportoenterostomy and steroid therapy. Immunohistochemistry was used to characterize biliary structures using cytokeratin 19. Specimens were defined as DPM-positive if a concentric cellular arrangement was detected. This retrospective study included comparisons of preoperative characteristics, the postoperative jaundice period, and cumulative steroid doses between patients with and without DPM.

Results

Of the 31 patients with uncorrectable BA, 25 (80.6%) became jaundice-free. Ductal plate malformation was detected in 11 (35.5%) of the 31. Between the 2 groups, there were no differences in preoperative characteristics or in the postoperative jaundice-free rate. Among those who became anicteric, the postoperative jaundice period was 145.3 ± 69.9 days in the DPM-positive group (n = 9) and 81.8 ± 44.7 days in the DPM-negative group (n = 16) (P < .05). The total administered steroids were 149.7 ± 78.2 and 95.0 ± 60.2 mg/kg, respectively (P = .09).

Conclusion

Ductal plate malformation may disturb bile flow and require more steroid to improve bile drainage. Therefore, the presence of DPM in the liver predicts poor bile flow after hepatoportoenterostomy in infants with BA.     

A case of giant common carotid artery aneurysm associated with vascular Behçet disease: successfully treated with a covered stent

BACKGROUND: A carotid artery aneurysm associated with vascular Beh?et disease is extremely rare and often difficult to treat. In this article, we explore a definitive therapeutic strategy for pseudoaneurysm with vascular Beh?et disease. CASE DESCRIPTION: A 56-year-old man presented with swelling and a pulsatile subcutaneous mass of the left neck over a 6-month period. The diagnosis of vascular Beh?et disease had already been established from the history of right subclavian artery aneurysm, oral ulcerations, and inflammatory skin lesions. Radiologic examination revealed a giant left CCA aneurysm (6.5 x 5.5 cm) with partial thrombosis. Another asymptomatic aneurysm was found in the right ICA. Because mass effects due to aneurysmal rupture and growth rapidly progressed, we decided on radical treatment. The endovascular reconstruction of the carotid artery was selected instead of direct surgery because of skin and connective tissue disorders at the regional site. A covered stent (8 x 60 mm, Passager, Boston Scientific, Fremont, CA) was placed from the CCA to the ICA, covering the whole aneurysmal portion. Postoperatively, the cervical mass remarkably reduced in size, and the patient's symptoms dramatically improved. The left carotid artery was patent at 12-month follow-up. CONCLUSIONS: A covered stent is very useful in repairing arteries with pseudoaneurysm, particularly in cases unsuitable for direct surgery with parent artery occlusion. The influence of the foreign body at the inflammatory lesion and long-term patency of covered stents should be discussed.     

Irinotecan and cisplatin with concurrent split-course radiotherapy in locally advanced nonsmall-cell lung cancer: a multiinstitutional phase 2 study

BACKGROUND: The purpose was to determine the efficacy and toxicity of irinotecan and cisplatin with concurrent split-course thoracic radiotherapy (TRT) in locally advanced nonsmall-cell lung cancer. METHODS: Fifty patients fulfilling the following eligibility criteria were enrolled: chemotherapy-naive, good performance status (PS, 0-2), age <75, stage III, and adequate organ function. The patients received irinotecan 60 mg/m(2) intravenously on Days 1, 8, and 15, and cisplatin 80 mg/m(2) intravenously on Day 1 in the first group. The doses were reduced to 50 and 60 mg/m(2), respectively, in the second group. Two cycles of chemotherapy were repeated every 4 weeks. Split-course thoracic radiotherapy of 2 Gy/day commenced on Day 2 of each chemotherapy cycle, with 28 and 32 Gy administered in the first and second cycles, respectively. RESULTS: Fifty patients were eligible and 48 (16 in the first, 32 in the second group) patients were assessable for response, toxicity, and survival. The overall response was 83% (95% confidence interval [CI], 70%-93%). Grade 4 leukopenia, neutropenia, grade 3 or 4 diarrhea, pneumonitis, esophagitis, and fatigue occurred in 21%, 48%, 19%, 10%, and 19%, respectively. The median time to progression was 8.2 months. The median overall survival time and the 2- and 5-year survival rates were 20.1 months, 47.1%, and 17.1%, respectively. In subgroup analysis, grade 4 neutropenia, grade 3 or 4 diarrhea, the overall response, and the median survival times of the first/second groups were 63%/41%, 19%/19%, 75%/88%, and 13.1/33.4 months, respectively. CONCLUSIONS: This combined modality of irinotecan and cisplatin with concurrent TRT is active and further investigations are warranted at the second group dose level.     

Case of immune complex crescentic glomerulonephritis with consistently high titers of MPO-ANCA for 6 years

A male patient, now 65 years old, experienced fever, hemoptysis, and respiratory failure about six years ago. Soon thereafter, he developed rapid progressive renal dysfunction with pulmonary hemorrhage and positive findings for MPO-ANCA. We commenced methylprednisolone pulse (MP) therapy followed by oral prednisolone (PSL) and intravenous cyclophosphamide (CY) for the treatment of ANCA-associated microscopic polyangiitis (MPA). Therapeutic efficacy was obtained comparatively rapidly. Light microscopic findings of a percutaneous renal biopsy demonstrated focal necrotizing and crescentic glomerulonephritis. Immunofluorescent microscopy indicated diffuse deposition of IgG and C3 along the periphery of the tufts and in the mesangium. On the basis of these findings, the condition was diagnosed as immune complex crescentic glomerulonephritis associated with MPO-ANCA. MPO-ANCA titers were high (714 EU) at onset and remained high (250-450 EU) over the ensuing 6 years with oral administration of PSL 5 mg. Though his condition remitted completely, his MPO-ANCA titers recently increased to above 600 EU once more. We conducted a follow-up renal biopsy to ascertain if the fluctuation of MPO-ANCA titers reflected an early stage of relapse. Light microscopic findings of the biopsied tissue revealed no signs of necrosis or crescentic formation of the glomeruli. Immunofluorescent microscopic findings were negative. The elevated MPO-ANCA titers were not valuable for the early prediction of relapse in our case, and the immune complex may have played an important role. When judging relapse and remission in ANCA-associated glomerulonephritis, it is important to evaluate the overall clinical findings and histopathological findings in addition to the serial ANCA titers.     

C4d Immunohistochemistry in glomerulonephritis with different antibodies

Background The presence of C4d in the kidney is generally detected particularly for the diagnosis of antibody-mediated rejection in renal transplants. In frozen sections of immunofluorescence (IF) staining with anti-C4d monoclonal antibodies (mAbs), we noted intrinsic C4d deposition even in normal glomeruli though their pathogenic or an intrinsic role is unkown. An anti-C4d polyclonal antibody (C4dpAb), which is suitable for paraffin immunoperoxidase (IP) staining, is less used than mAbs, and it has demonstrated that intrinsic C4d is not evident. To establish a stable and reproducible procedure for C4d detection with the C4dpAb and to determine the staining characteristics of it, the present study aimed to test whether the method was comparable with IF with a mAb. Methods We compared the C4dpAb with the mAb in adjacent sections of human diseased kidneys, and then compared IP with IF of C4dpAb. Two ways of antigen retrieval was examined for IP. Results On comparing the two antibodies for glomerular staining with IF, we found that the pattern and intensity (C4dpAb showed intrinsic C4d with IF) were similar. In addition, C4dpAb staining with IP and IF demonstrated that the intrinsic staining in the normal glomerulus was mostly undetectable by IP, whereas IF showed distinct staining. Likewise, C4d deposition with IP in some cases was apparently weaker than that on IF, suggesting that this deposition is not intrinsic but indicates pathogenic complement activation. Conclusions The advantage of the C4dpAb for immunohistochemistry is of value for reconsidering the role of C4d in glomerular diseases.