Combined trans-sphenoidal and simultaneous trans-ventricular-endoscopic decompression of a giant pituitary adenoma: case report

Objective  This is the first report of the simultaneous combined use of trans-sphenoidal and trans-ventricular-endoscopic route for decompression of a giant pituitary adenoma. Method  A 38 year old man presented to us with symptoms of raised intracranial pressure along with visual and hypothalamic disturbances. The CT scan revealed destruction of the sella by a large (5 × 3.5 × 2.5 cm) well defined enhancing mass in the sella and suprasellar region extending laterally up to the cavernous sinuses and both carotid arteries and superiorly into the lumen of the 3rd ventricle producing obstructive hydrocephalus. On T2WI of the non-contrast MRI scan the mass was iso-intense to grey matter suggesting the possibility of a firm nature of the adenoma. The tumour was first approached by the standard trans-sphenoidal route and as predicted from the pre-operative MRI, the tumour was found to be firm and not amenable to suction. After decompression of the intra-sellar part of the tumour, the intracranial pressure was raised in an attempt to make the remainder of the tumour descend into the sella but without success. The suprasellar part of the tumour was then simultaneously addressed via a trans-ventricular-endoscopic route but the firm tumour did not yield to endoscopic instruments viz. biopsy forceps, angiographic catheter and electrosurgical probes. It was then gently pushed down towards the sella and decompressed piecemeal by using trans-sphenoidal instruments. The sellar cavity was reconstructed using fat, fascia lata graft and a piece of septal bone. Results  Post-operatively, the patient showed a remarkable improvement of his symptoms of raised intracranial pressure, hypothalamic dysfunction and visual disturbances. Follow-up imaging at 2 months and 1 year, did not show any residual or recurrent tumour. Conclusions  This novel technique of the combined trans-sphenoidal and simultaneous trans-ventricular-endoscopic approach is a viable option for patients with giant fibrous pituitary adenoma when the tumour is not yielding to the trans-sphenoidal route alone.     

Late relapse of germ cell tumors

Objective  To assess the main characteristics of late relapsing malignant germ cell tumors (MGCTs). These tumors are rare and occur by definition 2 years or later after successful treatment. Methods  We present relevant literature on relapsing MGCT in order to highlight the following issues: incidence, impact of initial treatment on the subsequent risk of late relapse, treatment, and survival. Results  A pooled analysis of 5,880 patients with MGCT revealed late relapses in 119 of 3,704 (3.2%) and in 31 of 2,176 (1.4%) patients with non-seminoma and seminoma, respectively. The retroperitoneal space is the predominant site of relapse in both histological types. The initial treatment is important for the risk and localization of late relapses. Patients with single site teratoma are usually cured by surgery alone, whereas viable MGCT or teratoma with malignant transformation may require multimodal treatment with chemo- and/or radiotherapy as well as surgery. Surgery is the most important part in the treatment of late relapses. Salvage chemotherapy should, if feasible, be based on a representative biopsy. Five-year cancer-specific survival is above 50% in the recent large series and reaches 100% in case of single site teratoma. Conclusions  Treatment of late relapsing MGCT patients is challenging and should be performed in experienced centers only. Referral of late relapsing patients to high-volume institutions ensures the best chances of cure and enables multimodal treatment, and contributes to increased knowledge of tumor biology as well experience with the clinical course of these patients.     

Slit diaphragm dysfunction in proteinuric states: identification of novel therapeutic targets for nephrotic syndrome

Several recent studies have demonstrated that the slit diaphragm of the glomerular epithelial cell (podocyte) is the structure likely to be the principal barrier in the glomerular capillary wall. Nephrin identified as a gene product mutated in congenital nephrotic syndrome located at the outer leaflet of plasma membranes of the slit diaphragm. The anti-nephrin antibody is capable of inducing massive proteinuria, which indicates that nephrin is a key functional molecule in the slit diaphragm. Expression of nephrin was reduced in glomeruli of minimal change nephrotic syndrome. Some recent studies demonstrated that podocin, CD2-associated protein and NEPH1 are also functional molecules in the slit diaphragm, and their expressions are altered in membranous nephropathy and also in focal glomerulosclerosis. These observations suggested that the alteration of the molecular arrangement in the slit diaphragm is involved in the development of proteinuria in several kinds of glomerular diseases. Recent studies of our group have demonstrated that type 1 receptor-mediated angiotensin II action reduced the expression of the slit diaphragm-associated molecules and that type 1 receptor blockade ameliorated proteinuria by preventing the function of angiotensin II on the slit diaphragm. By the subtraction hybridization techniques using glomerular cDNA of normal and proteinuric rats, we detected that synaptic vesicle protein 2B and ephrin B1 are involved in the maintenance of the barrier function of the slit diaphragm. Presented at the 36th Eastern Regional Meeting of the Japanese Society of Nephrology.     

Cardiac valvular surgery in dialysis patients: comparison of surgical outcome for mechanical versus bioprosthetic valves

Purpose  There has been a changing preference for bioprosthetic valves over mechanical valves in dialysis patients, but there is still much controversy. We reviewed our 17-year experience and assessed the influence of prosthesis choice. Methods  From 1990 to 2007, a total of 63 consecutive dialysis patients who underwent valvular surgery (64 operations including one reoperation) at our hospital were retrospectively reviewed. The mean age of the patients was 58.3 ± 9.0 years. The reasons for dialysis were glomerulonephritis (n = 32) and diabetes (n = 10). The major preoperative diagnosis was aortic stenosis (n = 44). The surgical procedures included aortic valve replacement (n = 44), mitral valve replacement (n = 7), double valvular replacement (n = 7), and mitral valve repair (n = 5). Prostheses for valve replacement were mechanical valves (n = 37) or bioprosthetic valves (n = 22). Follow-up was accomplished in 95.2%, and the mean follow-up period was 49 months. Results  Actuarial survivals at 1, 5, and 10 years were 85%, 64%, and 45% respectively. Freedom from cardiovascular events at 1 and 5 years was 61% and 41%, respectively. Mechanical valve patients had significantly higher early mortality than bioprosthetic valve patients (P = 0.03). However, both mechanical and bioprosthetic valve patients had similar survival and event-free rates (P = 0.87 and P = 0.27, respectively) in the midterm results. The mechanical group had a higher rate of bleeding events. There was no structural valve deterioration up to the 5-year follow-up. Conclusion  The choice of prosthesis did not influence the surgical outcome except for early mortality. Careful consideration of preventive measures against bleeding is important, and prosthesis selection should be based on the patient’s profile as well as the criteria for nondialysis patients.     

Radiosurgery for large cerebral arteriovenous malformations

Background  Radiosurgery is an effective treatment option for patients with small to medium sized arteriovenous malformations. However, it is not generally accepted as an effective tool for larger (>14 cm³) arteriovenous malformations because of low obliteration rates. The authors assessed the applicability and effectiveness of radiosurgery for large arteriovenous malformations. Method  We performed a retrospective study of 46 consecutive patients with more than 14 ml of arteriovenous malformations who were treated with radiosurgery using a linear accelerator and gamma knife (GK). They were grouped according to their initial clinical presentation—17 presented with and 29 without haemorrhage. To assess the effect of embolization, these 46 patients were also regrouped into two subgroups—25 with and 21 without preradiosurgical embolization. Arteriovenous malformations found to have been incompletely obliterated after 3-year follow-up neuroimaging studies were re-treated using a GK. Findings  The mean treatment volume was 29.5 ml (range, 14.0–65.0) and the mean marginal dose was 14.1 Gy (range, 10.0–20.0). The mean clinical follow-up periods after initial radiosurgery was 78.1 months (range, 34.0–166.4). Depending on the results of the angiography, 11 of 33 patients after the first radiosurgery and three of four patients after the second radiosurgery showed complete obliteration. Twenty patients received the second radiosurgery and their mean volume was significantly smaller than their initial volume (P = 0.017). The annual haemorrhage rate after radiosurgery was 2.9% in the haemorrhage group (mean follow-up 73.3 months) and 3.1% in the nonhaemorrhage group (mean follow-up 66.5 months) (P = 0.941). Preradiosurgical embolization increased the risk of haemorrhage for the nonhaemorrhage group (HR, 28.03; 95% CI, 1.08–6,759.64; P = 0.039), whereas it had no effect on the haemorrhage group. Latency period haemorrhage occurred in eight patients in the embolization group, but in no patient in the nonembolization group (P = 0.004). Conclusions  Radiosurgery may be a safe and effective arteriovenous malformation treatment method that is worth considering as an alternative treatment option for a large arteriovenous malformation.     

Posterior lumbar interbody fusion versus posterolateral fusion in spondylolisthesis: a prospective controlled study in the Han nationality

In this prospective study, our aim was to compare the clinical outcome of posterior lumbar interbody fusion (PLIF) and posterolateral fusion (PLF) in spondylolisthesis. A total of 138 patients with spondylolisthesis were randomly assigned to two groups: those operated on with pedicle screw fixation and posterior lumbar interbody fusion by autografting (PLIF), and those operated on with pedicle screw fixation and posterolateral fusion by autografting (PLF). The patients were followed-up for four years. Clinical evaluation was carried out using the Oswestry disability index (ODI) and pain index (VAS). Radiography was performed preoperatively and postoperatively to assess the fusion. Both surgical procedures were effective, but the PLF group showed more complications related to hardware biomechanics. There was no significant statistical difference in clinical and functional outcome in the two groups. The PLIF group presented a better fusion rate than the PLF group.     

Clinical and radiographic reports following cervical arthroplasty: a 24-month follow-up

We reviewed patients with cervical disc prosthesis replacement for single-level cervical disc disease to evaluate its clinical effect and maintenance of cervical spine motion. Fifteen patients underwent Bryan artificial cervical disc replacement and were followed-up for at least 24 months. No neurological or vascular complications were observed during or after operation. JOA, VAS, and NDI scores showed statistical significant improvement in our follow-up. The procedure achieved an 87% (13/15) satisfactory rate at 24-month evaluations according to Odom's criteria. The range of motion (ROM) of the cervical spine, treated segment, adjacent segment, and functional spinal unit (FSU) decreased at early follow-up, but they recovered to the preoperative level at 12- and 24-month follow-up. Also, preoperative lordosis of the cervical spine and FSU were not only maintained but also even improved during the 24-month follow-up. No obvious degeneration of adjacent discs were found at MRI. There were no cases of prosthesis subsidence or extrusion. The cervical disc prosthesis showed a good clinical outcome; it also restored ROM of the cervical spine and reestablished cervical curvature in our 24-month follow-up. But to be sure of its long term effect, a longer follow-up is needed.     

Custom prosthetic replacement for distal radial tumours

We analysed the results of 24 cases of aggressive benign and malignant tumours of the distal radius treated by resection and prosthetic replacement between 1995 and 2006. Patient ages ranged from 18 to 74 years, averaging 33 years; 18 were males. Recurrent giant cell tumour was the most common tumour. The prosthesis used was a bipolar hinge custom mega prosthesis manufactured locally. Average follow-up was 78 months. The average Musculoskeletal Tumor Society (MSTS) functional score achieved was 75%. The ten-year prosthesis survival was rate 87.5%. Infection was the most common complication.

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Resume  Prothèses sur mesure dans les tumeurs du radius distal. Indications de remplacement. Nous avons analysé les résultats de 24 cas de tumeurs malignes et bégnines agressives de l’extrémité distale du radius traitées par résection et prothèses entre 1995 et 2006. Les patients étaient agées de 18 à 74 ans avec un age moyen de 33 ans dont- 18 étaient des hommes. La cause la plus fréquente a été la récidive de tumeur à cellules géantes. La prothèse utilisée était une prothèse bipolaire à charnière sur mesure fabriquée localement. Le suivi moyen a été de 78 mois. Le score moyen MSTS après traitement était de 75%, la courbe de survie à 10 ans de 87,5%. La complication la plus fréquente a été l’infection.

     

Post-chemotherapy nerve-sparing retroperitoneal lymph node dissection for advanced germ cell tumor

Objective:   To report our experience with post-chemotherapy nerve-sparing retroperitoneal lymph node dissection (RPLND) for advanced germ cell tumor (GCT).
Methods:   Between 1994 and 2008, 92 patients with advanced GCT underwent RPLND after multiple treatments with systemic chemotherapy at our institution. A nerve-sparing RPLND was carried out in 78 patients (84.8%; median age 32 years). Of them, 19 had a seminoma and 59 had a non-seminoma.
Results:   Lumbar splanchnic nerves controlling ejaculatory function were macroscopically preserved during RPLND. Bilateral and unilateral lumbar splanchnic nerves were preserved in 40 patients and 38 patients, respectively. Sixty-five patients could be evaluated for ejaculation. Fifty-four patients (83.1%) achieved antegrade ejaculation with a median postoperative interval of 3 months (range: 1–10 months). Twenty-eight patients (28/30: 93.3%) and 26 patients (26/35: 74.3%) undergoing bilateral and unilateral nerve-sparing RPLND had antegrade ejaculation, respectively ( P  = 0.041). Only two patients (2.6%) had mediastinal and retroperitoneal recurrences during a median follow-up of 42 months (range: 1–138 months), respectively. However, these patients were cured by chemotherapy and surgery.
Conclusions:   Post-chemotherapy nerve-sparing RPLND preserves ejaculatory function in the majority of patients with advanced GCT without increasing the risk of local recurrence.