The optic nerve in children with craniosynostosis

Optic nerve (ON) damage especially from papilloedema can be prevented with timely detection. The detailed ocular and radiographic status of 33 unoperated craniosynostosis (CS) was documented prospectively; the ON was also assessed by flash VER and A-scan echography for ON thickness. Fluorescein angiography (FA) was done for all suspected and early papilloedemas. ON involvement diagnosed in 10/33 showed highly significant correlations with multiple suture synostoses (p = 0.004) and exorbitism (p < 0.001). VER latency was increased in all 5 ON atrophies; and also in 1/5 papilloedemas without atrophic changes, urgent surgery in which reversed VER changes, and vision later improved from 6/60 to 6/9. FA was valuable for confirming the clinical diagnosis of the earliest papilloedemas; the FA also returned to normal with immediate therapy along with reversal of the clinical picture. ON echography especially helped in monitoring regression of disc oedema. Ours seems the first report especially in CS where a critical follow-up of all above parameters in 21 operated / 33 could objectively establish the role of each investigation in conjunction with careful clinical monitoring especially of the ON head. Such comprehensive evaluation should help reduce the blindness often seen in such children.     

Simulation and analysis of the electromyographic interference pattern in normal muscle. Part II: Activity, upper centile amplitude, and number of small segments

We have defined three new features of the electromyographic (EMG) interference pattern (IP): activity, upper centile amplitude (UCA), and number of small segments (NSS). These parameters were measured in simulated IPs constructed by adding together motor unit action potentials (MUAPs) recorded with a concentric needle EMG electrode. The activity increases linearly with the number of MUAP discharges to approximately 80% of its theoretical maximum value. The UCA correlates strongly with the peak-to-peak amplitude of the largest MUAP in the IP and the mean segment amplitude and does not depend on the discharge rate of the largest MUAPs. We infer that the UCA defines the upper limit of the peak-to-peak amplitude of the MUAPs contained in the IP. The NSS increases with the number of MUAP discharges, but reaches a constant value at higher MUAP discharge rates, probably because small amplitude MUAPs are masked by the large amplitude MUAPs. The potential value of these parameters in automated IP analysis is discussed.     

Intravenous pulse cyclophosphamide — a new regime for steroid-resistant minimal change nephrotic syndrome

The treatment of steroid-resistant minimal change nephrotic syndrome (MCNS) continues to pose a therapeutic challenge. We conducted a randomised prospective controlled trial to evaluate the efficacy of IV cyclophosphamide compared with oral cyclophosphamide in 13 children with biopsy-proven steroid-resistant MCNS. All 7 patients receiving IV cyclophosphamide achieved remission; this was sustained in 4 patients, while 3 relapsed. However, even these 3 patients subsequently became steroid sensitive. Of the 6 patients who received oral cyclophosphamide, 2 dropped out, 1 responded and 3 children continued to remain steroid resistant. The children who received IV cyclophosphamide had more sustained remissions, longer periods without proteinuria and fewer significant side effects; this was achieved at a lower cumulative dose.     

Cerebellar intra-axial dermoid cyst: a case of unusual location

Objects Intra-axial dermoid cysts are rare intracranial space occupying lesions, more so in the pediatric age group. Dermoid cysts account for about 0.2 to 1.8% of all intracranial tumors and are commonly located in the cisternal spaces, mainly in the cerebellopontine angle and parasellar cisterns. A purely intra-axial position as reported in this paper is quite exceptional. Materials and methods We report a case of intra-axial dermoid cyst located in the cerebellum that was surgically treated. The origin, clinical course, radiological features, pathological findings, and surgical treatment are discussed based on a review of the literature. Conclusion An intra-axial dermoid cyst is a rare entity and a preoperative diagnostic dilemma, as the radiologic findings overlap with many other intra-axial cerebellar masses. The case is reported in this paper to highlight the fact that, although rare, the possibility of an intra-axial mass being a dermoid cyst does exist.     

Solitary oncocytoma of the submandibular salivary gland in an adolescent female: A case report

We report a rare presentation of Oncocytoma of the submandibular salivary gland in an young adolescent female who presented with progressive swelling in the left submandibular region since childhood with pain for the past one month. Fine needle aspiration revealed Oncocytoma for which a conventional submandibular excision was done. There are few articles about the rare presentation of submandibular Oncocytoma and literature favors older population for this rarity. This case was unique because of its presentation in a young individual and there were no supporting published articles available till date.     

Early results with the thrust plate prosthesis in young patients with osteoarthritis of the hip

The purpose of this study was to evaluate the results of the Thrust Plate Prosthesis as a treatment option for osteoarthritis of the hip in young patients. Of the fifty patients (63 hips) reviewed, 31 (62%) were males and 19 (38%) females. Pre-operative diagnosis included primary osteoarthritis (23), developmental dysplasia (8), avascular necrosis (7), Perthes (4), post-traumatic arthritis (3), rheumatoid arthritis (2), ankylosing spondylitis (1), psoriatic arthropathy (1) and slipped upper femoral epiphysis (1). All components were implanted uncemented with metal-on-metal articulation. The average follow-up was 4.04 years (range 12 months-8.5 years). The mean age of the patients was 42.3 years (range 21-57 years). The mean pre-operative Harris Hip Score was 41.9 (range 12-89) and at final follow-up 89.91 (range 41-100). In 25 hips with > or = 5 yr follow-up, the average HHS at final follow-up was 84.5 (range 50-100). Complications included dislocation (2), transient sciatic nerve palsy (1), discomfort from lateral strap (2), implantation of wrong femoral head (1), revision (3 = 4.76%) and implant loosening (4) (6.35%). The thrust plate prosthesis is a useful alternative in young patients with hip arthritis and the results are comparable with other uncemented hip replacements. The added advantage is preservation of the proximal femoral bone stock, which can prove useful in future revisions.