Transoral Odontoidectomy - Our Experience in A Limited-Resource Setup.

BACKGROUND: Although posterior procedures are used for most cases of basilar invagination, transoral decompression is an important tool in complex deformities with severe degeneration and neural compression. METHODS: Consecutive patients who underwent transoral odontoidectomy for basilar invagination and atlantoaxial dislocation at the neurosurgical unit of Lady Reading Hospital Peshawar between June 2016 and January 2022 were retrospectively included. Preoperative and postoperative neurological, clinical, and radiological parameters were recorded and compared. RESULTS: Of the 33 patients included in this study, 22 were men and 11 were women. The mean age was 23.4 years ±8.4 years (mean ± standard deviation). The most common presenting symptoms were gait abnormalities and neck pain. A total of 28 patients were treated for the first time, while 5 patients had prior posterior fusion. The mean distance of the tip of the dens above the chamberlain line was 8.9 ± 4.2 mm, while the mean anterior atlantodental interval was 6.5 ± 2.1 mm. Seven patients had an anomalous course of vertebral artery on at least one side. A total of 28 patients improved significantly, while 5 patients did not improve at the follow-up. The mean follow-up was 8.5 months ±6.3 (mean ± standard deviation). The mean Nurick and Modified Japanese Orthopedic Association scores improved postoperatively (P < 0.05). Three patients underwent reoperation including one with vertebral artery injury. Three patients underwent tracheostomy. CONCLUSIONS: With proper patient selection, transoral odontoidectomy can be safely performed in a limited-resource setting.

Clinical presentation and outcome of autosomal dominant polycystic kidney disease in Pakistan: a single center experience.

OBJECTIVE: To delineate clinical presentation and outcome of Autosomal Dominant Polycystic Kidney Disease (ADPKD) in terms of need for renal replacement therapy of kidney transplantation in Pakistani patients. METHODS: Patients with ADPKD were identified using strict clinical criteria. Medical charts were evaluated retrospectively for initial presenting complaints, co-morbids, family history of ADPKD, any palpable mass on abdominal examination, cardiac examination for any abnormal finding and use of any anti-hypertensive drugs. Laboratory parameters were assessed. Chi square and Logistic regression analyses at 95% CI were used for statistical significance. A p value of less 0.05 was considered statistically significant. RESULTS: A total of 56 patients fulfilled our criteria of ADPKD. There were 40 (71.4%) males and 16 (28.6%) females in our study. The mean age at the time of diagnosis of ADPKD was 47.0 +/- 14.5 years. Mean follow up period for all patients was 7.6 +/- 4.2 years. Most common form of presentation was hypertension in 38 (67.9%) patients. Kidneys were palpable in 33 (58.9%), liver in 16 (28.6%) and spleen in 6 patients (10.7%). Microscopic haematuria was observed in 38 (67.8%) patients while gross haematuria was present in 10 (17.9%) patients. The murmur of mitral valve prolapse was found in 10 patients on clinical examination which was later confirmed on transthoracic echocardiography (TEE). On MRI/MRA 2 (3.6%) patients each had berry aneurysm and AV malformations. Three patients (5.4%) received renal transplant and 19 (33.9%) patients were dialysis dependent at the end of study. A total 11 (20%) were lost to follow up. Two patients (3.5%) died during six years follow up. Male sex and uncontrolled hypertension were most important predictors of poor prognosis (p < 0.03 and < 0.048 respectively). CONCLUSION: Pre-symptomatic patients with ADPKD should be monitored with blood pressure measurements and assessment of their renal function. The advantages of such monitoring include the ability to prevent or control infection and hypertension, to identify potential kidney donors from among the family, to offer advice on marriage and childbearing, and to provide prenatal diagnosis.

Pre-operative renal function and selective renal vein renin levels as markers of favourable outcome in renovascular hypertension.

OBJECTIVE: To evaluate factors that affect outcome following revascularization in patients with renovascular hypertension. METHODS: This study was conducted at the Aga Khan University Hospital. It included all the patients diagnosed to have Renovascular hypertension confirmed by renal angiography between July 1996 to September 2000. Using ANOVA (Analysis of variance) and paired-sample t-test, several preoperative factors were evaluated for their ability to predict postoperative improvement of blood pressure and renal function. For descriptive purpose patients were divided into cured, improved and failure groups. RESULTS: Of the total 15 patients, 9 were males and 6 were females. Before the surgical procedure, 13 patients were on 3 or more antihypertensive drugs. Eleven patients received vein grafts, three patients had PTFE (polytetraflouroethyline) grafts while one patient underwent angioplasty and stenting. The patients had a median follow-up period of nine months with a range of 2 to 84 months. Five were completely cured, as they became normotensive without antihypertensive therapy after operation, whereas four showed marked improvement in BP control postoperatively, requiring two antihypertensive drugs. Six patients showed no improvement requiring 3 or more drugs. Extended cure or improvement of renovascular hypertension was achieved in nine patients. Normal preoperative serum creatinine level, high preoperative unstimulated peripheral renin levels and renal vein renin ratio of at least 1.75:1 were the most significant predictive factors for favorable outcome. CONCLUSION: This study confirms the long term effectiveness and safety of renovascular reconstruction in the relief of severe hypertension. The results further suggest that normal preoperative renal function, high renal vein renin ratio and high preoperative unstimulated peripheral renin levels are associated with the most favourable postoperative outcome (p <0.01). Preoperative severity and duration of hypertension and degree of disparity in kidney sizes do not affect the overall results in terms of postoperative improvement in renal function and blood pressure control (p=0.734).

Do clinical manifestations of Systemic Lupus Erythematosus in Pakistan correlate with rest of Asia?

OBJECTIVE: Systemic Lupus Erythematosus (SLE) is known to be different among people with different racial, geographical and socio-economic back grounds. Asia has diverse ethnic groups broadly, Orientals in the East and Southeast Asia, Indians in South Asia and Arabs in the Middle East. These regions differ significantly from the Caucasians with reference to SLE. The purpose of this study was, therefore, to delineate the clinical pattern and disease course in Pakistani patients with SLE and compare it with Asian data. METHODS: Patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatism Association admitted at the Aga Khan University Hospital between 1986 and 2001 were studied by means of a retrospective review of their records. The results were compared with various studies in different regions of Asia. RESULTS: Demographically, it was seen that SLE is a disease predominantly of females in their third decade, which is generally consistent with Asian data. There was less cutaneous manifestations, arthritis, serositis, haematological and renal involvement compared to various regions in Asia. The neurological manifestations of SLE, however, place Pakistani patients in the middle of a spectrum between South Asians and other Asian races. CONCLUSION: This study has shown that the clinical characteristics of SLE patients in our country may be different to those of other Asian races. Although our population is similar to South Asians, but clinical manifestations of our SLE patients are considerably different, suggesting some unknown etiology. Further studies are required to confirm the above results and to find statistically sounder associations.

Ischemic monomelic neuropathy: a complication of vascular access procedure.

Ischemic monomelic neuropathy (IMN) is an infrequently recognized type of ischemic neuropathy produced by shunting of blood or due to acute noncompressive occlusion of the major proximal limb artery. Most reports about this complication appear in the neurology literature. IMN predominantly occurs in diabetic patients with evidence of peripheral neuropathy and atherosclerotic peripheral vascular disease. We report a case of ischemic monomelic neuropathy occurring in a patient with end stage diabetic nephropathy following PTFE (polytetrafluoroethylene) graft placement in proximal upper limb for chronic maintenance hemodialysis.

Renal involvement in systemic lupus erythematosus in Pakistan.

OBJECTIVE: To find the prevalence of lupus nephritis, delineate its clinical, immunological and therapeutic characteristics and compare them with the data worldwide. PATIENTS AND METHODS: Between 1985 and 2001, 198 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatism Association (ARA) admitted to the hospital were studied by means of a retrospective review of their records. RESULTS: Renal involvement was found in 89 (45%) patients. Biopsy showed lupus nephritis in 42 patients; there were 9 male and 33 females. Mean age at initial presentation was 27 years and mean duration of follow-up was 2 .3 years. The histological types (WHO Classification) were mainly class. 4 (n = 27), class 3 (n = 7) and class 5 (n = 6). Immunoflourescence showed a predominantly granular pattern of IgG, IgA and C3. Renal manifestations included renal failure (50%), microscopic hematuria (67%), active urine sediment (22%), and proteinuria (74%). Proteinuria was nephrotic range in 45% patients. Treatment was with combinations of prednisolone and cyclophosphamide (n = 13), prednisolone and azathioprine (n = 27). 19 patients received high dose methyl prednisolone (1 gm/day for 3 days). There was no difference in mortality rate between prednisolone and cyclophosphamide and prednisolone and azathioprine treatment groups. The overall mortality rate was 17% (n = 7). Mortality was higher in WHO class 4 and 5 as compared to class 2 and 3 (p < 0.001). CONCLUSION: The prevalence of lupus nephritis in our population is an intermediate between Caucasians and other Asians. Certain clinical characteristics in our patients with lupus nephritis are different as compared to various other studies. Because of limited resources for treatment in developing countries, we believe that patients with lupus nephritis should be treated with improved ancillary medical therapies and more effective immunosuppressive regimens.

Clinico-laboratory findings in male lupus patients from a tertiary care hospital, Pakistan.

This study was conducted to delineate the clinical pattern of a cohort of Pakistani male patients with systemic lupus erythematosus (SLE). Clinical and laboratory data were collected of 24 male patients who were diagnosed with SLE and admitted to a tertiary care hospital between 1986 and 2001. Imaging and invasive studies (including aspirations and biopsies) were also recorded. Fourteen patients (58%) had renal involvement, with WHO class 4 and 5 comprising 89% of the cases. Eight patients (33%) had neurological involvement. Out of these 8 patients, 3 presented with psychosis (12.5%) and 4 (17%) with seizures. Twenty three patients (96%) had hematological involvement, 6 (25%) had serosal and 10 (41%) had articular involvement. Cutaneous lesions were noted in 10 (41%) patients. A majority of the patients were noncompliant and were lost to follow-up; therefore, ultimate outcome could not be clearly delineated. A high index of suspicion for SLE in males may permit early diagnosis and dictate the need for more aggressive therapy.

Renovascular hypertension: factors affecting the outcome following surgical revascularisation.

This study was conducted at the Aga Khan University Hospital, Karachi, Pakistan to evaluate factors that affect outcome following revascularisation in patients with renovascular hypertension. We included all the patients diagnosed to have renovascular hypertension, confirmed by renal angiography, between July 1997 and September 2000. Of the total 15 patients, nine were males and six were females. Eleven patients received venous grafts, three received polytetraflouroethyline (PTFE) grafts while one patient underwent angioplasty and stenting. All were followed-up for a period of nine months (median) with the range from 2 to 84 months. A total of 33.3% of the study patients were completely cured, as they became normotensive without anti-hypertensive therapy after operation, while 27% showed marked improvement in blood pressure control post-operatively. Thus, extended cure or improvement of renovascular hypertension was achieved in 60% of patients. Normal pre-operative serum creatinine level, high pre-operative unstimulated peripheral renin levels and renal vein renin ratio of at least 1.75:1 were the most significant predictive factors for favorable outcome (p < 0.012). The pre-operative severity and duration of hypertension as well as degree of disparity in kidney sizes did not predict the post-operative improvement in renal function and blood pressure control.

Unusual Presentations of Wegener's granulomatosis: Pitfalls in Early Diagnosis.

Wegener's Granulomatosis (WG) is a disorder characterized by necrotizing granulomatous vasculitis that primarily affects the upper and lower respiratory tracts and the kidneys. Although the cause is unknown, there is strong evidence that this is an autoimmune disease and immunosuppressive therapy with cyclophosphamide and corticosteroids efficiently relieves the symptoms and, prevents relapses. We report on four cases of WG that presented with unusual features. The first patient presented with vague joint pain, blackish discoloration of the skin and leg ulcers. The second patient presented as fever of unknown origin and gingival hyperplasia. The third patient presented with hearing loss while the fourth presented with arthritis, conjunctivitis and painful swelling of the ear. It is emphasized that WG is a complex disease and can involve multiple organ systems. Early recognition and institution of proper treatment are crucial for optimal outcome.

Ischemic monomelic neuropathy: a complication of vascular access procedure.

Ischemic monomelic neuropathy (IMN) is an infrequently recognized type of ischemic neuropathy produced by shunting blood or acute occlusion of a major proximal artery in the extremities. IMN predominantly occurs in diabetic patients with evidence of peripheral atherosclerotic vascular disease and neuropathy. We report a case of ischemic monomelic neuropathy occurring in a diabetic patient with end-stage renal disease following the placement of polytetrafluoroethylene (PTFE) graft as a vascular access in the proximal upper arm for chronic hemodialysis.