Cochlear implantation in a profoundly deaf child with cystic leukoencephalopathy without megalencephaly.

BACKGROUND: Cochlear implantation candidacy criteria have continued to evolve over the years, and cochlear implantation is possible with many inner-ear and brain anomalies with good hearing and linguistic outcomes. Cystic leukoencephalopathy without megalencephaly is a rare disease in children, with only 30 cases reported in the literature, but it is associated with hearing loss in only three cases. Radiological investigations can help in diagnosing this rare entity before proceeding with cochlear implantation. CASE REPORT: A four-year-old female child born out of consanguinity with normal psychomotor development, bilateral sensorineural hearing loss and an incidental magnetic resonance imaging finding of cystic leukoencephalopathy without megalencephaly underwent successful cochlear implantation. Her post-operative period was uneventful with successful mapping of the cochlear implant. CONCLUSION: This is the first reported case of cystic leukoencephalopathy without megalencephaly and with sensorineural hearing loss in which cochlear implantation was performed successfully. White matter and temporal lobe abnormalities should not deter paediatric cochlear implantation.

Quantification of biomaterial dispersion during otologic procedures and role of barrier drapes in Covid 2019 era - a laboratory model.

BACKGROUND: Aerosol generation during temporal bone surgery caries the risk of viral transmission. Steps to mitigate this problem are of particular importance during the coronavirus disease 2019 pandemic. OBJECTIVE: To quantify the effect of barrier draping on particulate material dispersion during temporal bone surgery. METHODS: The study involved a cadaveric model in a simulated operating theatre environment. Particle density and particle count for particles sized 1-10 µ were measured in a simulated operating theatre environment while drilling on a cadaveric temporal bone. The effect of barrier draping to decrease dispersion was recorded and analysed. RESULTS: Barrier draping decreased counts of particles smaller than 5 µ by a factor of 80 in the operating theatre environment. Both particle density and particle count showed a statistically significant reduction with barrier draping (p = 0.027). CONCLUSION: Simple barrier drapes were effective in decreasing particle density and particle count in the operating theatre model and can prevent infection in operating theatre personnel.

Randomized Trial of Primary PCI with or without RoutineManual Thrombectomy

During primary percutaneous coronary intervention (PCI), manual thrombectomymay reduce distal embolization and thus improve microvascular perfusion. Smalltrials have suggested that thrombectomy improves surrogate and clinical outcomes,but a larger trial has reported conflicting results.MethodsWe randomly assigned 10,732 patients with ST-segment elevation myocardial infarction(STEMI) undergoing primary PCI to a strategy of routine upfront manualthrombectomy versus PCI alone. The primary outcome was a composite of deathfrom cardiovascular causes, recurrent myocardial infarction, cardiogenic shock, orNew York Heart Association (NYHA) class IV heart failure within 180 days. The keysafety outcome was stroke within 30 days.ResultsThe primary outcome occurred in 347 of 5033 patients (6.9%) in the thrombectomygroup versus 351 of 5030 patients (7.0%) in the PCI-alone group (hazard ratio in thethrombectomy group, 0.99; 95% confidence interval [CI], 0.85 to 1.15; P = 0.86). Therates of cardiovascular death (3.1% with thrombectomy vs. 3.5% with PCI alone;hazard ratio, 0.90; 95% CI, 0.73 to 1.12; P = 0.34) and the primary outcome plusstent thrombosis or target-vessel revascularization (9.9% vs. 9.8%; hazard ratio,1.00; 95% CI, 0.89 to 1.14; P = 0.95) were also similar. Stroke within 30 days occurredin 33 patients (0.7%) in the thrombectomy group versus 16 patients (0.3%)in the PCI-alone group (hazard ratio, 2.06; 95% CI, 1.13 to 3.75; P = 0.02).ConclusionsIn patients with STEMI who were undergoing primary PCI, routine manual thrombectomy,as compared with PCI alone, did not reduce the risk of cardiovasculardeath, recurrent myocardial infarction, cardiogenic shock, or NYHA class IV heartfailure within 180 days but was associated with an increased rate of stroke within30 days. (Funded by Medtronic and the Canadian Institutes of Health Research;TOTAL ClinicalTrials.gov number, NCT01149044.

(Copper-curcumin) ß-cyclodextrin vaginal gel: delivering a novel metal-herbal approach for the development of topical contraception prophylaxis.

Delivering a safe and effective topical vaginal contraceptive is the need of present era. We explored the potential of a metal (copper) and herbal moiety (curcumin) for this topical contraceptive prophylaxis. Complex of copper and curcumin (Cu-Cur) was synthesized and the concerns regarding its aqueous solubility was resolved by including it into the hydrophobic cavity of ß-cyclodextrin (ß-CD) as (Cu-Cur)CD inclusion complex. Dose assessment was made on the basis of in-vitro spermicidal assays and cell cytotoxicity studies. Finally the (Cu-Cur)CD loaded vaginal gel was prepared, characterized and evaluated for in-vitro spermicidal activity and preclinical toxicity studies. Spectral and morphological characterizations confirmed the synthesis of (Cu-Cur) and (Cu-Cur)CD inclusion complex. Spermicidal assays and Hela cell cytotoxic data revealed an optimized 1.5% (Cu-Cur)CD for further studies. 1.5% w/w (Cu-Cur)CD loaded carbopol 974p gel provided 100% motility even at 2-fold dilution and preclinical toxicity studies in Rats and Rabbits revealed its highly safe profile. The hypothesis of considering metal-herbal complex and its cyclodextrin complex has worked and the well planned strategy of including it in (ß-CD) cavity provided a preeminent platform for vaginal delivery. In-vitro assays and preclinical toxicity analysis confirmed its potential to be used as highly safe and effective prophylaxis.

Single piece artificial urinary sphincter for secondary incontinence following successful repair of post traumatic urethral injury.

Post traumatic urethral injury is uncommon in children. The management of this condition is dependent on the severity of injury. Initial suprapubic cystostomy with delayed repair is the conventional treatment. Successful reconstruction of urethral injury may be followed by urethral stricture, incontinence, impotence, and retrograde ejaculation. Successful repair of post traumatic urethral injury followed by secondary incontinence in children has not been well addressed in literature. We report the management of one such child, with satisfactory outcome with implantation of a new model of single piece artificial urinary sphincter in the bulbar urethra by perineal approach.

Minimal access surgery in children:a 5 year study.

Over a period of 5 years, we analyzed our data on outcome, feasibility, and safety of Minimal Access Surgery (MAS) in 211 children. The outcome was compared objectively with age matched controls with similar diagnosis undergoing open surgery over the same period. There was no significant difference between mortality, morbidity, re-exploration rates and analgesic requirement between MAS and open surgery. There was a significant difference in the length of stay in hospital, in favour of laparoscopic cholecystectomy, appendectomy, nephrectomy, splenectomy, surgery for intra-abdominal testis compared to open surgery but not for children undergoing surgery for appendicular perforation and intestinal pathology. All parents preferred the cosmetic outcome of minimal access surgery.

Permanent pacemaker insertion after CoreValve transcatheter aortic valve implantation: incidence and contributing factors (the UK CoreValve Collaborative).

BACKGROUND: Permanent pacemaker (PPM) requirement is a recognized complication of transcatheter aortic valve implantation. We assessed the UK incidence of permanent pacing within 30 days of CoreValve implantation and formulated an anatomic and electrophysiological model. METHODS AND RESULTS: Data from 270 patients at 10 centers in the United Kingdom were examined. Twenty-five patients (8%) had preexisting PPMs; 2 patients had incomplete data. The remaining 243 were 81.3±6.7 years of age; 50.6% were male. QRS duration increased from 105±23 to 135±29 milliseconds (P<0.01). Left bundle-branch block incidence was 13% at baseline and 61% after the procedure (P<0.001). Eighty-one patients (33.3%) required a PPM within 30 days. Rates of pacing according to preexisting ECG abnormalities were as follows: right bundle-branch block, 65.2%; left bundle-branch block, 43.75%; normal QRS, 27.6%. Among patients who required PPM implantation, the median time to insertion was 4.0 days (interquartile range, 2.0 to 7.75 days). Multivariable analysis revealed that periprocedural atrioventricular block (odds ratio, 6.29; 95% confidence interval, 3.55 to 11.15), balloon predilatation (odds ratio, 2.68; 95% confidence interval, 2.00 to 3.47), use of the larger (29 mm) CoreValve prosthesis (odds ratio, 2.50; 95% confidence interval, 1.22 to 5.11), interventricular septum diameter (odds ratio, 1.18; 95% confidence interval, 1.10 to 3.06), and prolonged QRS duration (odds ratio, 3.45; 95% confidence interval, 1.61 to 7.40) were independently associated with the need for PPM. CONCLUSION: One third of patients undergoing a CoreValve transcatheter aortic valve implantation procedure require a PPM within 30 days. Periprocedural atrioventricular block, balloon predilatation, use of the larger CoreValve prosthesis, increased interventricular septum diameter and prolonged QRS duration were associated with the need for PPM.

Radiation therapy with implanted cardiac pacemaker devices: a clinical and dosimetric analysis of patients and proposed precautions.

AIMS: To report the radiation planning dosimetric aspects and clinical outcomes of patients with implanted cardiac pacemakers. MATERIALS AND METHODS: Between 2005 and 2009, eight patients with in situ cardiac pacemakers of varied primary site were treated at our hospital. All patients underwent computed tomography-based treatment planning. The target volumes, organs at risk and pacemaker device were all contoured. A treatment plan optimally covering the target area and maximally sparing the pacemaker was generated. All patients were evaluated at baseline, during radiotherapy and after radiotherapy conclusion by a cardiologist as well as pacemaker company personnel. RESULTS: The median age at presentation was 67 (range 53-77) years. There were three men with head and neck primaries, two men with lung primaries and three women with breast primaries. The prescribed dose ranged from 45 to 70 Gy in 25-35 fractions with a daily dose of 1.8-2.0 Gy. Four patients had the pacemaker implanted on the same side as the radiotherapy target. The dose ranges for the minimum, mean and maximum doses to the pacemaker were 0.06-2.0, 0.07-20.6 and 0.14-60.0 Gy, respectively. Radiation therapy was safely delivered in all patients without any untoward effects. At 5 months of median follow-up, all patients were well with no malfunction of the pacemaker. CONCLUSION: A series of eight patients with in situ pacemakers treated with radiotherapy is reported. Radiotherapy can be safely delivered in patients with implanted cardiac pacemakers. However, it mandates a cautious approach in planning and treatment delivery to ensure the least possible dose to the pacemaker. Close liaison with the cardiologist and a pacemaker clinic before, during and after the course of treatment is essential to ensure patient safety.

Nutritional assessment of patients under hemodialysis in nepal medical college teaching hospital.

Malnutrition is very common in hemodialysis patients and is predisposed by many factors. Malnutrition is associated with increased morbidity and mortality. Total of 26 patients (16 males and 10 females) who were under hemodialysis in our center were included in the study. With the help of Malnutrition Score (MS) developed by Kalanter-Zadeh, nutritional status of the patients was assessed. Patients also underwent different anthropometric measurements such as Body Mass Index (BMI), Triceps skin fold thickness (TSF), Mid Arm Circumlference (MAC) and Mid Arm Muscle Circumference (MAMC) and laboratory investigations. Mean age of the study population was 42.58 +/- 16.32 years (range 19 to 74 years). Females were older than males. MS of the study population was 15.82 +/- 3.76 (range 9-24). Female patients were having higher MS than males (16.5 +/- 4.11 vs. 15.06 +/- 3.55). Based on MS, 22 patients (84.6%) had mild to moderate malnutrition, 2 (7.7%) patients were having severe malnutrition and remaining 2 (7.7%) had normal nutrition score. Females were having lower BMI, MAC and MAMC but higher value of TSF. Significant negative correlation was present between MS and weight, BMI, MAC and MAMC. Calculated Urea Reduction Ratio (URR) of study population was 57.27 +/- 10.89. URR was higher in females than in males (61.77 +/- 12.74 vs. 54.45 +/- 8.85). Only 23.0% of the study population had URR of >65.0%. Protein Catabolic Rate (nPCR) in our study was 0.77 +/- 0.28 g/kg/day. Malnutrition is very common in our center which is >90% when MS was considered. In our study it negatively correlated with weight, BMI, MAC and MAMC. Dialysis inadequacy was present in around 75.0% of our study population.

Factors affecting renal scarring in posterior urethral valves.

AIM: To retrospectively review the occurrence of renal scarring in patients with posterior urethral valves (PUV), and correlate it with various causative factors. METHODS: The records of 52 patients treated for PUV by the authors were reviewed. Patients with vesico-ureteric reflex (VUR) dysplasia syndrome were excluded from the study. The patients were divided into group I (no renal scarring, n=18) and group II (renal scars present, n=34) based on dimercapto-succinic acid scans. The mode of treatment, presence/absence of breakthrough urinary tract infections (UTI), presence/absence of history of nocturnal/diurnal incontinence, presence/absence of VUR, stable postoperative serum creatinine, rate of drainage of ureters on diethylene triamine penta acetic acid (DTPA) scans and management of bladder dysfunction, if any, were noted. The presence or absence of renal scarring was statistically correlated with occurrence of any of the above factors. RESULTS: Primary valve fulguration was performed in 41 patients and 11 patients had an initial vesicostomy. The median follow up was 3.5 years (range 1.5-15 years). Renal scarring was present in either kidney in 34 patients (bilateral 14, unilateral 20). The mode of initial treatment did not affect the incidence or rate of scarring. The preoperative and postoperative serum creatinine at the end of 1 year did not differ between the two groups. Presence/severity of VUR did not affect the pattern of renal scarring. A highly significant correlation between the occurrence of renal scarring and presence of diurnal incontinence (P< or =0.007, odds ratio=4.5) and breakthrough UTI (P< or =0.002, odds ratio=7.0) was observed. There was also correlation with slow drainage in the ureters on a DTPA scan (P< or =0.0005). Detrusor instability and low compliance on urodynamic assessment did not affect occurrence in the limited number of patients studied. The rate of somatic growth in both groups was retarded as compared to normal healthy counterparts. CONCLUSION: Breakthrough UTI, diurnal incontinence and poor drainage of ureters on DTPA are associated with a higher incidence of renal scarring. Mode of initial treatment, presence or absence of VUR, and bladder abnormalities do not affect renal scarring in the short term.

The influence of small fibre muscle mechanoreceptors on the cardiac vagus in humans.

We have previously shown that activation of muscle receptors by passive stretch (PS) increases heart rate (HR) with little change in blood pressure (BP). We proposed that PS selectively inhibits cardiac vagal activity. We attempted to test this by performing PS during experimental alterations in vagal tone. Large decreases in vagal tone were induced using either glycopyrrolate or mild rhythmic exercise. Milder alterations in vagal tone were achieved by altering carotid baroreceptor input: neck pressure (NP) or neck suction (NS). PS of the triceps surae was tested in 14 healthy human volunteers. BP, ECG and respiration were recorded. PS alone caused a significant decrease (P < 0.05) in R-R interval (962 +/- 76 ms at baseline compared to 846 +/- 151 ms with PS), and showed a reduction in HR variability, which was not significant. The decrease in R-R interval with PS was significantly less (P < 0.05, n = 3) following administration of glycopyrrolate (-8.1 +/- 4.5 ms) compared to PS alone (-54 +/- 11 ms), and also with PS during handgrip (+10 +/- 10 ms) compared with PS alone (-74 +/- 15 ms) (P < 0.05, n = 5). Milder reductions in vagal activity (NP) resulted in a small but insignificant further decrease in R-R interval in response to PS (-107 +/- 17 ms compared to PS alone -96 +/- 13 ms, n = 5). Mild increases in vagal activity (NS) during PS resulted in smaller decreases in R-R interval (-39 +/- 5.5 ms) compared to PS alone (-86 +/- 17 ms) (P < 0.05, n = 8). BP was not significantly changed by stretch in any tests. The results indicate that amongst muscle receptors there is a specific group activated by stretch that selectively inhibit cardiac vagal tone to produce tachycardia.

Extrahepatic portal hypertension: quality of life and somatic growth after surgery.

AIM: This study was carried out in order to determine whether children with extrahepatic portal hypertension show any improvement in growth parameters and quality of life after elective surgery. PATIENTS AND METHODS: All patients with extrahepatic portal hypertension who underwent surgery between April 1999 and March 2002 were studied prospectively. Height and weight before and after surgery were converted into Z scores for comparison. The quality of life was assessed by changes in scholastic ability, physical activity, social interaction, and economic effects on the family. RESULTS: At the end of a minimum follow-up period of 1 year, of the 30 children, 50 % and 76 % showed improvement in weight and height Z scores, respectively, compared to their scores while on medical management. Z scores below - 2 SD for weight and height were seen in 57 % and 37 % of patients prior to surgery, and only in 40 % and 20 %, respectively at the end of follow-up, which ranged from 1 - 4 years. The improvement in height and weight was more in those who had undergone splenectomy with either devascularization or central splenorenal shunt compared to those who had undergone side-to-side lienorenal shunt without splenectomy. The children, who had dropped out of school while on medical management, either rejoined school or attended vocational courses after surgery. There was an improvement in school performance and physical activity in 85 % of the children. Improvement in personality was seen in 73 % of the affected patients. The cost incurred for surgery was one-third of the amount spent while on medical management. CONCLUSIONS: Growth parameters improved significantly in children after surgical intervention for portal hypertension. Overall improvement in scholastic abilities, physical activity, and social interaction was noted in a majority of the patients. As a one-time procedure in a developing country, surgery is also more cost effective.

Extrahepatic portal hypertension in children: observations on three surgical procedures.

This paper presents a comparative prospective study of three modalities of surgical treatment for extrahepatic portal hypertension in children: central splenorenal shunt after splenectomy (CSS), side-to-side lienorenal shunt (SSLR) without splenectomy, and splenectomy and gastroesophageal devascularization (SGD). In an 18-month period, 27 procedures were performed: 10 CSS, 10 SSLR, and seven SGD. The outcomes were evaluated by fall in portal pressures, hematological parameters, shunt patency, splenic regression, and disappearance of esophageal varices. All three procedures were comparable in the fall of portal pressure after surgery. The average blood loss and operating time were statistically significant in favor of SSLR compared with CSS. At 3-month follow-up, shunt patency was confirmed by duplex Doppler study in all the patients in the SSLR group and in nine out of 10 patients in the CSS group. In the CSS and SGD groups, hypersplenism resolved in all the patients. In the SSLR group, blood counts improved in only five out of eight affected children. No patient re-bled during a follow-up of 3-5 years. There were no cases of hepatic encephalopathy or overwhelming postsplenectomy sepsis. In conclusion, CSS is useful when there is a large spleen, severe hypersplenism, and a shuntable splenic vein. SSLR is suitable when there is only mild splenomegaly, mild hypersplenism, and a shuntable splenic vein. Splenectomy and devascularization is the choice when there is no shuntable splenic vein.

Cutaneous tuberculosis in Indian children: the importance of screening for involvement of internal organs.

AIMS AND OBJECTIVES: Resurgence of skin tuberculosis especially with drug-resistant strains has been well documented in recent years, but this problem has not received much attention in the paediatric age group. Hence, we carried out the present study to analyse the clinical and therapeutic aspects of cutaneous tuberculosis in children. MATERIALS AND METHODS: A detailed clinical examination, investigations, such as haemogram, serology for HIV, Mantoux test, chest X-ray, cytology, culture and histopathology were carried out in all children. They were treated with antitubercular therapy (WHO regimen), and the clinical response was followed up. RESULTS: Of 142 patients with cutaneous tuberculosis, 68 were children (40 females, 28 males). These children were aged from 9 months to 14 years. The duration of the disease varied from 1 month to 6 years. Family history of tuberculosis was present in 28 (41.2%) of the patients. Scrofuloderma was the most common presentation encountered in 30 (44.1%) patients with preferential involvement of the cervical (56.2%) and inguinal (20%) regions. Fifteen (22.1%) patients had lupus vulgaris, of which the keratotic type was the most common (46.7%), 16 had lichen scrofulosorum, three had tuberculosis verrucosa cutis, and four had more than one type of tuberculosis. Involvement of the lung in 14 (20.6%), bone in seven (10.2%), and both in four (5.9%) was found. Histopathology corroborated the clinical diagnosis in 54 (80.6%), culture was positive in six (8.8%). Fifty (73.5%) patients completed the treatment with an excellent response, no multidrug resistant cases were seen. CONCLUSIONS: Cutaneous tuberculosis in children continues to be an important cause of morbidity, there is a high likelihood of internal involvement, especially in patients with scrofuloderma. A search is required for more sensitive, economic diagnostic tools. Response to treatment at 4 weeks often helps in substantiating the diagnosis of tuberculosis in doubtful cases.

Risk factors in the primary management of anorectal malformations in Northern India.

The purpose of this study was to determine the major factors influencing the survival of babies with anorectal malformation (ARM) during the primary management in the neonatal period in a tertiary referral center. The outcome of 125 neonates with ARM, treated in a single hospital during a 2-year period, was analyzed retrospectively with particular reference to birth weight, time of arrival, and the type of ARM, including pouch colon and major associated anomalies, to see whether any of them had a significant effect on initial survival. High ARM (HARM) was present in 75, low ARM (LARM) in 36, and pouch colon in 14 babies. There were a total of 28 deaths (22%). In babies with isolated HARM weighing >2.5 kg and brought to the hospital within the first 48 h of life, the survival was 100%, which dropped to 80% when brought later than 48 h. Of the babies with birth weight <2.5 kg and isolated HARM but who were brought to the hospital within the first 48 h of life, 80% survived, whereas of those reaching the hospital after 48 h, only 55% survived. Two babies with pouch colon and two of the four babies with LARM and associated anomalies died. In the HARM group, of the 31 babies with major associated anomalies, only 37% with birth weight <2.5 kg and 58% with birth weight >2.5 kg survived. Low birth weight, major associated anomalies, and delay in referral were the three important factors that influenced the outcomes of babies with ARM during the neonatal period.

Preoperative angioembolisation for life-threatening haemorrhage from Wilms' tumour: a case report.

Wilms' tumour is one of the most common abdominal tumours of childhood. Severe perirenal bleeding resulting in consumptive coagulopathy and colonic obstruction are rare complications of Wilms' tumour. We present a case report of one patient with these two complications, their successful management with preoperative angioembolisation and emergency nephrectomy, and a review of the relevant literature.

An audit of neonatal colostomy for high anorectal malformation: the developing world perspective.

A high divided sigmoid colostomy has been recommended for staged management of high anorectal malformation. We audited our cases of neonatal colostomy for high anorectal malformation to assess its effectiveness. A retrospective study was carried out of all surgical newborns admitted with high imperforate anus as the single diagnosis at our centre between December 1998 and December 2000. Morbidity and mortality were analysed after retrospective stratification into two groups (group A: birth weight >2.5 kg; group B: birth weight <2.5 kg). The chi square test was used to test the statistical significance in terms of outcome in the two groups. Overall mortality was 16%. Group A consisted of 34 babies: 30 with divided sigmoid colostomy and four with transverse loop colostomy. One baby with a divided sigmoid colostomy died from wound complications and septicaemia (mortality 2.9%). All four babies with transverse loop colostomy done under local anaesthesia survived, despite being sick on arrival. Group B consisted of 16 babies: 15 with sigmoid colostomy and one with transverse loop colostomy, with seven deaths (44%). None of the five babies with transverse loop colostomy done under local anaesthesia died, despite being sick on arrival, whereas all eight babies who died had undergone sigmoid colostomy under general anaesthesia. The difference in the outcomes of babies in groups A and B is highly significant ( p <.01). Sick, small (<2.5 kg) and septic babies arriving late to the unit do not appear to tolerate general anaesthesia and divided sigmoid colostomy well, despite that procedure's long-term advantages. Divided sigmoid colostomy has produced excellent results in babies >2.5 kg, but in the context of the developing world and limited critical care availability, transverse loop colostomy under local anaesthesia may save lives.