Causes and Outcome of Central Venous Catheter Repair in Children with Intestinal Failure on Home Parenteral Nutrition.

OBJECTIVES: Children requiring a central venous catheter (CVC) for long-term parenteral nutrition (PN) are at risk of CVC breakage. Modern intestinal failure (IF) management aims to preserve vascular access sites. CVC repair rather than removal is hence attempted for broken catheters. The aim of this study was to describe causes and outcomes of CVC repairs in home PN dependent children. METHODS: All patients (ages 0-17 years) with CVC dependency enrolled in the IF rehabilitation program of a pediatric referral center were identified, and those who underwent a CVC repair between January 2019 and November 2020 included. Data on associated cause for breakage and incidence of central line-associated bloodstream infections (CLABSIs) post repair were documented retrospectively. Descriptive statistics including medians, percentages, and frequencies were used. RESULTS: Forty children, 15 males (37%) and 25 females (63%), were identified. Fifteen of 40 (37.5%) patients underwent a total of 29 CVC repairs (0.1 repairs per 1000 catheter days); 8 of 15 (53%; 33% females) were <5 years of age. The most common reason for repair was CVC fracture by biting (41%) followed by catheter occlusion with intraluminal PN deposition (13.2%). Repair was successful in 100% cases. Only 1 patient had a confirmed CLABSI post repair (1/29 repairs; 3%) who presented 3 days late after the initial catheter breakage. Catheter salvage was successful with antibiotics. CONCLUSION: CVC repair in our cohort was very successful and infection rate after repair minimal. CVC repair rather than removal is recommended to preserve central venous access.

Chronic kidney disease causes and outcomes in children: Perspective from a LMIC setting.

BACKGROUND AND OBJECTIVE: Chronic kidney disease (CKD) constitutes a major public health challenge, with a global prevalence of 15-74.7 cases /million children. Preventing CKD in children, slowing its progression and management of complications are essential, especially in challenged health systems in low middle income countries (LMIC). We conducted a retrospective review to assess the underlying cause and stage of CKD at presentation and clinical outcomes in children and adolescents at the Indus Hospital and Health Network (IHHN) in Karachi, Pakistan. METHODS: Children 0-16 years with CKD stage 1 and/or higher at presentation were included. Data including demographics, clinical status and lab results at presentation and during follow-up, surgical intervention if any, kidney function at last visit and outcome at last follow-up was recorded. RESULTS: A total of 229 children diagnosed with CKD are included in our study. The median age at diagnosis was 10 years with male: female ratio of 1.8:1. Only 5% children presented in stage 1 CKD. The rate of adverse outcomes is 4.5 times higher in children with CKD stage 3-5 compared to early CKD. Congenital anomaly of kidney and urinary tract (CAKUT) was the underlying cause in 49% children. Children with glomerular disease had comparatively worse outcome. Proteinuria, hypertension, anemia and bone disease were associated with high morbidity and mortality. CONCLUSION: The true epidemiology of childhood CKD is unknown in Pakistan. Our cohort showed better CKD outcomes in children diagnosed early with appropriate surgical and medical follow-up. Prompt diagnosis, treatment and prevention of progression can be life-saving in our setting. CKD registry data can inform policy changes that can prevent poor outcomes.

Long-term Follow-up of Exstrophy-epispadias Complex from a Lower-middle Income Country: A Case Report and Review of the Literature.

Bladder exstrophy-epispadias complex (EEC) is a rare congenital defect where the abdominal muscles and bones fail to close in the mid-pelvis. It is crucial to understand the health-related quality of life (QOL) of exstrophy patients who have undergone multiple correctional surgeries. We herein discuss a case of bladder EEC that was repaired through a series of procedures at a resource-limited hospital in Karachi, Pakistan. A 21-year-old male, who was born with EEC, underwent bladder augmentation, Mitrofanoff procedure, bladder neck reconstruction, ureter implantation and a right nephrectomy in a single one-stage procedure during late childhood for urinary incontinence. However, this required a further revision because the urinary incontinence persisted, with difficulty in catheterizing the Mitrofanoff channel. On follow-up after 10 years, our patient currently describes normal QOL with near-normal sexual function. Validated questionnaires for QOL, erectile dysfunction, incontinence and prostatic function were used to assess the patient's post-operative status in these domains. Through our report, we conclude that such patients can have a normal QOL by means of a holistic multidisciplinary management, which includes timely surgical corrections along with an additional focus on the psycho-social and sexual aspects of this condition.

Glycogenic hepatopathy, an underdiagnosed cause of relapsing hepatitis in uncontrolled type 1 diabetes mellitus.

Glycogenic hepatopathy is a rare condition that causes significant hepatomegaly and elevated liver enzyme levels in uncontrolled type 1 diabetic patients. It develops due to excessive accumulation of glycogen in the hepatocytes. It is typically reversible with good glycemic control and rarely progresses to mild fibrosis, but not cirrhosis.

Fungal Esophagitis in a Child with Insulin Dependent Diabetes Mellitus.

Esophagitis in children is not uncommon, mostly due to gastro-esophageal reflux. Other conditions like eosinophilic and infective esophagitis need to be elucidated in differential diagnoses. Fungal orCandida esophagitisusually occurs in high risk children who are immune-compromised, malnourished, on steroid therapy or have uncontrolled diabetes mellitus. An eleven-year girl presented with uncontrolled type I diabetes mellitus and recurrent epigastric pain with vomiting. Her oral intake was satisfactory. There was no dysphagia and odynophagia. Physical examination was normal with good oral hygiene. Failure in responding to conventional medications led to endoscopic evaluation, which revealed white patches and esophageal inflammation and diagnosed as fungal esophagitis on histopathology. Although infective esophagitis is encountered sporadically in pediatric age group, but it should always be considered in high risk individuals and when conventional medication fails to resolve the symptoms.

Blurred vision as the sole initial presentation of disseminated lung malignancy.

Symptomatic choroidal metastasis (CM) is a rare presenting feature of disseminated lung malignancy. Detection of the primary malignancy usually precedes ocular findings. We report a rare case of blurred vision secondary to bilateral CM as the sole initial manifestation of disseminated lung malignancy in a female patient. Pulmonary symptoms appeared 2 months later. She received radiotherapy and palliative chemotherapy and survived for 7 months after the initial presentation.

Tubed vs tubeless PCNL in children.

OBJECTIVE: To compare post-operative outcomes between tubeless and conventional large-bore nephrostomy tube drainage following percutaneous nephrolithotomy in children. METHODS: The study comprised 54 patients under 14 years of age who were undergoing percutaneous nephrolithotomy at 60 renal units and met the inclusion criteria. They were randomised to placement of a 16F nephrostomy tube (Group A, 30 renal units) or tubeless drainage (Group B, 30 renal units) at the end of the procedure. Patient age, number and position of stones, operating time, change in haemoglobin, post-operative analgesia requirement, length of hospital stay and post-operative complications were compared between the two groups, using SPSS version 17 and t test. RESULTS: Group A had 28 patients, while Group B had 26. The mean age in Group A was 7.2 +/- 3.2 years, and in Group B it was 6.3 +/- 3.6 years (age range 3-13 years and 1-13 years respectively). The mean size of stone was 28.6 +/- 16.7mm and 20.4 +/- 9.3mm; mean change in Hb was 0.78 +/- 0.69mg/dl and 0.63 +/- 0.54mg/dl; and the mean operating time was 54 +/- 20.7 minutes versus 66.9 +/- 22.9 minutes in the two groups respectively. There was significantly less requirement for post-operative pethidine in Group B versus Group A (p=0.01). The postoperative clearance and complications were comparable between the two groups, while the duration of hospital stay was significantly shorter in Group B compared to Group A (p=0.007). CONCLUSION: Tubeless percutaneous nephrolithotomy in children is safe and effective. Post-operative analgesia requirement is less and hospital stay is shortened compared to the conventional nephrostomy placement after percutaneous nephrolithotomy.

Bilateral ectopic ureters with bladder agenesis.

Agenesis of Urinary bladder is an extremely rare congenital genitourinary anomaly and is associated with other severe malformations that are incompatible with life. About 60 cases and 22 live births only have been reported in English literature. We report an eight year old girl with triad of complete agenesis of bladder and urethra, solitary functioning left kidney and an ectopic ureter opening into the vagina. The management of the child along with embryo-pathogenesis and literature review is discussed.

Effect of macular photocoagulation on visual acuity of Omani patients with clinically significant macular edema.

BACKGROUND: The aim of this study was to determine the effect of macular laser treatment on the visual acuity (VA) of Omani diabetic patients with clinically significant macular edema (CSME). Visual outcome was also correlated with duration and control of diabetes and presence or absence of hypertension and hyperlipidemia. MATERIALS AND METHODS: This is a retrospective noncomparative cohort study involving 101 eyes of 72 Omani diabetic patients. Change in VA was determined using Snellen's VA chart. The mean duration of follow-up was approximately 21 months (range, 16-24 months). RESULTS: 29.7% of the patients maintained their vision, 35.6% showed improvement, whereas 34.7% showed a decrease in their vision. Positive visual outcome showed a statistically significant direct relationship with tight control of diabetes and absence of hypertension and an inverse relationship with the duration of diabetes. Presence of hyperlipedemia did not show a statistically significant relationship with positive visual outcome. However, it showed a trend to better visual outcome in the absence of hyperlipedemia. Peak incidence of macular edema was seen at the age of 52.3 years. CONCLUSION: Macular photocoagulation was found to be an effective method of treatment for CSME among Omani diabetic patients, which has resulted in a positive visual outcome in 65.3% of the patients (stable and improved vision). Effective control of diabetes, duration of diabetes, and hypertension are the factors which influence the postlaser visual outcome.

Is ascending urethrogram mandatory for all urethral strictures?

OBJECTIVE: To determine the role of ascending urethrogram in decision making for patients with suspected urethral strictures. METHODS: Medical Records were reviewed of male patients presenting with lower urinary tract symptoms who were subjected to ascending urethrogram and selected cases for cystourethroscopy from January 2001 to December 2002. Cystourethroscopy was performed on those patients who complied with treatment for urethral stricture or who had persistent low flow of urine despite ascending urethrogram reporting no urethral stricture. Data was analyzed on SPSS 10.0. Sensitivity, specificity, positive and negative predictive values were calculated for ascending urethrogram as a measure to evaluate urethral stricture. RESULTS: Ascending urethrogram was done on 92 patients. Of whom 55 were reported to have urethral stricture. The mean age of patients was 42.8 +/- 13.2 years. Of the 92 patients who had ascending urethrogram, 62 were subjected to cystourethroscopy proceed optical internal urethrotomy (OIU) in cases of stricture. It was that out of 45 reported urethral strictures on ascending urethrogram, 5 did not have stricture on cystourethroscopy (P = 0.001). Likewise in the same group of 62 patients, 17 patients were reported to have no stricture on ascending urethrogram, whereas subsequent cystourethroscopy revealed stricture in 4 patients. The calculated sensitivity of ascending urethrogram was 91% and specificity 72%. The calculated positive and negative predictive values were 89% and 76% respectively. CONCLUSION: The study concluded that Ascending Urethrogram does not completely rule out urethral stricture (Negative Predictive Value 76%). It was also observed that urethral stricture may be non-existent even though suggested in Ascending Urethrogram (Positive Predictive Value 89%). With a sensitivity of 91% and a low specificity of 72% of the ascending urethrogram for diagnosing urethral stricture, it would be advisable to subject the patient to cystourethroscopy proceed Optical Internal Urethrotomy in cases of urethral stricture. This will save cost, avoid infection reduce risk of radiation and contrast related hypersensitivity reaction.

Extraosseous bone formation in the renal pelvis.

PURPOSE: We evaluated the frequency and features of extraosseous bone formation in the kidneys of patients undergoing percutaneous nephrolithotomy. MATERIALS AND METHODS: Percutaneous nephrolithotomy was performed in 621 patients at our institution between 1997 and 2006. In 21 cases metaplastic bone arising from the urothelium was observed. Clinical, radiographic and histopathological features of this group were studied in detail. RESULTS: Patient age ranged from 7 to 40 years (median 11). Extraosseous bone formation was identified in 7 right (33%) and 14 left (67%) kidneys. In all cases extraosseous bone was identified at the angle of the pelvis and ureter, just proximal to the ureteropelvic junction. The typical radiographic appearance of a radiopaque eccentric halo surrounding an area of lesser radiological density connected with the urothelium was seen in 10 of 13 radiographs (77%). Histopathological evaluation showed well formed trabecular bone with surface osteoblastic activity, areas of intratrabecular adipose bone marrow and hematopoietic cells in 5 cases (24%); woven bone intimately related to trabecular bone with scattered hematopoietic cells in 14 (67%); and entirely woven bone with associated mineral deposits and prominent fibroblastic proliferation in 2 (10%). CONCLUSIONS: Although rarely reported in the literature, metaplastic bone formation in the renal pelvis was seen relatively frequently in our patient population. The pathogenesis of this phenomenon is not clearly understood. Recognition of extraosseous bone is important, since it has implications for management and prognosis. In-depth studies of this phenomenon are required to arrive at any conclusions regarding its etiology.

Laparoscopic nephrectomy: technique and outcome.

OBJECTIVE: To review case series of retroperitoneal laparoscopic nephrectomies. METHODS: Between May 2003 and May 2006, 60 laparoscopic nephrectomies were performed by a single surgeon. Laparoscopic route for nephrectomy was retroperitoneal in 50 cases and transperitoneal in 10 cases. RESULTS: The patients included 37 males and 23 females. The mean age was 18.5 +/- 16.6 years and ranged from 2 years to 60 years. Right sided nephrectomy was performed in 31 cases while left kidney was removed in 29 cases. The indications for nephrectomy were stones (n = 29), dysplasia (n = 11), chronic pyelonephritis (n = 8), pelvi ureteric junction obstruction (n = 4), tuberculosis (n = 3), vesicoureteric reflux (n = 3) and ureterocele(n = 2). Mean operating time for laparoscopic nephrectomy alone was 140 +/- 51.1 min (range 25-300 minutes). In our series, 19 additional surgical procedures were performed under the same general anaesthesia. The mean hospital stay for all patients was 3.1 +/- 2.39 days; however the mean hospital stay for those patients who underwent laparoscopic nephrectomy without any additional procedure is 2.2 +/- 0.92 days (1.5-4 days). The mean size of kidney removed was 7.4 +/- 3.13 cm (4.6-15.9 cm). Complications were seen in 2 patients (3%); one developed wound infection and the other was found to have a haematoma for which the wound was re-explored. Conversion to open nephrectomy was required in 7 patients (11.6%) due to bleeding from renal pedicle (n = 4), poor vision secondary to pus spillage from pyonephrotic kidney (n = 2) and failure to progress due to indwelling percutaneous nephrostomy. There was no mortality and none of the patients had prolonged ileus. Blood transfusion was not required in any case. There was no bowel injury or port site herniation. CONCLUSION: Laparoscopic nephrectomy is a feasible and safe technique. The length of hospital stay and convalescence is short and hence rapid return to normal activity is expected.

Single-center review of fluoroscopy-guided percutaneous nephrostomy performed by urologic surgeons.

PURPOSE: To review the success rate and complications of radiologically guided percutaneous nephrostomies (PCNs) performed by urologists and compare the complication rates with the standards recommended by the Society of Interventional Radiology (SIR) and the American College of Radiology (ACR). PATIENTS AND METHODS: From January 1996 to December 2005, 667 patients had 765 PCNs performed by three urologists, with 74 patients having simultaneous bilateral PCNs. The mean age of the patients was 29 years (range 8 months-95 years). The medical records were reviewed for underlying diseases, success rate, and complications of PCN; and the results were assessed in comparison with recommendations made by SIR and ACR. RESULTS: The PCN was successful in 742 renal units (97%). A total of 26 of the 667 patients (3.89%) had major complications: 12 (1.79%) had sepsis, 10 (1.49%) had hemorrhage sufficient to necessitate transfusion, 1 (0.14%) had pleural injury that was managed conservatively, and another patient had a vascular complication necessitating nephrectomy. None of the patients had bowel transgression. Minor complications occurred in 61 patients (9.1%): urinary-tract infection in 17, PCN tube dislodgement in 11, catheter obstruction by clot or debris in 12, urine leakage around the PCN site in 8, and loss of the PCN catheter in 13. CONCLUSION: Percutaneous nephrostomy is a relatively safe, minimally invasive, and effective procedure with a low rate of morbidity. Our overall results in term of success rate and major complications are within the threshold limits set by the SIR and ACR. Hence, trained urologists can produce results similar to those of interventional radiologists. Learning of PCN should be mandatory in the training curriculum for all urology residents.

Percutaneous nephrolithotomy (PCNL) in horse shoe kidneys.

OBJECTIVE: To review our experience of PCNL in horse shoe kidneys. METHODS: Between June 2001 and January 2005 we performed PCNL in 16 Horse shoe kidneys in 14 patients with calculi, 2 patients had bilateral calculi. Percutaneous puncture was made with patient in prone position. Their ages ranged between 4 to 52 years with a mean age of 26.3 years. Our mean stone burden was 820 mm2 (range 40-1370 mm2). PCNL access tract was made in upper pole of the kidney in 13 (80%) while 3 (20%) had mid-pole access. RESULTS: Stone clearance after primary PCNL was achieved in 12 kidneys. Of the remaining 4 kidneys, 2 underwent re-look PCNL via the same tract and one more kidney was rendered stone free, while the other had an insignificant residue of 3mm. Hence we achieved a stone clearance rate of 81% after primary and secondary PCNL. Further 2 kidneys underwent single session ESWL for residual stones and became stone free improving our complete stone clearance rate after auxiliary procedure to 93.7%. Mean hospital stay for these patients was 3.8 days (range 3-10 days). Follow up available for these HSK patients with PCNL was 11 to 49 months with a mean follow up of 29.1 months. Complications were seen in 3 (21%) patients who developed transient post operative pyrexia and 1 (7%) patient presented on the 18th post operative day with a stone fragment in ureter and leakage from PCNL site. He was managed by ureteric catheterization for 48 hours when he passed the fragment spontaneously and became dry. None of our patients developed post PCNL bleeding or wound infection. CONCLUSION: PCNL in horse shoe kidneys is no more difficult than normal kidneys and does not carry a greater risk than reported for normal kidneys. This effective modality resulted in almost 93% stone clearance with minimal complications.

Does percutaneous nephrolithotomy in children cause significant renal scarring?

OBJECTIVE: To determine the frequency of renal parenchymal damage following percutaneous nephrolithotomy (PCNL) in children. PATIENTS AND METHODS: Fifty-six children undergoing PCNL in 60 renal units between January 2000 and December 2004 were included in this prospective study, and were subjected to postoperative technetium-99m dimercaptosuccinic acid ((99m)Tc-DMSA). Using a standard questionnaire, demographics, number, size and location of stones, procedure details, outcome as indicated by clearance with PCNL alone or additional procedures, and follow up were documented. Presence of focal renal damage and its association with the PCNL tract were examined. RESULTS: Out of 60 renal units, cortical defects on (99m)Tc-DMSA scan were seen in 10 renal units (17%). In three of these kidneys, the site of focal defect corresponded to the access site for tract formation during PCNL. Two additional kidneys had scarring at multiple sites, one of which corresponded to the access site during PCNL. In the remaining five kidneys no association between focal renal damage and nephrostomy tract site could be ascertained. No association was seen between renal damage and the size of nephroscope used during PCNL. CONCLUSION: There exists a risk of focal damage to renal parenchyma from the formation of the nephrostomy tract. In our series, focal damage was seen in 5% of patients; this may be an overestimate since preoperative (99m)Tc-DMSA scans were not available for our patients. Meticulous technique is important combined with a smaller nephroscope to minimize renal damage. Long-term follow up of such children is required to assess how many are left with permanent renal scars.

Pregnancy after augmentation cystoplasty.

We report a case of 26 year old female who underwent ileo-cystoplasty for interstitial cystitis and two years later delivered a live baby through a lower segment caesarean section (LSCS) at 36 weeks.

Laparoscopic assisted percutaneous nephrolithotomy (PCNL) in ectopic pelvic kidney.

We report the case of a patient with pain and an abdominal palpable mass whose tests showed a right pelvic kidney with a 4-cm stone in the renal pelvis. We describe the successful management through laparoscopic assisted percutaneous nephrolithotomy (PCNL) in ectopic pelvic kidney, stressing that this method is a minimally invasive therapeutic option in such cases.

Paediatric percutaneous nephrolithotomy: setting new frontiers.

OBJECTIVE: To assess the safety and outcome of paediatric percutaneous nephrolithotomy (PCNL) for atypical cases and compare the results with 'standard' unilateral paediatric PCNL. PATIENTS AND METHODS: We retrospectively reviewed children who had had a PCNL between December 1997 and December 2004. Patients were grouped as follows: group 1, aged >5-16 years with normal anatomy and normal renal function undergoing unilateral PCNL or staged bilateral PCNL; group 2, < or = 5 years with normal anatomy and renal function undergoing unilateral PCNL; group 3, undergoing bilateral simultaneous PCNL; group 4, impaired renal function in addition to renal stone disease; group 5, renal anatomical abnormality with calculi in the same kidney. Demographics, stone profile, procedure and outcome indicators were analysed for each group. RESULTS: In all, 188 consecutive PCNLs in 169 children were included (mean age 3.3-10.3 years, mean stone burden 19.1-33.3 mm in the five groups). The mean duration of PCNL was 69-115 min. Stone clearance was satisfactory with single tract access in 90-100% of patients. Transient postoperative fever was the commonest complication (12.5-51%) followed by hyponatraemia and hypokalaemia. Blood transfusion was required in 0-7.7%. The mean stone clearance rates were 47-90% in the five groups; additional extracorporeal shockwave lithotripsy increased the cumulative clearance rates to 90-100%. CONCLUSION: PCNL is safe for treating renal stones, with excellent results and minimal complications. Comparable results are achieved in the very young child, children with anatomically abnormal kidneys, children with impaired renal function and children with bilateral renal stones undergoing simultaneous bilateral PCNL. Hence none of these factors should be considered as relative contraindications.

Comparison of 24 hours urinary citrate levels in urolithiasis patients and healthy controls.

OBJECTIVE: To identify the difference in urinary citrate excretion between Stone Formers (SF) and Healthy Volunteers (HV) as a metabolic risk factor, that predisposes to urinary stone formation and to compare levels of urinary citrate in (HV) with reference values. METHODS: The 24 hours urinary citrate was evaluated in 40 patients treated for renal citrate and declared stone free, and 40 age matched healthy adults taken as controls. Both the groups had a similar living environment, extrinsic factors, diet and similar genetic descent. RESULTS: There was no significant difference in urinary citrate excretion level among stone formers (mean 262 SD 197) and normal volunteer subjects (mean 269 SD 140). Using the previously defined normal values (200) of urinary citrate in the local population, 55% of stone patients in our study group were hypocitric. While using the same value, 45% of our normal volunteers were also hypocitric. If 320 was taken as normal limit, 70% of the patient's population and 72% of controls were hypocitric. The prevalence of hypocitraturia was similar in the age matched adult groups. CONCLUSION: Certain intrinsic factors in our local subjects may account for the high prevalence of urolithiasis than in western population. Although the urinary citrate excretion of stone patients is similar to normal volunteers, uniformly low urinary citrate excretion may be a feature as a nation and not a predisposing factor for the lithogenesis. This supports the view that there may be more often prominent influences in stone formers possibly of genetic origin.

Bed wetting in school children of Karachi.

OBJECTIVES: To estimate the frequency of primary nocturnal enuresis (PNE) in Pakistani children and to examine the factors associated with it. METHODS: A randomly selected cross-sectional study was conducted in five elementary schools, one in each of five districts of Karachi. The parents of 5000 children age between 3-13 years were asked to complete a questionnaire which included items about the frequency of daytime wetting and nocturnal enuresis, family history, urinary tract infection, parents and child's own concern about this problem and acquisition of treatments. RESULTS: Over all corrected response rate to the questionnaire was 69% (3395). Enuresis was present in 9.1% (308). There were 166 (53.9%) boys and 142 (46%) girls with a median age of 7 years. Only 54% (166) children sought help for their problem of which 26% consulted doctors, 16% visited homeopaths while 11% used hakeems and home remedies. Of the bed wetters, 30% were wet every night, 30% for more than three nights a week and 40% for less than three nights every week. Parents of 68.5% (211) children reported concern for the problem while 69.8% (215) children were also anxious about their enuresis. Among the concerned children group, 22% parents were not concerned about their child's problem. Family history of enuresis was present in 25.6% (79) children. CONCLUSION: The frequency of enuresis among the school going children in Karachi is 9.1% and is similar to that reported in European countries and other Asian countries including Korea and Taiwan. Enuresis causes concern to both parents and children, but only a small percentage of parents seek medical help for this problem.