Dissecting aneurysms of the posterior inferior cerebellar artery: retrospective evaluation of management and extended follow-up review in 6 patients

OBJECT: The authors report the management protocol and successful outcomes in 6 patients with dissecting aneurysms of the posterior inferior cerebellar artery (PICA). METHODS: Medical records and neuroimaging studies of 6 patients who underwent surgical treatment of dissecting PICA aneurysms were reviewed. The mean follow-up duration was 1.8 years. No patient was lost to follow-up review. RESULTS: Four patients presented with acute subarachnoid hemorrhage and 2 with PICA ischemia. All patients underwent surgery, which entailed proximal occlusion with distal revascularization in 3 cases and circumferential wrap/clip reconstruction in 3 cases. The revascularization techniques used were occipital artery-PICA bypass and PICA-PICA anastomosis. Delayed follow-up angiography was performed in all cases. In patients treated with proximal occlusion, delayed angiography showed minimal retrograde opacification of the dissected segments. The 3 patients treated with wrap/clip reconstruction showed unexpectedly significant normalization of their lesions on angiographic studies. Outcome was good in all cases. CONCLUSIONS: Dissecting PICA aneurysms are rare lesions with an apparent propensity for bleeding. Individualized management including distal revascularization with PICA sacrifice or circumferential wrap/clip reconstruction to reinforce the dissected segment produced good outcomes. Patients treated with aneurysm wrapping may show dramatic angiographic improvement of the dissected segment.     

Compound palmar ganglion with carpal tunnel syndrome

Compound palmer ganglion is an uncommon condition characterized by a swelling in the distal part of volar aspect of wrist and communicating with another swelling over palm across the flexor retinaculum. It commonly results from tuberculous tenosynovitis. It may lead to carpal tunnel syndrome and need surgical excision with division of flexor retinaculum.     

Atypical presentation of an impacted radiolucent esophageal foreign body

Patients with impacted esophageal foreign bodies usually present with gastrointestinal and rarely with respiratory symptoms. Impacted esophageal foreign bodies may be identified by radiologic studies. Ingested radiolucent foreign bodies may be more difficult to diagnose, especially if the patient presents with minimal symptoms. We report a rare case of a child who presented with stridor and obstructive sleep apnea. The cause of respiratory symptoms was thought to be due to enlarged tonsils and adenoids, and the patient was scheduled for tonsillectomy and adenoidectomy. On re-evaluation by the surgeon on the day of surgery, the procedure was changed to diagnostic microlaryngoscopy and bronchoscopy to rule out any other cause. The patient’s respiratory symptoms were resolved when an incidental discovery and retrieval of the radiolucent esophageal foreign body was made. The diagnosis of radiolucent esophageal foreign body can be difficult and can be easily missed without reasonable clinical suspicion.     

Encountering Meckel's diverticulum in emergency surgery for ascaridial intestinal obstruction

Background  

Meckel's diverticulum is the most common congenital anomaly of the gastrointestinal tract. In children with intestinal ascariasis, the diverticulum remains asymptomatic or rarely the Ascaris lumbricoides may lead to its complications in the presence of massive intestinal roundworm load. Given that preoperative diagnosis is seldom carried out, when Meckel's diverticulum is found at laparotomy for obstructive intestinal complications of roundworm, the diverticulum should be removed as complications may occur at any time. The aim of this study was to describe the findings of concomitant presence of Meckel's diverticulum who had surgical intervention in symptomatic intestinal ascariasis in children.     

Long-term followup of patients after redo bladder neck reconstruction for bladder exstrophy complex

PURPOSE: The aim of this study was to determine whether redo bladder neck reconstruction is effective in achieving continence after a failed bladder neck reconstruction procedure. MATERIALS AND METHODS: We retrospectively reviewed the hospital records of patients with bladder exstrophy who had undergone redo bladder neck reconstruction. There were 30 patients in the study, including 20 boys and 10 girls. Mean patient age at redo bladder neck reconstruction was 9.3 years (range 3.2 to 15.5). The patients were divided into 3 groups on the basis of the preoperative pattern of incontinence--incomplete wetters, complete wetters and those on continuous suprapubic drainage. Of the patients 15 already had undergone bladder augmentation, 12 had undergone a Mitrofanoff procedure and 12 had been treated with bulking agents injected in the bladder neck in an attempt to achieve continence. Four patients had undergone more than 1 bladder neck procedure. The patients were investigated with a combination of noninvasive urodynamics, cystoscopy, cystogram and ultrasound. All patients underwent Mitchell's modification of Young-Dees-Leadbetter bladder neck reconstruction. Additional procedures performed included augmentation cystoplasty and Mitrofanoff formation. RESULTS: Mean followup was 6.9 years (range 1.2 to 15.5). Postoperatively 28 patients were using clean intermittent catheterization to empty the bladder (5 per urethra, 23 via Mitrofanoff). Two patients remained on continuous suprapubic catheter drainage. A total of 18 patients (60%) were dry postoperatively (80% of girls and 50% of boys). Among dry patients only 3 were performing clean intermittent catheterization per urethra and 15 via a Mitrofanoff channel. No patient was able to void per urethra without the need for clean intermittent catheterization. The 2 patients on continuous suprapubic catheter drainage continued to remain so. At night only 50% of the patients were dry (5 on free drainage, 4 on clean intermittent catheterization, 6 not on any drainage). Those patients who did not respond satisfactorily to redo bladder neck reconstruction underwent subsequent additional procedures, which included injection of bulking agents (3 patients), insertion of an artificial urinary sphincter (1), Mitrofanoff formation (2) and bladder augmentation plus Mitrofanoff channel (1). Postoperative complications included difficulty with clean intermittent catheterization (8 patients), perivesical leak (1), recurrent epididymo-orchitis (1), upper urinary tract dilatation (2) and incisional hernia (1). Bladder neck closure was being considered in 5 patients. CONCLUSIONS: In our experience redo bladder neck reconstruction cannot achieve continence with volitional voiding per urethra. Although redo bladder neck reconstruction can render a significant number of patients dry, it is only effective if performed in conjunction with augmentation. Failure of the initial bladder neck reconstruction may be a reflection of a bladder that is of inadequate capacity and/or compliance. Therefore, bladder augmentation should be considered in all patients requiring redo bladder neck reconstruction. Bladder neck closure may be a better alternative to redo bladder neck reconstruction.     

Association between granzyme B and perforin I polymorphisms and allograft outcomes in Hispanic kidney transplant recipients

Granzyme B (GZMB) and perforin 1 gene (PRF1) are key effector molecules of cytotoxic T lymphocytes, in causing acute and chronic solid organ transplant rejection. In this study, we analyzed the impact of GZMB and PRF1 polymorphism on kidney allograft outcomes. In all, 527 de novo kidney Hispanic allograft recipients were genotyped for PRF1 (rs10999426, rs35947132) and GZMB (rs8192917, rs7144366). PRF1 (rs10999426, rs35947132) G alleles and GG genotypes were negatively associated with allograft rejection, demonstrating protection against allograft rejection (OR = 0.61, p = 0.005 for rs1099946; OR = 0.4, p = 0.01 for rs 35947132). On the other hand, the GA heterozygosity of PRF1 was found marginally associated with the rejection group (OR = 1.53, p = 0.05 for rs10999426; OR = 2.24, p = 0.07 for rs35947132). There was a significant increase in allograft survival in time period studied for the PRF1 (rs10999426) GG genotype, while the GA heterozygosity was associated with graft failure. We found no association for polymorphic markers in GZMB gene with allograft rejection. Survival was significantly improved for patients who were homozygous TT for the GZMB (rs8192917) (TT vs. CC/TT, p = 0.041). The result suggests that PRF1 and GZMB gene polymorphisms may determine the incidence of acute rejection or graft survival among Hispanic allograft recipients.     

Irreversible Loss of Vision in a Child due to Occipital Infarction after Gastroenteritis

A 2½-year-old girl developed a bilateral occipital infarct following severe gastroenteritis with bilateral vision of light perception. Evaluations for sickle cell anemia, hemolytic anemia and coagulopathies were negative. Cortical blindness is an uncommon but dramatic complication of gastroenteritis, hence the need of prompt hydration and other supportive measures to avoid irreversible visual loss or mental sequela.Key Words: Occipital infarct, Gastroenteritis, Visual recovery     

Identification of hemoglobin Q India (α 1–64 Asp–His) through ARMS-PCR. First report from Pakistan

Various hemoglobinopathies have been reported from Pakistan excepting the rare ones like hemoglobin Q India. Our purpose of study was to identify the mutation (α 1 64 aspartate to histidine) through amplification restriction mutation system-polymerase chain reaction (ARMS-PCR) in patients where hemoglobin Q has been detected via high performance liquid chromatography (HPLC) and also to evaluate the cost effectiveness of the two technologies. All patients irrespective of age and gender who underwent HPLC for identification of their hemoglobin variant during January 1, 2006 to January 30, 2007 were studied. The blood samples with unknown peak at a retention time of 4.7 min were evaluated at the molecular level. Analysis of HPLC tracings of 11,008 subjects over a thirteen-month period identified ten individuals with hemoglobin Q. Male to female ratio was 1:1.5 and their age was variable ranging from 1 to 49 (mean 22.8) years. The mean hemoglobin level was 11.3 g/dl while MCV (fl) and MCH (pg) were 73.0 and 20.8 respectively. HPLC showed an unknown peak of 17.7% which was detected as Hb Q. ARMS based PCR showed Hb Q specific product of 370 bp and also an amplified product of 766 bp as the control fragment in these samples. This is the first ever report that documents the presence of Hb Q India (α 64 Asp to His) in Pakistani population. We recommend that HPLC be used as a useful screening tool especially in developing countries where PCR facilities may not be accessible.