Ocular manifestations and surgical management of lid coloboma in a Chinese infant with linear nevus sebaceous syndrome

An 8-month-old Chinese girl with linear nevus sebaceous syndrome had a right upper lid coloboma and right superior scleral ectasia. At 20 months old, successful surgical repair of the lid coloboma was performed with particular attention paid to the sclera ectasia.     

Factors influencing survival in children with recurrent neuroblastoma

Despite advances in multimodal therapy for neuroblastoma, survival from advanced disease remains poor. Children are now offered a wide variety of salvage regimens following relapse. A retrospective review was performed on 31 patients with recurrent neuroblastoma treated at one institution between 1995 and 2001. At initial diagnosis, 27 patients had metastatic disease and 11 had N-myc amplification (NMA). The median time to recurrence from diagnosis was 16.1 months. Seventeen patients received salvage therapy, with a median of three salvage regimens per patient. The median survival time from relapse was 8.4 months. The median survival time was significantly shorter for recurrence less than 6 months after stem cell transplantation (2.9 vs. 13.3 months; P = 0.003) and for patients with NMA (2.7 vs. 15.1 months; P < 0.0001). Overall, salvage therapy led to a significantly longer median survival time (22.4 vs. 3.3 months; P = 0.0003); however, salvage therapy extended the median survival time only from 2.2 to 3.2 months for patients with NMA and from 0.7 to 5.8 months for patients with early relapse after stem cell transplantation. Multiple salvage regimens prolong survival significantly, especially for patients with no NMA and for relapses more than 6 months after stem cell transplantation, but the long-term disease-free survival after recurrent disease remains dismal.     

SIADH associated with ovarian immature teratoma: a case report

Immature teratoma in association with SIADH is rare. A 17-year-old patient presented with a pelvic mass and serum sodium concentration of 121 mmol/l. Subsequent investigation confirmed SIADH and grade 2 ovarian immature teratoma. No other causes of SIADH were found apart from the immature teratoma. There was no further recurrence of SIADH after the curative surgery and chemotherapy. We postulate that immature teratoma consists of neurohypophyseal structures which account for the ADH release.     

Synthesis of vitamin D in skin after burns

Severe burn injury is associated with vitamin D deficiency, low bone turnover, and abnormalities in calcium homoeostasis. Patients do not routinely receive vitamin D supplementation and sun exposure is currently not controlled. By analysis of skin biopsy samples for vitamin D3 precursors after exposure to ultraviolet B light we found that the conversion of 7-dehydrocholesterol to previtamin D3 was reduced in children a mean of 14 months after the burn. Low serum 25-hydroxyvitamin D concentrations were also found. We conclude that vitamin D supplementation is necessary after burn injury.     

Quantifying agitation in sedated ICU patients using heart rate and blood pressure

Agitation is a significant problem in the intensive care unit (ICU), affecting 71% of sedated adult patients during 58% of ICU patient-days (Fraser and Riker 2001 NY Health-Syst. Pharm. 20 17-24). Subjective scale-based assessment methods are currently used to assess the level of patient agitation, but are limited in their accuracy and resolution. This research develops an objective agitation measurement method using heart rate variability (HRV), systolic blood pressure (BP) and blood pressure variability (BPV) data, processed by wavelet transforms and autoregressive signal processing. A fuzzy inference system (FIS) is developed to classify changes in these signals that correlate with observed patient agitation, and combine them into a final agitation level. Proof of concept clinical trials on 13 normal subjects and 5 ICU patients has been performed to verify the validity of this approach in comparison with agitation graded by nursing staff using the Riker sedation-agitation scale (SAS). Results show good correlation with medical staff assessment with no false positive results during calm periods. Clinically, this initial agitation measurement method promises the ability to consistently and objectively quantify patient agitation to enable better management of sedation and agitation through optimized drug delivery leading to reduced length of stay.     

Neuroanatomy of adult strabismus: a voxel-based morphometric analysis of magnetic resonance structural scans

Cerebral deficit has been implicated in the genesis of strabismus and in the mechanisms adopted to compensate for the visual disorder. Voxel-based morphometry (VBM) was applied to magnetic resonance images of strabismic adults to detect any abnormal brain anatomy, which could not be easily identified by simple inspection. The gray matter volume in strabismic adults was smaller than that in normal subjects at the areas consistent with the occipital eye field (OEF) and parietal eye field (PEF). However, greater gray matter volume was found in strabismic adults relative to normal controls at the areas consistent with the frontal eye field (FEF), the supplementary eye field (SEF), the prefrontal cortex (PFC), and subcortical regions such as the thalamus and the basal ganglia. These opposite gray matter changes in the visual and the oculomotor processing areas are compatible with a hypothesis of plasticity in the oculomotor regions to compensate for the cortical deficits in the visual processing areas.     

Screening of colonic tumors by air-inflated magnetic resonance (MR) colonography

PURPOSE: To assess the accuracy of air-inflated magnetic resonance (MR) colonography for the detection of colonic lesions. MATERIALS AND METHODS: A total of 36 patients underwent both colonoscopy and air-inflated MR colonography. Breath-hold sequences (volumetric interpolated breath-hold examination (VIBE) coronal, and half-Fourier acquisition single-shot turbo spin-echo (HASTE) axial and coronal, both supine and prone) were performed with a 1.5T scanner. The detection of colonic lesions by MR colonography was then correlated with the findings from the colonoscopy performed on the same day. RESULTS: Two patients were unable to complete the MR colonography examination. Analysis was based on the results from 34 patients (17 males and 17 females, 38-70 years old, mean age = 54.9 years) who completed both examinations. MR colonography depicted two of two colonic tumors, one of one P4 (> 2 cm) polyp, one of two P2 (0.5-1 cm) polyps, and two of 11 P1 (< 0.5 cm) polyps. False-positive MR colonography interpretations were noted for one P1 polyp and two P2 polyps. The overall sensitivity, positive predictive value, and accuracy of MR colonography were 38%, 67%, and 46.2%, respectively. For the detection of endoluminal lesions > 5 mm, air-inflated MR colonography yielded a sensitivity of 75%, specificity of 93.3%, accuracy of 91.2%, positive predictive value of 60%, and negative predictive value of 96.6%. CONCLUSION: Air-inflated MR colonography is a new technique that deserves further investigation.     

Surgical treatment for primary hyperparathyroidism in Hong Kong: changes in clinical pattern over 3 decades

HYPOTHESIS: With the introduction of the blood chemistry multichannel autoanalyzer, primary hyperparathyroidism (HPT) is increasingly diagnosed. The clinical pattern of primary HPT has undergone a significant evolution in Western countries. A similar change can be documented in a geographic region where this condition is considered to be relatively uncommon. DESIGN: Unselected case series. SETTING: A tertiary referral endocrine surgical unit. PATIENTS: All patients with primary HPT surgically treated over the past 30 years. MAIN OUTCOME MEASURES: The prevalence of patients per 100,000 hospital admissions, clinical presentation, biochemistry study results, pathologic status, and main outcome were compared over three 10-year spans according to the introduction of the multichannel autoanalyzer in 1982: 1973-1982 (n = 20), 1983-1992 (n = 31), and 1993-2002 (n = 190). RESULTS: A 7-fold increase in the prevalence of patients with primary HPT who were surgically treated per 100,000 hospital admissions was observed over the past 10 years. The clinical presentation of patients with primary HPT had evolved progressively with a higher proportion of older patients (P<.001) being asymptomatic. On presentation, the condition had decreased in severity with lower serum calcium (P =.04), parathyroid hormone (P<.001), and alkaline phosphatase levels (P<.001) as well as a smaller adenoma size (P<.001). There was no significant change in the underlying pathologic condition and surgical success. CONCLUSION: Similar to the West but in contrast to that observed in other Asian countries, an increase in the prevalence of patients surgically treated for primary HPT is documented and a change in disease presentation as well as its severity is observed in our population group.     

Microvascular reconstruction of the hepatic artery in live donor liver transplantation: experience across a decade

HYPOTHESIS: Hepatic arterial anastomosis by means of a microvascular technique can be performed with a high success rate in live donor liver transplantation in adult recipients. DESIGN: Retrospective analysis of data collected prospectively. SETTING: Tertiary referral center. PATIENTS: From September 28, 1993, through December 23, 2002, 28 children received left lateral segment grafts (n = 23) or left lobe grafts (n = 5), and 124 adults received left lobe (n = 6) or right lobe (n = 118) grafts. Microvascular technique was used for hepatic arterial anastomosis. Attention was paid to exposure, orientation of the axis of arterial ends, and matching of size. Long microinstruments were used for arterial ends deeply located inside an adult's abdominal cavity. An average of 16 stitches was used for a vessel anastomosis 3 mm in diameter. INTERVENTIONS: Intraoperative and postoperative Doppler ultrasonography were performed. MAIN OUTCOME MEASURES: Hepatic artery thrombosis rate. RESULTS: All hepatic arterial anastomoses were patent after reconstruction. Complications occurred in 3 patients. They had hepatic arterial thrombosis at 19 days, 25 days, and 3 months after liver transplantation. The overall complication rate was 2%. CONCLUSION: With attention to exposure, appropriate instruments, and experience, a low complication rate of 2% can be achieved in hepatic arterial anastomosis by using a microvascular technique, even in adult patients with the liver graft situated deeply in the abdominal cavity.     

Ureteroscopic management of upper tract transitional cell carcinoma

The expanding experience with endoscopic techniques for treating upper tract urothelial malignancy demonstrates its safety and efficacy in carefully selected patients. Diagnostic accuracy can be enhanced, and pathologic confirmation of tumor grade and stage is possible. In carefully selected patients who have low-grade and low-stage disease, the results of endourologic management have been encouraging. Patients with an anatomic or functionally solitary kidney, bilateral disease, or significant renal insufficiency can often be considered candidates for endoscopic treatment as the first line of therapy. In the setting of low-grade, low-stage disease in a patient with a normal contralateral kidney, the role of endourologic management remains controversial. Adjuvant topical therapy with mitomycin C or BCG seems to be safe and well tolerated after endoscopic management of upper tract TCC.