Hypospadias surgery in children: improved service model of enhanced recovery pathway and dedicated surgical team.

INTRODUCTION: Children in Hong Kong are generally hospitalised for 1 to 2 weeks after hypospadias repairs. In July 2013, we introduced a new service model that featured an enhanced recovery pathway and a dedicated surgical team responsible for all perioperative services. In this study, we investigated the outcomes of hypospadias repair after the introduction of the new service model. METHODS: We conducted a retrospective study on consecutive children who underwent primary hypospadias repair from January 2006 to August 2016, comparing patients under the old service with those under the new service. Outcome measures included early morbidity, operative success, and completion of enhanced recovery pathway. RESULTS: The old service and new service cohorts comprised 176 and 126 cases, respectively. There was no difference between the two cohorts in types of hypospadias and surgical procedures performed. The median hospital stay was 2 days in the new service cohort compared with 10 days in the old service cohort (P<0.001). Patients experienced less early morbidity (5.6% vs 15.9%; P=0.006) and had a lower operative failure rate (20.2% vs 44.2%; P<0.001) under the new service than the old service. Multivariable analysis revealed that the new service significantly reduced the odds of early morbidity (odds ratio=0.35, 95% confidence interval=0.15-0.85; P=0.02) and operative failure (odds ratio=0.32, 95% confidence interval=0.17-0.59; P<0.001) in comparison with the old service. Of the new service cohort, 111(88.1%) patients successfully completed the enhanced recovery pathway. CONCLUSIONS: The enhanced recovery pathway can be implemented safely and effectively to primary hypospadias repair. A dedicated surgical team may play an important role in successful implementation of the enhanced recovery pathway and optimisation of surgical outcomes.

Recovery of mouse neuromuscular junctions from single and repeated injections of botulinum neurotoxin A.

Botulinum neurotoxin type A (BoNT/A) paralyses muscles by blocking acetylcholine (ACh) release from motor nerve terminals. Although highly toxic, it is used clinically to weaken muscles whose contraction is undesirable, as in dystonias. The effects of an injection of BoNT/A wear off after 3-4 months so repeated injections are often used. Recovery of neuromuscular transmission is accompanied by the formation of motor axon sprouts, some of which form new synaptic contacts. However, the functional importance of these new contacts is unknown. Using intracellular and focal extracellular recording we show that in the mouse epitrochleoanconeus (ETA), quantal release from the region of the original neuromuscular junction (NMJ) can be detected as soon as from new synaptic contacts, and generally accounts for > 80% of total release. During recovery the synaptic delay and the rise and decay times of endplate potentials (EPPs) become prolonged approximately 3-fold, but return to normal after 2-3 months. When studied after 3-4 months, the response to repetitive stimulation at frequencies up to 100 Hz is normal. When two or three injections of BoNT/A are given at intervals of 3-4 months, quantal release returns to normal values more slowly than after a single injection (11 and 15 weeks to reach 50% of control values versus 6 weeks after a single injection). In addition, branching of the intramuscular muscular motor axons, the distribution of the NMJs and the structure of many individual NMJs remain abnormal. These findings highlight the plasticity of the mammalian NMJ but also suggest important limits to it.

Clinical heterogeneity in children with gonadal dysgenesis associated with non-mosaic 46,XY karyotype.

INTRODUCTION: Gonadal dysgenesis is unique in disorders of sex development (DSD), in that it can be associated with 46,XX, 46,XY or mosaic 45,X/46,XY karyotypes. Gonadal dysgenesis can be partial or complete. Gonadal dysgenesis associated with the Y-chromosome has increased risk of gonadal germ cell neoplasms. Most of the literature focus on 45,X/46,XY gonadal dysgenesis, while there are scanty data on the condition when the karyotype is non-mosaic 46,XY. OBJECTIVE: To investigate the diversity of clinical pictures of children presenting with 46,XY DSD due to gonadal dysgenesis. METHODS: A retrospective study on consecutive patients diagnosed with 46,XY gonadal dysgenesis at age ≤18 years in a tertiary center from 1985 to 2015. The clinical presentations, phenotypes, gonadal features and associated anomalies were investigated. RESULTS: Twenty-eight patients with Y-chromosome gonadal dysgenesis were identified during the study period and six (21.4%) had non-mosaic 46,XY karyotype. Three had complete gonadal dysgenesis (CGD) with normal female phenotype, while the other three had partial gonadal dysgenesis (PGD). Of the three patients with CGD, two presented with the classical Swyer syndrome at adolescence, while the third presented at birth with multiple congenital anomalies. The three PGD patients presented with ambiguous genitalia at birth (n = 2), and isolated hypospadias (n = 1), which was associated with Frasier syndrome. Three patients had germ cell neoplasms: bilateral gonadoblastoma (n = 1), bilateral intratubular germ cell neoplasia unclassified (n = 1), and dysgerminoma + gonadoblastoma (n = 1). Two patients had global developmental delay with other congenital anomalies, and another patient had learning difficulties with borderline intelligence (Table). DISCUSSION: The findings suggest that 46,XY gonadal dysgenesis is much rarer than 45,X/46,XY gonadal dysgenesis. Patients differed in their clinical presentations and well-established syndromes happened in half of them. Overall, the risk of germ cell neoplasms and the association with other somatic anomalies appeared to be high. The study was limited by: its small number, single-center experience, and the possibility of missing the diagnosis in some male patients with mild undervirilization. CONCLUSION: Heterogeneity was noted in the clinical, phenotypic and gonadal features among pediatric patients with 46,XY gonadal dysgenesis.

Ureteral stents and flexible ureterorenoscopy.

Numerous methods of dilating the intramural ureter for ureteroscopy are effective when used by experts, but none is ideal. In the authors' experience, a ureteral stent left indwelling for 1 or, better, 2 weeks usually is the safest, most reliable, and least traumatic method.

Liver abscess concomitant with hemobilia due to rupture of hepatic artery aneurysm: a case report.

Hepatic artery aneurysm is an uncommon lesion, usually extrahepatic and rarely intrahepatic. Rupture of hepatic artery aneurysm into the biliary tract is a rare cause of hemobilia. Angiography is the diagnostic method of choice and surgical intervention is the procedure of choice for extrahepatic rupture. Interventional angiography with embolization is optimal for inaccessible intrahepatic aneurysm and extremely poor risk patients. Reported here is a rare case of left intrahepatic artery aneurysm ruptured into the intrahepatic bile duct in the course of liver abscess. Embolization of the hepatic artery resulted in cessation of bleeding.

Small cell carcinoma of the stomach. Case report.

Small cell carcinoma of the stomach is a rare neoplasm. We report the barium meal and computed tomography findings of a 70-year-old man who presented with a long history of postprandial abdominal pain.

Atypical skeletal tuberculosis mimicking tumor metastases: report of a case.

Skeletal tuberculosis (TB) generally involves the spine and large joints. Involvement of most other bones has been reported, but tuberculosis of the pubic symphysis is relatively unusual. This paper reports an unusual case of multiple bone lesions in the right symphysis, left sacroiliac joint and left elbow. The radiologic appearance simulated widespread metastatic disease or chondrosarcoma, but the diagnosis of tuberculosis was proven by biopsy and culture. Eight months after starting antituberculous treatment, the patient experienced an improvement in pain and limping gait, and felt well. Radiography of the pelvis also showed improvement. It is proposed that antituberculous therapy in such cases should be maintained for at least 1 year.

Detecting alcohol consumption as a cause of emergency general medical admissions.

In a general medical unit 27% of 104 admissions and 17% of bed occupancy were attributed to alcohol consumption, although only 10 of these 28 patients had classical alcohol-related conditions. Questioning on the amount of alcohol consumed was the most accurate method of screening for these patients. The brief MAST questionnaire, mean red cell volume, gamma-glutamyl transferase, aspartate transaminase and urate were all inadequate as screening tests. Only 13% of the admissions were defined as 'problem drinkers' on the brief MAST questionnaire, suggesting that harmful effects of alcohol are not confined to 'alcoholics'. Clinical suspicion and questions on the level of alcohol consumption are more efficient than questionnaire, biochemical or haematological screening tests in detecting alcohol-related medical problems.

The clip and drop back technique in the management of multifocal necrotizing enterocolitis: a single centre experience.

AIM: The surgical management of multifocal necrotizing enterocolitis (NEC) remains a major challenge. The "clip-and-drop" strategy with a second-look laparotomy permits re-assessment of bowel viability after optimization, thus offering the potential of both improving survival and conserving bowel length. This study reviews the outcome of this strategy in a single regional center. METHODS: Since 2000, NEC patients undergoing emergency laparotomy selectively underwent a "clip-and-drop" operation if there was peri-operative instability and/or multifocal disease with uncertain bowel viability. Bowel with full thickness gangrene was resected and bowel-ends were temporarily tied-off; a second-look definitive procedure was performed when the patient had stabilized. For this review, in-hospital and follow-up records were studied retrospectively for demographics, 30-day mortality and long-term outcome. MAIN RESULTS: Between 2000 and 2010, 16 patients underwent a "clip-and-drop" operation. The mean post-conception age was 32.8 weeks (27.7-41.7 weeks) with a median body weight of 1.4 kg (0.76-4.4 kg) at first operation. Preoperative radiograph showed free gas in 43.8% and portal venous gas in 37.5% of patients. 2 patients did not survive to the second laparotomy. 14 patients received a second laparotomy, after a mean of 51 h (35-74 h). 2 patients were found to suffer from NEC totalis on the second laparotomy and died without further procedures. All other patients (n = 12) had stoma formation. 1 patient died 4 days after stoma formation. The 30-day mortality for NEC with the "clip-and-drop" strategy was 31.6% (5/16). Among the 11 survivors, 1 died from liver failure complicated by short bowel syndrome at 5 months post operation, 2 others died from respiratory complications of prematurity despite adequate gastrointestinal function. The median follow-up time for the 8 long-term survivors was 45 months (7-129 months). Their median time to achieving full feeds was 41 days (range 21-105 days) after the second operation. CONCLUSION: The "clip-and-drop" strategy, when used in selected patients with multifocal NEC, may help bowel conservation in survivors.

Use of computed tomography to detect hepatic portal venous gas and intestinal intramural gas: a case report.

Hepatic portal venous gas and intestinal intramural gas were demonstrated by computed tomography (CT) in a case of ischemic bowel disease. The patient died on the day following the examination.

Early CT findings of acute cerebral infarction in the middle cerebral artery territory.

For an early and definitive diagnosis of acute cerebral infarction by computed tomography (CT), we retrospectively analyzed the initial CT findings of 14 patients with proven acute middle cerebral artery (MCA) infarction within 4 hours after stroke onset. The following results were obtained: (a) abnormal CT findings could be recognized quite early in 13 patients (92.9%), (b) the most common and earliest finding was loss of defination of the gray-white interface at the lateral margins of the insula. This sign was recognized in 12 patients (85.7%) and could be detected as early as 1 hour after stroke onset, (c) the next common finding was narrowing or blurring of the Sylvian fissure in 10 patients (71.4%) and could be detected 1.5 hours after stroke onset, (d) an obscured outline or partial disappearance of the lentiform nucleus was recognized in 7 patients (50%) and could be detected 1.5 hours after stroke onset, (e) effacement of the cerebral sulci was found in 6 patients (42.9%) and could be detected 2.5 hours after stroke onset, (f) increased density in MCA or its major branches was not seen in our patients.

Plain film radiographic findings in lumbar disc protrusion: correlation with computed tomography iohexol myelography.

A retrospective study was devised to determine the correlation between plain film radiographic findings and computed tomography iohexol myelography in the recognition of lumbar disc protrusion. A group of 61 patients who had chronic low back pain seen in Taitung Mackay Memorial hospital from January 1, 1988 to December 31, 1988 was studied. In comparison of 207 lumbar disc protrusion, reaching an accuracy close to 80% but cannot distinguish between disc bulging or herniation. Spur formation correlated to a lesser extent. Subluxation of facet joints, discogenic sclerosis and vacuum phenomenon were all specific to disc protrusion; however, their rare occurrences have limited their usefulness in the recognition of disc protrusion.