Quality of life and defecative function 10 years or longer after posterior sagittal anorectoplasty and laparoscopic-assisted anorectal pull-through for anorectal malformation.

PURPOSE: In our center, patients with anorectal malformation, including males with recto-vesical (RV)/recto-bladder neck (RBN)/recto-prostatic urethral (RU) fistulas, and females with recto-vaginal (RV) fistulas have been treated by posterior sagittal anorectoplasty (PSARP) before 2000, and by laparoscopic-assisted anorectal pull-through (LAARP) thereafter. We would like to compare the quality of life (QOL) and long-term defecative function between these two groups of patients 10 years after reconstructive surgery. METHODS: Patients who underwent LAARP between 2001 and 2005 were compared with historical controls treated with PSARP between 1996 and 2000. Degrees of continence were graded by the Krickenbeck classification and Kelly's score. QOL was assessed by Hirschsprung's disease/Anorectal Malformation Quality of Life (HAQL) questionnaire. Results were compared using Chi-square test and t test. RESULTS: There were 14 LAARP and 7 PSARP patients. All attained voluntary bowel movements. Moderate to severe soiling (Krickenbeck Grade 2 and 3) was found in 3/14 LAARP (21.4%) and 1/7 PSARP (14.3%) patients, p = 1.00. Constipation requiring use of laxatives was present in 3/14 LAARP (28.6%) and 1/7 PSARP (14.3%) patients, p = 0.62. Mean Kelly's scores were 3.79 ± 0.98 (LAARP) and 4.71 ± 1.25 (PSARP), p = 0.12. No patient required Malone antegrade continence enema (MACE). The QOL scores based on the HAQL questionnaire were comparable between the two groups in all areas except social functioning, in which the LAARP patients attained a significantly lower mean score (26.4 vs 71.7, p = 0.0001). CONCLUSION: The 10-year outcome between LAARP and PSARP patients in terms of QOL and defecative function is comparable. Impairment in social functioning in these patients is reflected by the self-reported lower level of functioning.

Common urological problems in children: primary nocturnal enuresis.

Enuresis is a common complaint in children, with a prevalence of around 15% at age 6 years. Evidence suggests that enuresis could affect neuropsychiatric development. The condition may represent an entire spectrum of underlying urological conditions. It is important to understand the difference between monosymptomatic and non-monosymptomatic enuresis. Primary monosymptomatic enuresis can be managed efficaciously with care in different settings, like primary care, specialist nursing, or paediatric specialists, while non-monosymptomatic enuresis requires more complex evaluation and treatment. The diagnosis, investigation, and management of the two types of enuresis are discussed in this review.

Application of indocyanine green in pediatric surgery.

The use of near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) has gained popularity in many fields in adult surgery, such as sentinel lymph node mapping, intra-operative solid tumor identification, and organ perfusion assessment. However, the clinical application of ICG in pediatric surgery is just at the beginning. This review paper presents the advantages, current applications and potential developments of NIR fluorescence imaging with ICG in our field.

Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program.

BACKGROUND: Congenital pulmonary airway malformation (CPAM) is an increasingly recognized disease with potential mortality. Owing to limited published studies, the true incidence is yet to be determined. We carried out this prospective study with the aim to estimate its true incidence on a population basis. METHODS: An antenatal ultrasonography program was implemented since 2009. Fetuses with suspected intra-thoracic lesions were monitored by regular follow-ups. Antenatal course, postnatal outcomes, and other demographics were compared to those of patients with CPAM in the previous decades (1989-2008). The incidence of CPAM was calculated in different periods. RESULTS: 66 CPAM patients were identified between 2009 and 2014 with 62 patients being detected by antenatal scan. In contrast, 45 patients were identified between 1989 and 2008 with 27 patients being detected antenatally. The incidence rate during the past and recent period was estimated as ~1 in 27,400 and ~1 in 7200 live births, respectively (p = 0.024). CONCLUSION: With increasing awareness of clinicians and the universal use of latest ultrasound technology, it is likely that more CPAM cases will be detected in the future. Here, we presented our best estimated incidence rate of CPAM, yet only a larger scale study can reveal its true incidence.

Galactocele in a Male Infant with Transient Hyperprolactinaemia: An Extremely Rare Cause of Breast Enlargement in Children.

Galactocele is a rare breast condition in infants. Here, we report a 16-month-old boy who developed progressive left breast enlargement. Ultrasonography and magnetic resonance imaging revealed a 4 cm cystic lesion at left breast. Hormonal assay showed transient hyperprolactinaemia with no known cause identified. Subsequently, galactocele was confirmed on histopathological examination after complete surgical excision. No recurrence was observed on regular follow-up.

Silver containing hydrofiber dressing promotes wound healing in paediatric patients with partial thickness burns.

INTRODUCTION: Burn injury is one of the most common reasons for admission in paediatric population. There is currently no international consensus on the best wound dressing material. Aquacel Ag, a new silver containing hydrofiber dressing material has been reported to produce good clinical results. Yet, only a limited number of studies exist in the paediatric population. This study aims to review our experience of burn management over the past 5 years and to evaluate the effectiveness of Aquacel Ag in the management of partial thickness burns. METHODS: A retrospective review of all patients admitted for burn injury between January 2010 and December 2014 was conducted. Patients' demographics, mechanism of injury, body surface areas involved, treatment applied, and clinical outcomes were analyzed. Patients with superficial injury, full thickness burns that required surgical debridement, burn area less than 2 % or more than 25 % of total body surface area, or incomplete clinical data were excluded from the comparative study. RESULTS: A total of 119 patients were identified. 114 (96 %) was due to domestic injury, of which 108 (91 %) was food-related. The most commonly affected areas were limbs (n = 89, 74.8 %), followed by trunk (n = 62, 74.8). 84 patients fulfilled the inclusion criteria and were recruited into the study. 31 patients received Aquacel Ag dressing and 53 patients received standard paraffin gauze dressing. The two groups showed no statistical difference in age, sex, percentage of total body surface area involved, and infection rate. Outcomes of patients treated with Aquacel Ag were compared with patients treated with standard dressing. The mean hospital stay was significantly shorter for the Aquacel Ag group (14.26 vs 23.45, p = 0.045). Aquacel Ag group required much less frequent dressing change (5.67 vs 20.59, p = 0.002). 5 patients in standard dressing group developed hypertrophic scar and required prolonged pressure garment, whereas only one hypertrophic scar was observed in the Aquacel Ag group. CONCLUSION: Aquacel Ag appears to promote early burn wound healing with less hypertrophic scar formation.

The feasibility and efficacy of multi-channel intraluminal impedance monitoring in children.

INTRODUCTION: The diagnosis of gastro-oesophageal reflux is currently based on clinical presentation and oesophageal pH monitoring. In recent years, the use of multi-channel intraluminal impedance (MII) monitoring has gained increasing attention in the adult population. However, its use in the paediatric population is still in the developing stage with only limited number of publications. This study aims to review our early experience of MII application in children. METHODS: A retrospective study of all patients who underwent MII monitoring between 2011 and 2014 at a tertiary referral centre was performed. Patients' medical records were reviewed with demographic data extracted. Number of reflux episodes and other MII parameters were analysed. RESULTS: In total, 34 patients were identified during the study period, with 20 males and 14 females. Indication for study included previous aspiration pneumonia (n = 13), persistent reflux or vomiting symptom (n = 10) and as part of routine assessment before gastrostomy (n = 11). At the time of study the average age was 69 months (range 9-216 months). 28 patients showed significant gastro-oesophageal reflux. On average patient has 36.1 acidic and 22.3 non-acidic reflux episodes during the 24 h monitoring period. Non-acidic reflux accounts for 38.1 % of the overall reflux episodes. The sensitivity of MII monitoring to detect reflux was higher compared to conventional pH study (73 vs 50 %, p = 0.1). CONCLUSION: MII monitoring is safe and feasible in children. Non-acid reflux should not be underestimated in paediatric population. MII appears to be more sensitive than conventional pH monitoring in our study, but its true significance is yet to be confirmed by larger study in the future.

Acute appendicitis presenting as pneumoperitoneum in a teenage boy undergoing chemotherapy.

Chemotherapy of paediatric haematological malignancies can induce infectious complications of the gastrointestinal tract, with predilection of the ileocaecal region. Common causes of right lower abdominal pain in the febrile patient with neutropaenia include acute appendicitis, typhlitis, ileus and intussusception. In this case report, we describe a teenage boy with acute appendicitis presenting with pneumoperitoneum during his course of chemotherapy. The incidence, aetiology, diagnosis, investigations and management of this uncommon presentation in a common disease are discussed. The controversial topic of the management of acute appendicitis in a febrile patient with neutropaenia is also reviewed and discussed.

Timely thoracoscopic decortication promotes the recovery of paediatric parapneumonic empyema.

INTRODUCTION: Parapneumonic empyema is one of the most commonly encountered yet difficult to manage paediatric thoracic conditions. Conservative treatment with chest tube drainage and fibrinolytic agents had been proposed but operative decortication remains the gold standard for refractory cases. Thoracoscopic decortication has been advocated in recent years due to its superiority in terms of post-operative pain, cosmesis and other long-term results. However, few studies investigated the effect of timing on peri-operative outcomes. This study aims to explore the benefits of early decortication. METHODS: Retrospective study of all patients who underwent thoracoscopic decortication between 1999 and 2013 at a tertiary referral centre was performed. Data were extracted from respective medical records. Patients' demographics, peri-operative outcomes, length of hospitalization and post-operative complications were analysed. RESULTS: A total of 28 patients were identified, 12 males and 16 females. Average age of patients was 4.5 years (range 12 months-14 years). Right-sided empyema was involved in 14 of the patients. Patients who underwent operation within 2 weeks from symptom onset (n = 16) showed significant shorter post-operative hospital stay (mean 9.5 vs 20.4 days, p = 0.003) and total hospitalization duration (mean 19.3 vs 38.8 days, p < 0.001). Correlation study demonstrated a strong relation between delay in operation and prolonged hospitalization (r = 0.63, p = 0.001). The peri-operative and post-operative outcomes were similar. No major post-operative complication was encountered except one patient who required a second decortication for residual empyema. CONCLUSION: Thoracoscopic decortication is a safe and feasible procedure for parapneumonic empyema. Timely surgery is recommended as it promotes early recovery and shorter hospitalization.

High-performance multi-megahertz optical coherence tomography based on amplified optical time-stretch.

As the key prerequisite of high-speed volumetric structural and functional tissue imaging in real-time, scaling the A-scan rate beyond MHz has been one of the major pursuits in the development of optical coherence tomography (OCT). Along with a handful of techniques enabling multi-MHz, amplified optical time-stretch OCT (AOT-OCT) has recently been demonstrated as a viable alternative for ultrafast swept-source OCT well above MHz without the need for the mechanical wavelength-tuning mechanism. In this paper, we report a new generation of AOT-OCT demonstrating superior performance to its older generation and all other time-stretch-based OCT modalities in terms of shot-to-shot stability, sensitivity (~90dB), roll-off performance (>4 mm/dB) and A-scan rate (11.5 MHz). Such performance is mainly attributed to the combined contribution from the stable operation of the broadband and compact mode-locked fiber laser as well as the optical amplification in-line with the time-stretch process. The system allows us, for the first time, to deliver volumetric time-stretch-based OCT of biological tissues with the single-shot A-scan rate beyond 10 MHz. Comparing with the existing high-speed OCT systems, the inertia-free AOT-OCT shows promises to realize high-performance 3D OCT imaging at video rate.

Pneumatosis intestinalis presenting as pneumoperitoneum in a teenage girl with pyloric stenosis.

A 16-year-old girl presented with free gas under the diaphragm after endoscopic balloon dilation for pyloric stenosis. There was no perforation site identified on laparotomy. However, there was massive pneumatosis intestinalis involving the entire small bowel.

Prokineticin signaling is required for the maintenance of a de novo population of c-KIT⁺ cells to sustain neuroblastoma progression.

High cellular heterogeneity within neuroblastomas (NBs) may account for the non-uniform response to treatment. c-KIT(+) cells are frequently detected in NB, but how they influence NB behavior still remains elusive. Here, we used NB tumor-initiating cells to reconstitute NB development and demonstrated that c-KIT(+) cells are de novo generated and dynamically maintained within the tumors to sustain tumor progression. c-KIT(+) NB cells express higher levels of neural crest and stem cell markers (SLUG, SOX2 and NANOG) and are endowed with high clonogenic capacity, differentiation plasticity and are refractory to drugs. With serial transplantation assays, we found that c-KIT expression is not required for tumor formation, but c-KIT(+) cells are more aggressive and can induce tumors ninefold more efficiently than c-KIT(-/low) cells. Intriguingly, c-KIT(+) cells exhibited a long-term in vivo self-renewal capacity to sustain the formation of secondary and tertiary tumors in mice. In addition, we showed that Prokineticin signaling and mitogen-activated protein kinase pathways are crucial for the maintenance of c-KIT(+) cells in tumor to promote NB progression. Our results highlight the importance of this de novo population of NB cells in sustainable growth of NB and reveal specific signaling pathways that may provide targets leading to more effective NB therapies.

The value of the 24-h delayed abdominal radiograph of barium enema in the diagnosis of Hirschsprung's disease.

AIM OF STUDY: The objective of this study is to determine the diagnostic value of the 24-h delayed film for Hirschsprung's disease (HD). Other features of the barium enema were also examined, in particular the correlation between the radiological transition zone (TZ) and the final pathology. METHODS: All patients with suspected HD from 2003 to 2013 who had undergone barium enema and rectal biopsy were reviewed retrospectively to study the correlation between radiological features of barium enema with the final diagnosis as well as severity. RESULTS: A total of 182 patients were admitted for suspected HD during the study period, of which 82 had both investigations done. 68 patients had radiological features suggestive of the disease and ultimately, 12 patients had the disease confirmed with rectal biopsy. Among those without radiological features of HD, 2 patients were found to have the disease. Thus, the sensitivity of the 24-h delayed film was 85.7 % and the specificity was 17.6 %. The positive predictive value (PPV) of this test was 20.6 % and the negative predictive value (NPV) was 85.7 %. Regarding the level of TZ, it was not detected in the barium enema in 7 (50 %) out of the 14 patients. For those with the presence of TZ, 6 (85.7 %) of them correlated well with the intra-operative findings and 4 (57.1 %) of them correlated well with the final histology. CONCLUSION: The 24-h delayed film of barium enema has a high NPV and is useful to rule out HD. However, rectal biopsy is still suggested for disease confirmation given its low PPV. Lastly, once present, the level of radiological TZ is also a useful predictor for the actual disease involvement.

Performance of megahertz amplified optical time-stretch optical coherence tomography (AOT-OCT).

Enabled by the ultrahigh-speed all-optical wavelength-swept mechanism and broadband optical amplification, amplified optical time-stretch optical coherence tomography (AOT-OCT) has recently been demonstrated as a practical alternative to achieve ultrafast A-scan rate of multi-MHz in OCT. With the aim of identifying the optimal scenarios for MHz operation in AOT-OCT, we here present a theoretical framework to evaluate its performance metric. In particular, the analysis discusses the unique features of AOT-OCT, such as its superior coherence length, and the relationship between the optical gain and the A-scan rate. More importantly, we evaluate the sensitivity of AOT-OCT in the MHz regime under the influence of the amplifier noise. Notably, the model shows that AOT-OCT is particularly promising when operated at the A-scan rate well beyond multi-MHz--not trivially achievable by any existing swept-source OCT platform. A sensitivity beyond 90 dB, close to the shot-noise limit, can be maintained in the range of 2 - 10 MHz with an optical net gain of ~10 dB. Experimental measurement also shows excellent agreement with the theoretical prediction. While distributed fiber Raman amplification is mainly considered in this paper, the theoretical model is generally applicable to any type of amplification schemes. As a result, our analysis serves as a useful tool for further optimization of AOT-OCT system--as a practical alternative to enable MHz OCT operation.

Megahertz all-optical swept-source optical coherence tomography based on broadband amplified optical time-stretch.

We demonstrate all-optical ultrahigh-speed swept-source optical coherence tomography (OCT) based on amplified optical time-stretch (AOT). Such an inertia-free wavelength-swept mechanism, via group velocity dispersion, enables us to realize OCT with an A-scan rate well above MHz. More importantly, the key significance of AOT-OCT is its simultaneous broadband Raman amplification during the time-stretch process-greatly enhancing the detection sensitivity compared with prior attempts to apply optical time-stretch to OCT. Here, we report on an AOT-OCT system which is operated at an A-scan rate of 7.14 MHz, a superior roll-off performance (>2 mm/dB), a record-high sensitivity of time-stretch-based OCT (>80 dB) with a broadband gain bandwidth of 80 nm, which results in an axial resolution of ∼15 µm. Our AOT-OCT system is thus able to, for the first time to the best of our knowledge, perform time-stretch-based OCT of biological tissue in vivo. It represents a major step forward in utilizing AOT as an alternative for achieving practical MHz OCT, without any long-term mechanical stability concerns as in typical swept-source OCT or bypassing the speed limitation of the image sensor employed in spectral-domain OCT.

Thoracoscopic resection of congenital cystic lung lesions is associated with better post-operative outcomes.

INTRODUCTION: The incidence of congenital cystic lung lesions has been increasing in recent years due to better antenatal detection. With the introduction and maturation of thoracoscopy, the operative management for these lesions has seen advancement in the last decade. In this study, we aimed to compare the post-operative outcomes of patients who had thoracoscopic resection with those who underwent open resection. METHODS: A retrospective review of all patients who underwent surgery for congenital cystic lung lesions between January 1996 and June 2012 in a tertiary referral center was conducted. Patients' demographics, operative procedures and post-operative outcomes were analyzed. RESULTS: Sixty-seven patients were identified over the past 15 years. Thirty-nine patients had thoracoscopic resections and 28 had open resections. Thirteen patients in the thoracoscopic group required conversion. Both groups had similar demographics in terms of age, body weight and laterality of lesions. The mean operative time and blood loss in the two groups were comparable. Patients in the thoracoscopic group had significantly shorter duration of chest tube drainage (4.3 vs. 6.9 days, p = 0.004), shorter intensive care unit stay (2.5 vs. 5.9 days, p = 0.003) and shorter hospital stay (6.9 vs. 12.0 days, p < 0.001). Post-operative complication rate was similar between the two groups. Patients with body weight less than 5 kg showed a significantly higher conversion to open surgery as compared to those with body weight more than 5 kg (62.5 vs. 25.8 %, p = 0.049). CONCLUSION: Successful thoracoscopic resection for congenital cystic lung lesions results in better post-operative outcomes. However, this technique remains technically challenging in patients with body weight less than 5 kg.