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.

Jaundice in infants and children: causes, diagnosis, and management.

Jaundice is caused by an accumulation of bilirubin in the blood. The presentation in infants and children can be indicative of a wide range of conditions, with some self-limiting and others potentially life-threatening. This article aims to provide a concise review of the common medical and surgical causes in children and discuss their diagnosis and management.

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.

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.

Laparoscopic inguinal hernia repair in premature neonates: is it safe?

OBJECTIVE: With advances in clinical medicine, many premature babies nowadays can have excellent survival outcomes. As the incidence of inguinal hernias in this group is high and there is scarce data in the literature regarding the optimal timing for repair, this study aims to review our experience in laparoscopic repair in premature infants. METHODS: In our centre, premature neonates with inguinal hernia noted during hospitalization were offered laparoscopic repair when the body weights reached 2.5 kg unless there is contraindication for laparoscopy. A retrospective review was carried out for all premature neonates who underwent laparoscopic inguinal hernia repair from 2001 to 2011. The operative results, complications, incarceration risk and postoperative apnea risk were recorded. RESULT: A total of 79 premature neonates received laparoscopic inguinal hernia repair during this period. The mean gestational age at birth was 31.9 weeks (27-36 weeks) and the mean gestational age at operation was 46.5 weeks (33-92 weeks). One patient had incarceration and required emergency operation while waiting for the elective repair. The mean operative time was 44.9 min (25-93 min). One patient (1.3 %) had recurrence. No postoperative apnea was noted in any patient. CONCLUSION: Laparoscopic hernia repair is safe and feasible in premature neonates when they attain reasonable body size, as long as there is excellent anaesthesia support. Low risk of incarceration was noted in this study and it is worth waiting for the body weight to build up and hence facilitate laparoscopic repair.

Performing Kasai portoenterostomy beyond 60 days of life is not necessarily associated with a worse outcome.

The introduction of Kasai portoenterostomy has dramatically improved the management and survival of children with biliary atresia. The success rate of this operation worldwide varies with different centers. In this respect, many authors have studied the correlation of a successful outcome with various factors, such as the experience and workload of the surgical center, the use of postoperative steroids, the underlying biliary anatomy, as well as the age of patients at the time of the operation. Indeed, the age of 60 days has been used by clinicians as a critical time beyond which the rate of success of the Kasai operation markedly reduces. Despite this worldwide adoption, clear evidence supporting this critical operative time is still lacking. We undertook a review of our experience in the management of children with biliary atresia and focused specifically on the issue of the timing of operation. We showed that performing the Kasai operation beyond the age of 60 days was not associated with a worse outcome and that a high percentage of patients could still achieve good bile flow with normal bilirubin postoperatively. Thus, we believe that until the age of 100 days, the age of the patients does not play a significant role in determining the success of the Kasai operation.

The first report of a single-port laparoscopic nephrectomy in a child.

There has been an exponential rise in the use of minimally invasive procedures in surgery, with obvious benefits to patients. Recently, transumbilical single-port laparoscopic surgery has been championed as the next major technical advance. In this article, we report the first case where single-port laparoscopic surgery has been used to manage a paediatric problem in the region.

Laparoscopic hernioplasties in children: the implication on contralateral groin exploration for unilateral inguinal hernias.

BACKGROUND: Inguinal hernias are commonly seen in the paediatric population. Controversies still exist regarding the need for contralateral groin exploration when an unilateral inguinal hernia is presented, since the true incidence of contralateral patent processus vaginalis is not known. With the advent of laparoscopic hernioplasty, the status of the contralateral side can be evaluated at the same setting. Here, we describe our experience in this issue after the introduction of laparoscopic hernioplasty in our unit. METHODS: A retrospective review was carried out between October 2002 and January 2008. All patients presented with unilateral inguinal hernias were included. The demographics of the patients and the operative findings at laparoscopy were recorded. Statistics were performed using Student t-test or chi(2) test as appropriate and p < 0.05 was taken as statistically significant. RESULTS: During the study period, 363 children were included in our study, of which there were 292 males and 71 females. 212 patients presented with right-sided hernias and 151 were left-sided. The mean age of patients at presentation was 48.8 months. The incidence of contralateral PPV overall was 39.7%. There was no decrease in incidence of having a contralateral inguinal hernia in relation to age. CONCLUSION: Laparoscopy can accurately diagnose contralateral PPV in children who undergo unilateral inguinal hernia repair and thus holds an advantage over open herniotomy. Furthermore, there should not be an age criteria for contralateral exploration for surgeons who perform open herniotomy.

Consumption of viscera as the most important risk factor in the largest outbreak of shellfish poisoning in Hong Kong, 2005.

Poisoning from shellfish toxins is associated with significant morbidity worldwide. During 10-15 March 2005, 36 clusters of shellfish poisoning related to consumption of fresh scallops (Atrina vexillum) were reported to the Center for Health Protection, Department of Health Hong Kong. We conducted a case-control study to identify the risk factors associated with shellfish poisoning. Detailed demographic, clinical, shellfish consumption data of these subjects were collected using standardized questionnaires. Fifty-eight cases and 44 controls were identified. The mean age for the cases was 37.5 years (range 10-81 years); 45% (n=26) were male. Ninety-five percent had onset of symptoms within 12 hours of consumption of scallops (range: 10 minutes to 30 hours, median: 45 minutes). Dizziness (87.9%) and blurred vision (53.4%) were the predominant symptoms. The mean number of pieces of scallop meat and viscera taken by the cases were 3.7 and 3.6, respectively, significantly higher than that for the controls, which were 1.6 (p<0.001) and 0.5 (p<0.001), respectively. Forty-two percent (n=22) and 19% (n=7) of cases and controls, respectively, took soup/sauce from the same dish that was cooked with the scallops (p=0.02). Consumption of scallop viscera was identified as the only significant risk factor (Adjusted OR=9.93, p=0.001) after adjusting for other risk factors. The result show that consumption of scallop viscera is an important risk factor for shellfish poisoning. The public should be warned specifically in health education messages to avoid eating viscera of scallops.

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.

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.

Nonlinear 3D projection printing of concave hydrogel microstructures for long-term multicellular spheroid and embryoid body culture.

Long-term culture and monitoring of individual multicellular spheroids and embryoid bodies (EBs) remains a challenge for in vitro cell propagation. Here, we used a continuous 3D projection printing approach - with an important modification of nonlinear exposure - to generate concave hydrogel microstructures that permit spheroid growth and long-term maintenance, without the need for spheroid transfer. Breast cancer spheroids grown to 10 d in the concave structures showed hypoxic cores and signs of necrosis using immunofluorescent and histochemical staining, key features of the tumor microenvironment in vivo. EBs consisting of induced pluripotent stem cells (iPSCs) grown on the hydrogels demonstrated narrow size distribution and undifferentiated markers at 3 d, followed by signs of differentiation by the presence of cavities and staining of the three germ layers at 10 d. These findings demonstrate a new method for long-term (e.g. beyond spheroid formation at day 2, and with media exchange) 3D cell culture that should be able to assist in cancer spheroid studies as well as embryogenesis and patient-derived disease modeling with iPSC EBs.

Selective vertical transmission of HIV-1 antiretroviral resistance mutations.

OBJECTIVE: To examine the patterns of vertical transmission of zidovudine (ZDV) resistance mutations. DESIGN: HIV-1 reverse transcriptase codons 10-250 were sequenced from 24 pairs of ZDV-exposed women and their HIV-infected infants as part of the Women and Infants Transmission Study. METHODS: Viral RNA was extracted from tissue culture supernatants and sequenced using fluorescent dye-primer chemistry and an automated sequencer. RESULTS: For 17 of these pairs, maternal and infant sequences were identical to one another and lacking known ZDV resistance mutations. The remaining seven maternal sequences contained known mutations associated with ZDV resistance at reverse transcriptase codons 70, 210, 215 and 219. In each case where the maternal HIV isolate showed a pure mutant species, the infant sequence was identical. When the maternal sequence showed the presence of a sequence mixture at codon 70 or 219, the infant's virus showed only wild-type sequence even when the ZDV-resistant mutant was quantitatively dominant in the mother. The single maternal HIV isolate showing mixed sequence at codon positions 210 and 215 transmitted an unmixed mutant to the infant at both positions. When maternal mixtures were present at sites not associated with ZDV resistance, only the dominant species appeared in the infant. CONCLUSIONS: When maternal HIV isolates contained mixed wild-type and ZDV-resistant subpopulations, only a single component of the mixture could be detected in the infected infants. Resistance mutants without the codon 215 mutation were not transmitted from mixtures, even when the mutants formed the majority of circulating maternal virus. In perinatal HIV transmission, specific ZDV-resistant HIV genotypes circulating in the maternal virus pool may influence whether infection in the infant will be established by a wild-type or ZDV-resistant HIV strain.

HIV-1 genotypic zidovudine drug resistance and the risk of maternal--infant transmission in the women and infants transmission study. The Women and Infants Transmission Study Group.

OBJECTIVES: Although the treatment of pregnant women and their infants with zidovudine (ZDV) has been remarkably effective in preventing the perinatal transmission of human HIV-1, many potentially preventable infections still occur. To examine whether the risk of perinatal infection is increased among women who carry ZDV-resistant HIV-1, the role of genotypic ZDV resistance in perinatal transmission was evaluated. METHODS: The reverse transcriptase (RT) region of clinical isolates from culture supernatants of 142 HIV-1-infected women enrolled in the Women and Infants Transmission Study (WITS), who had been treated with ZDV during pregnancy was sequenced. Results from genotypic sequencing were linked to demographic, laboratory, and obstetrical databases, and the magnitude of association of having consensus drug-resistant HIV-1 RT mutations with transmission was estimated. RESULTS: Twenty-five per cent (34/142) of maternal isolates had at least one ZDV-associated resistance mutation. A lower CD4 cell percentage and count (P= 0.0001) and higher plasma HIV-1 RNA (P=0.006) were associated with having any ZDV resistance mutation at delivery. Having any RT resistance mutation [odds ratio (OR): 5.16; 95% confidence interval (CI): 1.40, 18.97; P=0 0.01], duration of ruptured membranes [OR: 1.13 (1.02, 1.26) per 4 h duration; P= 0.02], and total lymphocyte count [OR: 1.06 (1.01, 1.10) per 50 cells higher level; P=0.009] were independently associated with transmission in multivariate analysis. CONCLUSION: Maternal ZDV resistant virus was predictive of transmission, independent of viral load, in these mothers with moderately advanced HIV-1 disease, many of whom had been treated with ZDV before pregnancy.

Reconstruction of human hard-palate mucosal epithelium on de-epidermized dermis.

Artificial hard-palate mucosa equivalents were reconstructed using keratinocytes derived from normal human hard-palate and de-epidermized dermis. Reconstructed hard-palate mucosal epithelium formed in three-dimensional culture was compared to native hard-palate mucosal epithelium and reconstructed oral buccal mucosal epithelium with regard to keratin expression. Artificial hard-palate mucosal epithelium reconstructed in medium with delipidized serum showed a differentiation pattern similar to that of hard-palate epithelium in vivo. The present study also confirmed that keratinocytes derived from hard-palate mucosa are intrinsically different from those of nonkeratinizing oral surfaces.

Electroejaculation and assisted reproductive techniques for anejaculatory infertility.

OBJECTIVE: To report our experience with electroejaculation combined with intrauterine inseminations (IUI) and other assisted reproductive technologies. METHODS: Anejaculatory men desirous of fertility were evaluated in our Assisted Reproductive Program. Between March 1991 and December 1994, 26 men (age 24-48) underwent a total of 84 electroejaculations. Causes of anejaculation included spinal cord injury (n = 23) and retroperitoneal lymph node dissection (n = 3). Female partners were super-ovulated to improve the pregnancy rate. The success in sperm retrieval and pregnancy rates were determined, and the quality of electroejaculates was evaluated. RESULTS: Seventy-seven of 84 (92%) electroejaculations were successful, defined by retrieval of more than 10 x 10(6) total and more than 10(6) motile spermatozoa. Mean sperm count was 65 million/mL (range 0-569), but mean motility was only 16% (range 0-66). Mean normal morphology was 27% (range 0-71). Ten couples attempted conception. Fifty cycles of IUIs were performed, resulting in four normal term infants and one spontaneous abortion (pregnancy rate 10% per IUI). One patient failed to conceive with eight cycles of IUIs but became pregnant with in vitro fertilization-embryo transfer with micromanipulation using electroejaculates; she delivered a set of healthy twins. Two couples elected donor sperm insemination after failing to conceive by IUI with electroejaculates; both became pregnant. CONCLUSION: Electroejaculation offers an encouraging pregnancy opportunity for anejaculatory men who otherwise are considered infertile. Marked asthenospermia is observed in electroejaculates, the etiology of which remains obscure. Further studies to elucidate the cause may improve pregnancy rates.

Correlation between semen parameters of electroejaculates and achieving pregnancy by intrauterine insemination.

OBJECTIVE: To investigate whether any parameter in the routine semen analysis of electroejaculates is correlated with success in achieving pregnancy by IUI. DESIGN: Retrospective observational study. SETTING: An Assisted Reproductive Program at a tertiary care university center. PATIENT(S): Twenty-seven anejaculatory men with spinal cord injury (n = 24) or history of retroperitoneal lymph node dissection (n = 3), thirteen of whom attempted conception with their wives. INTERVENTION(S): Anejaculatory men underwent rectal probe electroejaculation and electroejaculates were used for IUI. MAIN OUTCOME MEASURE(S): Statistical correlation of semen parameters between electroejaculates that resulted in pregnancy and those that did not. RESULT(S): Seven pregnancies resulted from 56 IUIs using electroejaculates (pregnancy rate = 12.5% per IUI). The total motile sperm count and percentage of normal morphology were significantly higher in the specimens that resulted in pregnancies than those that did not. However, there was no statistically significant difference observed in pH, sperm concentration, or percentage of motility between the two groups. Swim-up techniques used to process electroejaculates significantly improved the motility of the specimens. No pregnancy occurred beyond the fifth IUI attempt. Repeated electroejaculation and duration of spinal cord injury had no effect on the quality of the ejaculates. CONCLUSION(S): The total motile sperm count and the percentage normal morphology of electroejaculates correlate with success in achieving pregnancy by IUI. Because repeated electroejaculation does not improve quality of ejaculate, the initial semen analysis of electroejaculates is not only useful in counseling couples undergoing such treatment program but should be planned for use as an inseminate.