What Makes A "Successful" Kasai Portoenterostomy "Unsuccessful"?

OBJECTIVES: Clearance of jaundice (CoJ) is the first key objective of Kasai portoenterostomy (KPE) for biliary atresia (BA) and its achievement is by far the best index of long-term prognosis. We sought to identify the reasons for failure [subsequent liver transplant (LT)] in this cohort. METHODS: Review of single-center prospective BA database. Successful KPE was defined by achieving a postoperative bilirubin of ≤20 µmol/L. Pre-KPE and post-KPE variables were identified together with a multivariate logistic regression model to identify those observable at 3 months post-KPE. Data are quoted as median (range). A P value of ≤0.05 was significant. RESULTS: One hundred thirty-five infants underwent KPE between January 2012 and December 2018, of which 90 (67%) achieved CoJ. From these 20 (22%) (Cohort A) underwent LT with the remainder continuing with native liver (Cohort B) (median follow-up of 4.15 years). There was no difference in age at KPE ( P = 0.41), APRi (aspartate aminotransferase-to-platelet ratio) ( P = 0.07), associated anomalies ( P = 0.7), and cytomegalovirus status ( P = 0.7) between the 2 groups. Postoperatively, both cholangitis [any episode, 18/20 (90%) vs 15/70 (21%); P < 0.0001] and portal hypertension (PHT) [gastrointestinal (GI) bleed, 10/20 (50%) vs 2/70 (2.8%); P < 0.0001] were significantly more common in cohort A. Univariate analysis showed that the most significant predictive values at 3 months for LT by 2 years were high APRi, bilirubin, international normalized ratio, and ultrasound (US)-detected ascites with multivariate logistic modeling confirming these variables with predictive values of r2 = 0.79, AUROC = 0.98. CONCLUSIONS: Failure is not preordained at KPE but due to recurrent cholangitis and/or symptoms of PHT.

Predictors of long-term respiratory insufficiency of exomphalos major.

INTRODUCTION: Exomphalos major (EM) is associated with significant morbidity and even mortality with an important risk of long-term pulmonary disease. AIM: To assess the outcomes of exomphalos in a single tertiary pediatric unit and to identify prognostic factors for patients with respiratory insufficiency who still require ventilatory assistance at six months. MATERIAL AND METHODS: All infants admitted to our institution over a 10-year period (2005 to 2015) with exomphalos were retrospectively reviewed. EM was defined when the abdominal wall defect measured >= 5 cm and/or contained liver within the sac. Data were collected on patient demographics, prenatal course and imaging, birth information, immediate and long-term outcomes. Those with long-term respiratory insufficiency were identified as the primary outcome and reviewed to assess prognostic factors. A p value of ≤0.05 was regarded as significant. Data are quoted as median(range). RESULTS: A total of 46 infants were diagnosed with exomphalos during the study period, with most (n = 30, 65%) defined as exomphalos major. Respiratory complications occurred in 16 (35%) with 8 (50%) of these requiring long-term (≥6 months) mechanical ventilation and 5 (31%) required a tracheostomy. On univariate analysis, resuscitation at birth (p = 0.0004), birth weight <3000 g (p = 0.008), use of nitric oxide (p = 0.004), high frequency oscillatory ventilation (HFOV) (p = 0.001), pulmonary hypoplasia (p<0.0001) and pulmonary hypertension (PHTN) (p = 0.02) were significantly associated with respiratory insufficiency. The strongest predictive model for ventilation support at six months was resuscitation at birth in combination with PH (OR = 1.57). Five infants (11%) died at 5(1-122) days. CONCLUSIONS: In patients with EM, the presence of pulmonary hypertension along with resuscitation at birth are the most important prognostic factors for long-term respiratory insufficiency. Acknowledgement of these factors allows for better parental counselling regarding respiratory outcomes.

Medical alert card: a valuable tool in the management of Hirschsprung's-associated enterocolitis from parental perspective.

PURPOSE: Awareness of Hirschsprung's-associated enterocolitis (HAEC) among patient's families and medical staff can lead to prompt recognition of symptoms and earlier implementation of management. We designed an HAEC medical alert card to raise awareness of HAEC among medical staff and carers of children with Hirschsprung's disease (HD). Our aim was to investigate parental opinion on the utility of this tool. METHODS: All patients diagnosed with HD in two institutions over a period of 14 years received an HAEC alert card and were invited to answer a 1-year follow-up structured questionnaire. RESULTS: A total of 123 patients received an HAEC card. The response rate for the follow-up questionnaire was 62% (n = 76). The majority 96% (n = 73) of the responders considered the card useful. A total of 89% (n = 68) of patients or parents stated that they carry the card with them, while 39% (n = 30) of them have used it on 57 occasions. The majority (83%; n = 25) of these declared that, when presented, the card increased awareness among medical staff and on 53% (n = 16) occasions prompted contact with the tertiary centre. CONCLUSION: The HAEC medical card was found useful by most parents of HD patients. This tool increased awareness of HAEC and improved communication between peripheral hospitals and tertiary paediatric institutions. Therefore, we feel the HAEC alert card may be used in institutions with high HD addressability.

Adolescent de novo hydroceles - should they be dealt with by inguinal or scrotal approach?

AIM: The objective of this study was to investigate the optimal approach for the treatment of hydroceles in adolescents. MATERIALS AND METHODS: A retrospective chart review of all adolescents (10-16 years old) diagnosed with a de-novo hydrocele in 2 tertiary care institutions over a 10 year period (2007-2016) was performed comparing the inguinal and trans-scrotal (Jaboulay) approaches. RESULTS: Fifty-three boys with a mean age of 13.4 years (range 10-16 years) were diagnosed with hydrocele. The inguinal approach was used in 31 (59%) patients for treatment of their hydrocele. In 19 (61%) of these cases a patent processus vaginalis (PPV) ligation was performed. In the other 12 (39%) patients the PPV was closed or not found and a further repair of the hydrocele through the same inguinal incision was performed. A transcrotal Jaboulay procedure was performed in 22 (41%) of the patients. There was no difference in the complications rate between inguinal and trans-scrotal approaches (p = 0.71). Age of presentation less than 12 years was associated with the presence of a PPV (p < 0.05). CONCLUSIONS: A trans-scrotal approach should be considered as first-line in adolescents when the history is not suggestive of a communicating hydrocele. Children less than 12 years of age are more likely to have a PPV and an inguinal approach may be more appropriate. TYPE OF STUDY: Retrospective comparative study LEVEL OF EVIDENCE: Level II.

Patients attitude towards surgeons attire in Our Lady of Lourdes Hospital Drogheda.

BACKGROUND: A doctor's competence and professionalism is often judged on the basis of attire. Our Lady of Lourdes (OLOL) is a leading Irish hospital in the implementation of Bare Below the Elbows (BBTE) policy, however surgical attire is not standardised and there is great variability in attire worn on wards. We aimed to evaluate patients attitude towards surgeons attire in OLOL. METHODS: A prospective survey of adult surgical in-patients was conducted from October 2013 to February 2014. A twelve-question questionnaire was used as data collection tool, using a five point Likert scale to assess patients response to each question. Data were collected on patient demographics, patients level of trust and confidence based on different surgical attire, and patients perception of different attire worn by surgical teams. RESULTS: There were 150 completed surveys during the study period with a male to female ratio of 44% to 56% respectively. The mean patient length of in-hospital stay (LOS) was 4.7 days (range 1-22). The most commonly represented age group was 30-40 years (18%), with a comparable spread among all age groups. The majority of patients found the attire worn by surgeons on the ward to be very appropriate (93%). Majority of responders believed scrubs to be the most appropriate attire for surgeons on wards (39%), followed by shirt and tie with white coat (38%) followed by short sleeved shirt and no tie (18%). Shirt and tie with white coat had a positive effect on patients trust in 63% of responders, a negative effect in 10% and no effect in 26%. Scrubs had a positive effect on patients trust in 63%, negative effect in 11% and no effect in 25%. Short sleeved shirt and no tie had a positive effect in 44%, negative effect in 25% and no effect in 30% of patients. CONCLUSION: Patients in OLOL find attire worn by surgeons to be appropriate. Shirt and tie with white coat or scrubs remains the patient's choice attire for surgeons. Shirt and tie with white coat or scrubs has a more positive effect on trust of patients compared to short sleeved shirt and no tie.

Diagnostic laparoscopy in a twelve year old girl with right iliac fossa pain: A life changing diagnosis of complete androgen insensitivity syndrome.

INTRODUCTION: Right iliac fossa (RIF) pain is one of the most common presenting complaints faced by general surgeons in the emergency department. Correct diagnosis and appropriate surgical intervention can often pose a challenge. PRESENTATION OF CASE: A 12-year-old girl presented to the emergency department with a four day history of initially central acute abdominal pain, now localised in the RIF. During laparoscopy, the following findings were made: macroscopically dilated appendix, right and left gonads at the internal opening of the inguinal canal, empty pelvis with a rudimentary uterus on the right side. No evidence of fallopian tubes or connection of uterus to the vagina and broad based, non-inflamed Meckel's diverticulum. An incidental diagnosis of complete androgen insensitivity syndrome was made. DISCUSSION: Androgen insensitivity syndrome (AIS) is a disorder of hormone resistance characterised by a female phenotype in an individual with an XY karyotype and testes producing age-appropriate normal concentrations of androgens. CONCLUSION: This case report highlights the advantage of laparoscopy as a diagnostic and treatment tool in a twelve year old girl with multiple intra-abdominal findings. While the ultimate diagnosis responsible for her symptom of RIF pain was acute appendicitis, the additional diagnosis of CAIS and incidental Meckel's would have otherwise likely gone undetected.