Regional cluster of vanishing gastroschisis: A comparative study of antenatal and post-natal outcomes.

AIM: Vanishing gastroschisis describes the in utero spontaneous closure of the periumbilical defect. It is usually associated with intestinal loss due to ischaemia, necrosis and atresia. This comparative study aims to investigate the spectrum of pathology, antenatal ultrasound characteristics and post-natal outcomes. METHODS: Our tertiary centre provides antenatal and post-natal care of major congenital anomalies for a population of 1.6 million. Medical records were retrospectively evaluated for all cases of vanishing gastroschisis from May 2014 to May 2015. Cases of normal variant gastroschisis born during the same period were used for comparison. Maximum antenatael bowel diameter measurements were compared using the Mann-Whitney U-test. RESULTS: Six infants with vanishing gastroschisis were born during the study period, representing 50% of all live-born gastroschisis. Antenatal ultrasound showed progressively increasing intra-abdominal bowel dilatation, with antenatal intra-abdominal bowel diameter significantly greater in vanishing, than normal, variant gastroschisis (23.2 vs. 4.1 mm, P < 0.01). The classification of vanishing gastroschisis severity comprised two type I, three type II and one type III cases. Complete midgut atresia affected three infants, leading to overall mortality of 50% for the vanishing gastroschisis group versus 0% in the normal variant group (P = 0.05). CONCLUSION: Vanishing gastroschisis is a severe, often catastrophic variant of gastroschisis. Aetiological factors contributing to the recent high incidence of this rare complication in our population of newborns remain unknown, prompting secondary prevention strategies to salvage the midgut. We propose closer antenatal surveillance for fetuses with intra-abdominal bowel dilatation >10 mm to prompt consideration of earlier delivery to improve morbidity and mortality.

Ultrasound guided hydrostatic enema reduction of ileocolic intussusception: a safe and effective technique.

BACKGROUND: Currently, the primary management of ileocolic intussusception in children is usually by non-operative image-guided enema reduction. In most centres around the world especially in Australasia the predominant technique is the pneumatic reduction under fluoroscopic guidance. At our institution, we have been performing ultrasound-guided hydrostatic reduction since 2012.This is an audit to determine the efficacy and safety of ultrasound-guided hydrostatic reduction for intussusception. METHODS: Following ethics approval, a retrospective review of all patients presenting to our institution with intussusception and subsequently undergoing hydrostatic reduction over a period of 9 years (2012 to-2020) was performed. The parameters studied included (i) successful reduction, (ii) recurrence, (iii) need for surgery and (iv) lead point at surgery. RESULTS: The mean age at presentation was 12 months. One hundred and eight children were diagnosed to have ileocolic intussusception. One hundred and six underwent ultrasound-guided hydrostatic reduction with successful reduction in 96 (90.5%) patients. Reduction was unsuccessful in 10 patients (9.5%). Of these eight were noted to have a pathological lead point (four-Meckel's diverticulum and four-Lymphoma) at the time of the surgery. The intussusception recurred in six patients (6.25%) within 24 h. No reduction related perforation occurred during the study period. CONCLUSION: Ultrasound-guided hydrostatic reduction is a safe and effective technique for managing intussusception as it allows continuous monitoring of the reduction of the intussusception without exposing the children to ionizing radiation.

Towards a complete cycle of care: a multidisciplinary pathway to improve outcomes in complex abdominal wall hernia repair.

INTRODUCTION: The burden of complex abdominal wall hernia (CAWH) is increasing, with associated high morbidity and healthcare costs. This study evaluates current evidenoptce regarding multidisciplinary care for CAWH patients to improve patient outcomes. METHODS: A systematic review of Scopus, MEDLINE, Embase, PubMed, Web of Knowledge and Cochrane Library was conducted to identify proposed or established multidisciplinary team (MDT) pathways, necessary MDT constituents, and to evaluate patient outcomes. The pre-optimization pathways were then compared with a recent Delphi consensus statement. RESULTS: Seven articles matched the relevant search criteria. Three were concept articles, without prospective data analysis. Four were case series that applied multidisciplinary care and included limited data analyses with outcomes reported up to 50 months. The consensus was that CAWH MDT requires multiple clinical specialties, including hernia, upper gastrointestinal, colorectal and/or plastic and reconstructive surgeons, along with allied health specialists, radiologists, anaesthetists/pain specialists and infectious diseases consultants. A successful MDT should aim to achieve pre-optimization and plan the definitive repair. These pre-optimization pathways were similar to the recent Delphi consensus by international hernia experts. Using these data, we propose a CAWH multidisciplinary pathway model in an Australian tertiary hospital involving a stepwise approach with well-defined referral criteria, perioperative high-risk management with pre-optimization, surgical planning, postoperative care and follow-up protocols. This pathway incorporates prospective data collection in a Clinical Quality Registry (CQR) to validate its appropriateness. CONCLUSIONS: CAWH MDT can provide comprehensive, patient-centred care with improved postoperative outcomes. CQR are important to better evaluate long-term outcomes and ensure rigorous quality control.

Testicular Appendage Torsion-To Explore the Other Side or Not?

OBJECTIVE: To investigate and compare the risks vs benefits of synchronous contralateral scrotal compartment exploration when testicular appendage torsion is diagnosed intra-operatively. METHODS: Emergency scrotal explorations performed at the Women's and Children's Hospital between 2002 and 2017 were retrospectively analysed to identify patients with testicular appendage torsion. Primary outcome measures were metachronous acute scrotum re-presentations and returns to theatre. Outcomes were compared between groups that underwent unilateral and bilateral scrotal compartment exploration. RESULTS: Testicular appendage torsion was diagnosed intra-operatively in 575 patients and 90.4% underwent unilateral scrotal exploration. Re-presentations with metachronous acute scrotum on the non-index side occurred in 8.5% of unexplored and 3.6% of previously explored sides (P = .29). Non-index side returns to theatre occurred in 5.4% of unexplored and 0% previously explored sides (P = .097). Future metachronous contralateral testicular appendage torsion was diagnosed in 4.2%. Post-operative complications were comparably low in both groups. The number needed to treat to prevent a return to theatre for metachronous contralateral testicular appendage torsion is 24. CONCLUSION: The low morbidity of exploring the contralateral side is justifiable, but confers only limited benefit of preventing low likelihood future metachronous contralateral pathology. In balancing these risks, we recommend contralateral exploration as advisable but not a necessity.

Acquired factor VII deficiency associated with Wilms tumor.

We present the case of a 2-year-old female with Wilms tumor whose initial evaluation revealed a prolonged prothrombin time (PT) and normal activated partial thromboplastin time. Mixing studies demonstrated correction of the PT and the Factor VII activity was 17% in the absence of a Factor VII inhibitor. She underwent successful resection of the tumor with fresh frozen plasma support and no excessive bleeding. Post-operative testing demonstrated normal PT at 3 days and 1-month. Although acquired von Willebrand factor deficiency has a known association with Wilms tumor, paraneoplastic factor VII deficiency associated with Wilms tumor is previously unreported.

Sutured point-fixation versus Jaboulay fixation for salvaged testicular torsion in children.

BACKGROUND: Surgical techniques for fixation of the testis are varied and subject to ongoing debate. Non-sutured techniques may avoid the theoretical morbidities of sutured fixation of the testis yet are criticized for insufficient prophylaxis against future torsion. This study aims to compare outcomes between sutured (point-fixation) versus Jaboulay fixation. METHODS: Emergency scrotal explorations performed at a tertiary hospital in the state of South Australia between February 2002 and December 2017 were analyzed to identify cases of testicular torsion. Primary outcome measures included future testicular torsions and return to theater episodes following initial testicular fixation. Secondary outcome measures included re-presentations and post-operative complications. RESULTS: A total of 482 scrotal compartments were explored in 244 boys with acute testicular torsion. Testis fixation was performed using sutured point-fixation in 58.4% and Jaboulay tunica plication in 41.6%. No future testicular torsion occurred regardless of fixation technique. There were no significant differences in returns to theater (0.4% versus 1.2%, p = 0.12), re-presentations (6.9% versus 6.0%, p = 0.83), and post-operative complications (1.7% versus 1.8%, p = 1.0) in testes that previously underwent sutured or Jaboulay fixation, respectively. CONCLUSION: Jaboulay testicular fixation techniques are comparable with sutured point-fixation techniques in effectiveness and morbidity. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level III.

Intrauterine Fetal Death With Vanishing Gastroschisis and Post Mortem Examination Findings.

We present a severe case of vanishing gastroschisis resulting in intrauterine death with post mortem examination findings. Gastroschisis is defined as a full thickness paraumbilical abdominal wall defect associated with evisceration of fetal intestine. It is almost always right-sided. Vanishing gastroschisis is an extremely rare form of gastroschisis that results in short bowel syndrome due to exteriorized bowel disconnected from the lumen of the rest of the bowel proximally as well as distally in association with partial or complete closure of the abdominal wall. This case is only the second published case of vanishing gastroschisis resulting in intrauterine fetal death including post mortem examination findings. It highlights the importance of being aware of this rare form of gastroschisis and provides insights regarding pathogenesis, ultrasound surveillance, and antenatal counseling.

Bilateral perinatal testicular torsion: successful salvage supports emergency surgery.

Perinatal testicular torsion (PTT) has poor rates of testicular salvage. Although rare, bilateral PTT carries the risk of anorchia. We present a case of a 2-day-old term infant with acute onset right-sided scrotal discolouration and tenderness. The infant was promptly taken to the operating theatre for emergency scrotal exploration. Bilateral extravaginal testicular torsion was identified, with the right testis appearing to have a more established ischaemic appearance compared to that on the left side. Intraoperative findings were representative of metachronous PTT with a short time period of only several hours separating the torsion events. Both testes were detorted and fixated in the scrotum. The infant made an uneventful recovery. Outpatient clinic review at 6 weeks and 6 months postoperatively confirmed no clinical evidence of testicular atrophy. Given the potential for contralateral torsion and the morbidity of anorchia, our experience supports the role for emergency scrotal exploration in suspected PTT.

Ultrafast energy transfer between the 3MLCT state of [Ru(II)(dmb)2(bpy-an)]2+ and the covalently appended anthracene.

The time scale for triplet-triplet energy transfer (EnT) between a Ru(II) chromophore and a ligand bound anthracene acceptor in [Ru(II)(dmb)2(bpy-an)]2+ (dmb = 4,4'-dimethyl-2,2-bipyridine; bpy-an = 4-(9-anthrylethylene), 4-methyl-2,2-bipyridine) has been measured using femtosecond transient absorption spectroscopy. The appearance of the anthracene excited state is monitored following photoexcitation to a metal-to-ligand charge transfer (MLCT) state via the pi pi* absorption of the triplet excited state of anthracene. Our time-resolved experiments show the presence of fast, sub-100 ps energy transfer to the anthracene occurring on two characteristic time scales of 23 and 72 ps.