Fetal ascites in cloacal malformations-a red flag.

INTRODUCTION: Cloacal malformation is a rare anomaly that remains a diagnostic challenge prenatally, despite the current advances in ultrasonography and MRI. This condition can in some, present with isolated ascites or with other findings, such as a pelvic cyst or upper urinary tract dilatation. In a minority, the ascites may be progressive, questioning the role of antenatal intervention. METHODS: We report on ten patients that have been identified from our Cloaca database between 2010 and 2022. RESULTS: The presence of ascites was associated with extensive bowel adhesions and matting, leading to a challenging initial laparotomy and peri-operative course. CONCLUSIONS: Antenatal finding of ascites in newborns with cloacal malformations should raise a red flag. The surgeon and anaesthetist should be prepared for the operative difficulties secondary to bowel adhesions and the higher risk of haemodynamic instability at the initial surgery. An experienced team at initial laparotomy in such patients is vital. LEVEL OF EVIDENCE: II.

Diagnostic yield and safety of ultrasound-guided percutaneous testicular biopsies in children.

BACKGROUND: Traditionally, testicular biopsy is performed using an open surgical approach. Ultrasound-guided percutaneous biopsy is a less invasive alternative and can be performed in children. OBJECTIVE: The aim of this study is to report our technique and to assess the diagnostic accuracy and safety of ultrasound-guided percutaneous biopsy of testicular masses in children. MATERIALS AND METHODS: This is a 16-year retrospective review of ultrasound-guided percutaneous testicular biopsies at a single pediatric hospital. RESULTS: We performed nine ultrasound-guided testicular biopsies in 9 patients (median age: 3 years, range: 4 months-11 years; median weight: 20.9 kg, range: 8.4-35 kg; median volume of testicular lesion biopsied: 4.4 mL, range: 1.2-17 mL). A percutaneous co-axial technique was used for 5/9 biopsies with absorbable gelatin sponge tract embolization performed in 4 of those biopsies. A non-co-axial technique was used in 4/9 biopsies. A median of three cores, range 2-6, were obtained. The diagnostic yield was 89% with one biopsy yielding material suggestive of, but insufficient for, a definitive diagnosis. The most common histological diagnosis was leukemic infiltration, occurring in 6/9 biopsies. Of the remaining three biopsies, one biopsy was suggestive of, but not confirmatory for, juvenile granulosa cell tumor and two biopsies confirmed normal testicular tissue; the long-term follow-up of which demonstrated normal growth and no lasting damage. There was one (clinically insignificant) complication out of nine biopsies (11%, 95% confidence interval 0-44%): a mild, self-resolving scrotal hematoma. CONCLUSION: Ultrasound-guided testicular biopsy can be performed safely in children as an alternative to open surgical biopsy, with a high diagnostic yield and low complication rate.

Shockwaves and the Rolling Stones: An Overview of Pediatric Stone Disease.

Urinary stone disease is a common problem in adults, with an estimated 10% to 20% lifetime risk of developing a stone and an annual incidence of almost 1%. In contrast, in children, even though the incidence appears to be increasing, urinary tract stones are a rare problem, with an estimated incidence of approximately 5 to 36 per 100,000 children. Consequently, typical complications of rare diseases, such as delayed diagnosis, lack of awareness, and specialist knowledge, as well as difficulties accessing specific treatments also affect children with stone disease. Indeed, because stone disease is such a common problem in adults, frequently, it is adult practitioners who will first be asked to manage affected children. Yet, there are unique aspects to pediatric urolithiasis such that treatment practices common in adults cannot necessarily be transferred to children. Here, we review the epidemiology, etiology, presentation, investigation, and management of pediatric stone disease; we highlight those aspects that separate its management from that in adults and make a case for a specialized, multidisciplinary approach to pediatric stone disease.

Bladder function after conservative surgery and high-dose rate brachytherapy for bladder-prostate rhabdomyosarcoma.

BACKGROUND: Conservative surgery (CS) brachytherapy (BT) techniques for local therapy in bladder-prostate rhabdomyosarcoma (BP-RMS) seek to retain organ function. We report bladder function after high-dose rate (HDR) BT combined with targeted CS for any vesical component of BP-RMS. PROCEDURE: Prospective cohort of all BP-RMS patients between 2014 and 2019 receiving HDR-BT (iridium-192, 27.5 Gy in five fractions) with/without percutaneous endoscopic polypectomy (PEP) or partial cystectomy (PC). Functional assessment included frequency-volume chart, voided volumes, post-void residual, flow studies, continence status and ultrasound scanning; abnormalities triggered video urodynamics. RESULTS: Thirteen patients (10 male), aged 9 months to 4 years (median 23 months), presented with localised fusion-negative embryonal BP-RMS measuring 23-140 mm (median 43 mm) in cranio-caudal extent. After induction chemotherapy, local treatment consisted of PC+BT in three, PEP+BT in four and BT alone in six. At a median 3.5 years (range 21 months to 7 years) follow-up, all were alive without relapse. At a median age of 6 years (4-9 years), the median bladder capacity was 86% (47%-144%) of that expected for age, including 75% (74%-114%) after PC. Radiation dose to the bladder was associated with urinary urgency, but not bladder capacity or nocturnal enuresis. Complications occurred in two: one urethral stricture and one vesical decompensation in a patient with pre-existing high-grade vesico-ureteric reflux (VUR). The remaining patients were dry by day; five with anticholinergic medication for urinary urgency. Three patients are enuretic. CONCLUSIONS: Day-time dryness at a median 3.5 years after CS-HDR-BT was achieved in 92%, with 85% voiding urethrally, and 62% attaining day-and-night continence aged 4-9 years. We report reduced open surgery with minimally invasive percutaneous surgery, with HDR-BT or BT alone being suitable for many.

Single institutional experience of peripheral applications of a liquid embolic agent: Ethylene Vinyl Alcohol Copolymer.

OBJECTIVE: To evaluate the safety and efficacy of ethylene vinyl alcohol (EVOH) copolymer for the treatment of a variety of peripheral vascular pathologies. RESULTS: Between October 2010 and October 2017, 43 patients who underwent total 54 EVOH embolization procedures for the treatment of peripheral vascular pathologies were included. The cases which involved the use of EVOH for the treatment of nonvascular, neurologic, ophthalmologic, otolaryngologic or head-neck pathologies were excluded. The demographic data, technical and clinical success rates, and procedure-related details and complications were obtained. The most common indications for EVOH embolization were type II endoleaks (n = 18) and peripheral arteriovenous malformations (n = 14). The majority of cases (62.5%) used EVOH without any adjunct embolic material. The results of this study showed 100% technical success rates and 89% clinical success rates. No events of nontarget embolization or other procedure-related complications were noted. The mortality & morbidity rates were 0%. The loss to follow up rate was 16% (9 /54). The mean follow-up period was 134 days (range, 30 to 522 days). CONCLUSION: The single institutional experience supports the safety and efficacy of EVOH embolization in the treatment of various peripheral vascular conditions.

Seamless post-operative removal of JJ stents in the paediatric population: Securing the JJ stent to the tip of the urethral balloon catheter avoids a further operation.

In children, removal of a post-procedural JJ stent requires a further cystoscopy under general anaesthetic. We describe securing the distal end of the ureteric stent to the tip of a balloon catheter at the end of the primary procedure. Once post-operative oedema has subsided, the stent is removed seamlessly in tandem with the balloon catheter. This can be done on the ward by nursing staff without the need for general anaesthesia or sedation. Our experience in the first 10 successive patients (aged 1.6-16.3 years) demonstrates this technique to be safe, easy to learn and well tolerated.

Cystoscopic injection sclerotherapy for bladder venous malformations.

Vascular malformations of the urinary bladder are rare in children and their management can be challenging. This minimally invasive approach was first described by the authors in lead 2013 and demonstrated that cystoscopic injections were safe without breech into the peritoneal cavity [1]. Cystoscopic injection sclerotherapy can be very successful in managing the complications of bladder vascular malformations and thereby avoiding extensive surgery. This VideoBank article demonstrates the technique of cystoscopic sclerotherapy for the management of bladder venous malformations.

Fatal Lung Toxicity After Intralesional Bleomycin Sclerotherapy of a Vascular Malformation.

Although there has been increased utilization of bleomycin in the treatment of low-flow vascular malformations in children, previous studies report minor adverse effects limited to skin changes/necrosis and flu-like symptoms (Horbach et al. in J Plast Reconstr Aesthet Surg 69(3):295-304, 2016). However, there have been rare reported cases of pulmonary injury observed in children after bleomycin intralesional administration. We report a case of fatal lung toxicity in a 15-month-old girl after injecting 7 units of bleomycin into a left cheek macrocystic lymphatic malformation. 1 week after therapy, she developed respiratory distress with imaging findings of pneumothorax and diffuse alveolar damage. Despite extensive management and resuscitative efforts of presumed pneumonitis, further decline resulted in death via respiratory failure. Early detection of pulmonary toxicity would allow prompt therapy and could avoid significant pulmonary damage.

Inhibition of mTOR Signaling Enhances Maturation of Cardiomyocytes Derived From Human-Induced Pluripotent Stem Cells via p53-Induced Quiescence.

BACKGROUND: Current differentiation protocols to produce cardiomyocytes from human induced pluripotent stem cells (iPSCs) are capable of generating highly pure cardiomyocyte populations as determined by expression of cardiac troponin T. However, these cardiomyocytes remain immature, more closely resembling the fetal state, with a lower maximum contractile force, slower upstroke velocity, and immature mitochondrial function compared with adult cardiomyocytes. Immaturity of iPSC-derived cardiomyocytes may be a significant barrier to clinical translation of cardiomyocyte cell therapies for heart disease. During development, cardiomyocytes undergo a shift from a proliferative state in the fetus to a more mature but quiescent state after birth. The mechanistic target of rapamycin (mTOR)-signaling pathway plays a key role in nutrient sensing and growth. We hypothesized that transient inhibition of the mTOR-signaling pathway could lead cardiomyocytes to a quiescent state and enhance cardiomyocyte maturation. METHODS: Cardiomyocytes were differentiated from 3 human iPSC lines using small molecules to modulate the Wnt pathway. Torin1 (0 to 200 nmol/L) was used to inhibit the mTOR pathway at various time points. We quantified contractile, metabolic, and electrophysiological properties of matured iPSC-derived cardiomyocytes. We utilized the small molecule inhibitor, pifithrin-α, to inhibit p53 signaling, and nutlin-3a, a small molecule inhibitor of MDM2 (mouse double minute 2 homolog) to upregulate and increase activation of p53. RESULTS: Torin1 (200 nmol/L) increased the percentage of quiescent cells (G0 phase) from 24% to 48% compared with vehicle control (P<0.05). Torin1 significantly increased expression of selected sarcomere proteins (including TNNI3 [troponin I, cardiac muscle]) and ion channels (including Kir2.1) in a dose-dependent manner when Torin1 was initiated after onset of cardiomyocyte beating. Torin1-treated cells had an increased relative maximum force of contraction, increased maximum oxygen consumption rate, decreased peak rise time, and increased downstroke velocity. Torin1 treatment increased protein expression of p53, and these effects were inhibited by pifithrin-α. In contrast, nutlin-3a independently upregulated p53, led to an increase in TNNI3 expression and worked synergistically with Torin1 to further increase expression of both p53 and TNNI3. CONCLUSIONS: Transient treatment of human iPSC-derived cardiomyocytes with Torin1 shifts cells to a quiescent state and enhances cardiomyocyte maturity.

Shared perioperative decision making: a shift in the doctor-patient paradigm.

The high-risk surgical patient only constitutes approximately 4% of the elective non-cardiac surgical population but contributes to the vast majority of in-hospital deaths following surgery. This, in conjunction with a high morbidity rate, can lead to a perioperative pathway fraught with challenges. It is incredibly difficult to anticipate which complications may arise and the risks involved before surgery. It is for this reason that patients need to be engaged in the decision-making processes regarding their perioperative care involved before major surgery. A combination of good medical practice, medicolegal influences and a governmental drive have begun to result in a shift away from paternalistic medicine to a shared decision-making approach. This article defines shared decision making, explores its benefits and limitations and addresses the relevant legal literature.

An Assembled Prototype Multimaterial Three-Dimensional-Printed Model of the Neck for Computed Tomography- and Ultrasound-Guided Interventional Procedures.

A low-cost, semirealistic, multimaterial prototype phantom of the neck was developed for computed tomography- and ultrasound-guided interventions, using three-dimensional (3D) printing with a variety of materials as well as through molding techniques. This dual-modality phantom can be used by trainees for practicing procedures and can also serve as a prototype for developing more complex and realistic 3D-printed models, particularly with the continued development and advancement in multimaterial 3D printing technologies.

Educational survey of regional general practitioner's management of paediatric patients with undescended testis.

INTRODUCTION: Recent recommendations have lowered the ideal age of surgery for undescended testis (UDT) to 3-6 months of age. However, many publications demonstrate that age at surgery is still above the recommended age of 1 year as originally suggested in 1996. AIM: Through a web-based educational survey, we aimed to combine questions regarding General Practioner's (GPs) management of these patients with educational slides with advice to update them with current recommendations. METHODS: The regional GPs were invited by email and letter to undertake the web-based questionnaire devised using SurveyMonkey(®). Educational slides were shown after each questionnaire slide. Feedback was immediate and a one-page summary was emailed to the GP on completion. A pre- and post-educational intervention audit was undertaken to ascertain the change in age of referral for patients <5 years of age. RESULTS: 144 (36%) of 401 GPs undertook this survey. 84% were happy assessing infants (<1year) with UDT. 16% were unhappy discussing management with parents for palpable UDT. 52% were happy discussing malignant risk with parents. 80% thought that ultrasonography was routinely used. Optimal referral time was thought to be 6-12 months (42%) and time of surgery was 1-2 years (50%). 72% would refer a patient with palpable UDT after 6 months of age. Only 41% were happy to assess testicular size at puberty. 98% found this format of an educational survey was helpful. The average age of referral for patients <5 years improved significantly after educational intervention from 2.8 years in 2010 to 1.25 years in 2013 (p < 0.01). DISCUSSION: With an interactive survey, we were able assess and also educate the regional GPs with regard to management of paediatric patients with UDT. There is a varied range of knowledge and practice demonstrated which we hoped to standardise and thereby increase efficiency and decrease the age of referral. A large majority would refer patients with UDT after 6 months of age that would make the target of surgery <6 months unachievable unless they are aware of current recommendations. This study is limited by a 36% response rate but that is comparable to other surveys. Also, referrals come from other sources that were not included. The causal effect of the educational survey is hypothesised. CONCLUSION: This educational survey has confirmed the varied management by GPs. The referral age was demonstrated to be reduced after this intervention and this process was widely accepted by GPs that undertook the educational survey.

The role of PET scan in monitoring the progression of fibrosing mediastinitis.

We present the case of a 31-year-old man who presented with acute chest pain. Computed tomography scan showed a mediastinal mass engulfing right main-stem bronchus and another mass surrounding descending aorta. Positron emission tomography (PET) scan showed high mass metabolic activity. Histopathological evaluation revealed fibroinflammatory scarring. He was diagnosed with idiopathic fibrosing mediastinitis, started on prednisone and tamoxifen treatment, and monitored with serial PET scans. Nine months after treatment initiation, paraaortic abnormality had resolved and mediastinal mass had regressed.

Analysis of Hand Size and Ergonomics of Instruments in Pediatric Minimally Invasive Surgery.

Moving from the study conducted in 2004 on adult surgeons we want to analyze the ergonomics applied in pediatric minimally invasive surgery. An online survey was conducted among the members of the European Paediatric Surgeons' Association that included 14 questions pertaining to demographic, surgical glove size, double glove use, prior hand surgery, and the ease or difficulty in using different types of laparoscopic instruments. A total of 138 pediatric surgeons completed the survey. The difficulty score (DS) was similar between the 3.5- and 5-mm instruments. Other specialized instruments such as Ligasure, Ultracision, Clip applicators, endobags, and staplers were found to have higher DS. The needle holder was the only instrument that is part of the normal 5-mm operating sets, which was found to have a higher DS. Our survey found increased DS with the endobag and stapler, but this was not significant. Also prior hand surgery or double glove use was not associated with difficulty in usage of minimally invasive instruments when compared with normal hands in this survey.

A case of massive airway clotting after use of activated factor VII for massive hemoptysis: management with flexible bronchoscopy and cryoadhesion.

This case describes the use of flexible bronchoscopy and cryoadhesion in an 83-year-old man who developed large airway clots following treatment with activated factor VII for a complication of pulmonary hemorrhage during coronary artery bypass graft.

Long-term outcomes of laparoscopic intracorporeal inguinal hernia ligation in infants under 1 year of age.

BACKGROUND: The majority of current literature regarding laparoscopic inguinal hernia repair group all their pediatric patients together. This study aimed to evaluate the long-term outcomes of patients who underwent laparoscopic intracorporeal inguinal hernia ligation under the age of 1 year to ascertain if there were hidden advantages or limitations in this particular group. PATIENTS AND METHODS: Patients under the age of 1 year at time of laparoscopic intracorporeal inguinal hernia ligation by a single pediatric surgeon were identified. Data were obtained from case notes, hospital electronic records, and parental telephone consultation. RESULTS: One hundred fourteen patients under the age of 1 year underwent laparoscopic inguinal hernia repair from November 2003 to October 2010. The male:female ratio was 4:1. The mean corrected age at surgery was 15.5 (range, 3-42) weeks, and the mean weight at surgery was 5.3 (range, 1.9-9.8) kg. All patients' case notes and electronic records were accessed. Forty-two percent of parents were contactable (mean of 4 years following surgery). There was one recurrence on the asymptomatic side in a boy that presented 3 years later. Five patients required orchidopexy following laparoscopic hernia repair, although 3 cases were known to be undescended at the time of surgery. One patient had intermittent ipsilateral groin swelling that resolved spontaneously after 6 months. CONCLUSIONS: Laparoscopic intracorporeal inguinal hernia ligation in patients under the age of 1 year is safe and effective. Recurrence rates may be lower than those in patients under 1 year of age undergoing a traditional open repair. However, there may be a higher rate iatrogenic of cryptorchidism in this laparoscopic group.

Outcomes of a structured training programme for paediatric laparoscopic inguinal hernia repair.

INTRODUCTION: A structured training programme for laparoscopic paediatric inguinal hernia (LPIH) repair was devised. This programme was evaluated to ensure patient safety by assessing long-term outcomes of operated patients. METHOD: Training Programme - Trainees undergo at least monthly consultant-supervised simulation sessions using the LPIH model. They then undertake live-operating in a step-wise progression: (A) laparoscopic ports insertion and wound closure, (B) intra-corporeal knot tying, and (C) purse-string suturing of hernial orifice. Study - All patients undergoing LPIH repair from July 2003 to Sept 2011 were analysed. Trainee involvement was recorded prospectively, and patients were classified: Group 1 - Consultant only; Group 2 - Trainee performed step A; Group 3 - Trainee performed steps A & B; Group 4 - Trainee performed all steps. RESULTS: 224 patients were identified (175 male; 49 female). Average age at surgery was 2.2 years [range: premature (35/40 weeks) to 15 years]. The laterality of the inguinal hernia was: right (n=133), left (n=75), bilateral (n=16). Primary operator was defined as Group 1 n=88 (39%), Group 2 n=25 (12%), Group 3 n=57 (25%), and Group 4 n=54 (24%). Hernia recurrence occurred in 2 (1.8%) children where consultant operated and two (1.8%) where a trainee operated. Post-operative groin swelling (resolved spontaneously) occurred in 2 (1.8%) where consultant operated and 2 (1.8%) of the trainee cases. There were 3 children with iatrogenic post-operative cryptorchidism requiring subsequent orchidopexy, all from Group 3. CONCLUSION: This study shows that comparable outcomes can be achieved with a structured training programme for LPIH repair for trainees. The exception appears to be iatrogenic cryptorchidism that only occurred in trainee Group 3.

Transverse colonic volvulus in a child: successful management with decompression and delayed laparoscopic colopexy.

We report a case of colonic volvulus in a 7-year-old child with normal development. Operative findings at laparotomy showed a 270-degree transverse colonic volvulus (TCV), with colonic ligamentous attachment abnormalities. A loop ileostomy was formed following detorsion, with delayed laparoscopic colopexy. This is the first case to describe decompression by diversion ileostomy as acute management of TCV with delayed laparoscopic colopexy, so avoiding unnecessary bowel resection.