Fundoplication in children with esophageal atresia: preoperative workup and outcome.

Up to 45% of esophageal atresia (EA) patients undergo fundoplication during childhood. Their esophageal dysmotility may predispose to worse fundoplication outcomes compared with patients without EA. We therefore compared fundoplication outcomes and symptoms pre- and post-fundoplication in EA patients with matched patients without EA. A retrospective review of patients with- and without EA who underwent a fundoplication was performed between 2006 and 2017. Therapeutic success was defined as complete sustained resolution of symptoms that were the reason to perform fundoplication. Fundoplication indications of 39 EA patients (49% male; median age 1.1 [0.1-17.0] yrs) and 39 non-EA patients (46% male; median age 1.3 [0.3-17.0] yrs) included respiratory symptoms, brief resolved unexplained events, typical symptoms of gastroesophageal reflux disease, recurrent strictures and respiratory problems. Post-fundoplication, therapeutic success was achieved in 5 (13%) EA patients versus 29 (74%) non-EA patients (P<0.001). Despite therapeutic success, all 5 (13%) EA patients developed postoperative sustained symptoms/complications versus 12 (31%) non-EA patients. Eleven (28%) EA patients versus 3 (8%) non-EA patients did not achieve any therapeutic success (P=0.036). Remaining patients achieved partial therapeutic success. EA patients suffered significantly more often from postoperative sustained dysphagia (41% vs. 13%; P=0.039), gagging (33% vs. 23%; P<0.001) and bloating (40% vs. 17%; P=0.022). Fundoplication outcomes in EA patients are poor and EA patients are more susceptible to post-fundoplication sustained symptoms and complications compared with patients without EA. The decision to perform fundoplication in EA patients with proven gastroesophageal reflux disease needs to be made with caution after thorough multidisciplinary evaluation.

Clinical significance of liver histology on outcomes in biliary atresia.

AIM: Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes. METHODS: Retrospective chart review of all BA cases between 1999 and 2014. Age at KP, liver biopsy results, use of ursodeoxycholic acid or prophylactic antibiotics and occurrence of PHT and ascending cholangitis post-KP were recorded and related to the primary post-KP outcome measures. RESULTS: BA was diagnosed in 29 patients. Twenty-four of 29 patients underwent KP. Median age at KP was 68 days (29-104). Fourteen of 24 (58.3%) had bridging fibrosis and 5 of 24 (20.8%) had cirrhosis at time of KP. Median follow-up was 8.4 years (2.08-15.58 years). Bilirubin normalisation within 6 months occurred in 7 of 24 (29.2%) patients and 5-year NLS was 45.8% (11/24). Fourteen of 24 (58.3%) had PHT and 18 of 24 (75%) patients had ascending cholangitis post-KP. Absence of bridging fibrosis in liver histology at KP was the only factor to be significantly associated with improved 5-year NLS. None of the other variables examined had a significant association with either 5-year NLS or bilirubin normalisation by 6 months. CONCLUSION: Five-year NLS in this series was 45.8%. Absence of bridging fibrosis at time of KP was the only factor significantly associated with improved 5-year NLS.

Reduction of Urinary Tract Infection in Pediatric Surgical Patients Using NSQIP-P and Quality Improvement Methodology.

BACKGROUND: Hospital-acquired urinary tract infections (UTIs) have a detrimental effect on patients, families, and hospital resources. The Sydney Children's Hospital Network (SCHN) participates in the NSQIP-Pediatric (NSQIP-P) to monitor postoperative complications. NSQIP-P data revealed that the median UTI rate at SCHN was 1.75% in 2019, 3.5 times higher than the NSQIP-P target rate of 0.5%. Over three quarters of the NSQIP-P identified patients with UTI also had a urinary catheterization performed intraoperatively. A quality improvement project was conducted between mid-2018 and 2021 to minimize catheter-associated UTIs (CAUTIs) at SCHN. STUDY DESIGN: NSQIP-P samples include pediatric (younger than 18 years) surgical patients from an 8-day cycle operative log. NSQIP-P data are statistically analyzed by the American College of Surgeons and provide biannual internationally benchmarked reports. The project used clinical redesign methodology with a 6-phase process for quality improvement projects. RESULTS: The objectives of the project were to reduce urinary catheter duration of use, educate parents or carers, and improve catheter care and insertion technique by health staff. The duration of a urinary catheter in situ reduced from a median of 4.5 to 3 days from 2017 to 2021. The median NSQIP-P UTI rate at SCHN was reduced by 47.4% from 1.75% in 2019 to 0.9% in 2022. CONCLUSIONS: A multifactorial approach in quality improvement has been shown to be an effective strategy to reduce UTI rates at SCHN, and patient outcomes were improved within a 3-year timeframe. Although this project has reduced UTI rates at SCHN, there remain opportunities for further improvement.

Congenital mesoblastic nephroma: review of current management and outcomes in a single centre.

BACKGROUND: Congenital mesoblastic nephroma (CMN) is a rare tumour of the kidney with an overall excellent prognosis. Once considered a benign tumour, it is now recognized to carry a risk of recurrence and metastases with subsequent poor outcomes. The potential for genetic aberrations such as ETV6-NTRK3 fusion raises the potential for targeted treatments in certain patients. The optimum mode and frequency of surveillance is unclear. This study aims to assess this institution's experience with CMN and long-term outcomes. METHODS: A single centre retrospective review was performed of all confirmed cases of CMN between October 2001 and January 2021. RESULTS: Nine cases of CMN in patients under 12 months of age were identified. The histopathology, management and outcomes of these patients are discussed. CONCLUSION: CMN overall has a very good prognosis, but a subgroup does exist that will have poor outcomes. It is difficult to accurately identify this group to target adjuvant therapy.

A complicated case of terminal ileitis post-COVID-19 infection requiring bowel resection.

Since the beginning of the COVID-19 pandemic, there have been reports of children developing systemic hyperinflammatory response to COVID-19 infection, known as Paediatric Inflammatory Multisystem Syndrome Temporally associated with SARS-COV-2 (PIMS-TS). Here we would like to discuss a case of a 15-year-old male with PIMS-TS presenting as complicated terminal ileitis, requiring ileocaecal resection. Histopathologic findings of the ileocaecal specimen revealed thickened bowel mucosa, with features of granulomatous inflammation similar to Crohn's disease, without features of intestinal vasculitis or viral particles. His post-operative course was complicated by fresh rectal bleeding, requiring urgent blood transfusion and endoscopy. Source of bleeding was not identified and his bleeding self-resolved. Gut biopsies of the remaining bowel taken during endoscopy were normal. Although this case suggests that the inflammatory process is focal to the terminal ileum with a good short-term prognosis, further follow-up is required to assess for long-term effects and risks of bowel disease.

Fetal abdominal wall defects in an Australian tertiary setting: contemporary characteristics, ultrasound accuracy, and outcome.

PURPOSE: In this study, we aimed to comprehensively evaluate risk factors, ultrasound estimation of fetal weight, prenatal management, and pregnancy outcomes of gastroschisis and omphalocele at a metropolitan Australian hospital. MATERIAL AND METHODS: This was a retrospective single-center cohort study from 2006 to 2014 at a tertiary hospital with colocated neonatal surgical facilities. Demographic, pregnancy, ultrasound, birth and neonatal data were compared between gastroschisis and omphalocele. Correlation between routine (Hadlock 1 &2) and specific (Siemer) estimated fetal weight (EFW) estimation formulae with birth weight (BW) was made for those 50 gastroschisis cases with ≥2 third trimester scans and last scan ≤2 weeks prior to birth. RESULTS: There were 126 abdominal wall defects: 83 gastroschisis and 43 omphalocele. Consistent with international literature, the average maternal age was lower for gastroschisis and rates of smoking higher, while there were more intrauterine deaths and pregnancy terminations in omphalocele. Gastroschisis mothers were more likely living outside Sydney, had more infections in pregnancy and were followed with a larger number of antenatal visits, with a shorter period from the last visit to birth. In omphalocele pregnancies, amniocentesis was more likely performed, with more abnormal results than in gastroschisis fetuses. All EFW formulae had a good correlation between Z score for the last US and actual BW (ICC 0.693-0.815), with Hadlock 2 being the best. Siemer formula had the best correlation from first to the last scan. Gastroschisis newborns were born earlier (36.8 versus 38.2 wks p = .001), with smaller birthweight (2.52 versus 3.03 kg, p < .001), a longer request of intensive care (central line, parenteral nutrition, intubation) and second surgery, along with more multisystem complications (average 1.5 versus 0.7, p = .004) and a longer hospital stay (58.8 versus 36.8 d, p < .001). CONCLUSION: Demographic, antenatal, and pregnancy outcome data for abdominal wall defects correlated well with the international literature. Hadlock 1-2 gave the most consistent EFW estimate, with all formulae showing good correlation.

Analysis of variants in GATA4 and FOG2/ZFPM2 demonstrates benign contribution to 46,XY disorders of sex development.

BACKGROUND: GATA-binding protein 4 (GATA4) and Friend of GATA 2 protein (FOG2, also known as ZFPM2) form a heterodimer complex that has been shown to influence transcription of genes in a number of developmental systems. Recent evidence has also shown these genes play a role in gonadal sexual differentiation in humans. Previously we identified four variants in GATA4 and an unexpectedly large number of variants in ZFPM2 in a cohort of individuals with 46,XY Differences/Disorders of Sex Development (DSD) (Eggers et al, Genome Biology, 2016; 17: 243). METHOD: Here, we review variant curation and test the functional activity of GATA4 and ZFPM2 variants. We assess variant transcriptional activity on gonadal specific promoters (Sox9 and AMH) and variant protein-protein interactions. RESULTS: Our findings support that the majority of GATA4 and ZFPM2 variants we identified are benign in their contribution to 46,XY DSD. Indeed, only one variant, in the conserved N-terminal zinc finger of GATA4, was considered pathogenic, with functional analysis confirming differences in its ability to regulate Sox9 and AMH and in protein interaction with ZFPM2. CONCLUSIONS: Our study helps define the genetic factors contributing to 46,XY DSD and suggests that the majority of variants we identified in GATA4 and ZFPM2/FOG2 are not causative.

A 9-year audit of fetal chest masses in an Australian maternal-fetal medicine cohort.

INTRODUCTION: To assess fetal vs. neonatal diagnoses, pregnancy outcomes and need for surgery in babies prenatally diagnosed with congenital pulmonary airway malformation (CPAM) or bronchopulmonary sequestration (BPS). METHODS: Retrospective single-centre cohort study of fetuses with a prenatal diagnosis of CPAM or BPS between 2006 and 2014. Data collected included serial antenatal ultrasound information and neonatal/infant diagnoses and outcomes. RESULTS: Initial ultrasound diagnosis (n = 63) was CPAM in 51 and BPS in 12: nineteen (30%) fetuses had mediastinal shift and 2 (3%) had hydrops. All neonates with known birth outcome (n = 56) were liveborn. Final diagnosis in 52 infants (83%) with neonatal imaging and/or histopathology confirmed CPAM in 17/44 (39%) and BPS in 6/9 (67%). Of 34 prenatally suspected but unconfirmed CPAM lesions: 10 had no lesion on neonatal imaging, one no neonatal imaging performed, five confirmed BPS, 11 other lung/thoracic lesions, seven were lost to follow-up. There was one infant death. 27/63 infants (43%) had post-natal surgery. More neonates requiring neonatal respiratory support/resuscitation had surgery compared to those who did not (67% vs. 29%, P = 0.008). Patients with suspected CPAM or BPS on both initial and final ultrasound were more likely to have post-natal surgical management than when a lesion was no longer visible on final antenatal ultrasound (68% vs. 23%, P = 0.001). CONCLUSIONS: Over 50% of antenatally suspected CPAM/BPS either regressed or had an alternate post-natal diagnosis. Perinatal outcome was good, with the majority of neonates/infants conservatively managed. Persistently visible antenatal lesion and need for neonatal respiratory support predicted ultimate surgical management.

Is fundoplication required after the Foker procedure for long gap esophageal atresia?

BACKGROUND: Fundoplication has been performed almost universally in children treated with the Foker procedure (FP) for long gap esophageal atresia (LGEA). We report our experience with pharmacological management and endoscopic surveillance rather than early routine fundoplication in infants treated with the FP. METHODS: A retrospective chart review was performed of all children treated with the Foker procedure at our institution. RESULTS: Nine children have undergone the FP since 2007. Median time between FP and definitive anastomoses was 22days. All nine children kept their native esophagus. There were three minor anastomotic leaks, all treated nonoperatively. All children required dilatation of anastomotic strictures (range 2-15). All have been treated with proton pump inhibitors. Three children had eosinophilic esophagitis and one had Barrett's esophagus. Only two children in this series have undergone fundoplication, which was performed for symptomatic and persistent erosive esophagitis. CONCLUSION: The question of early versus delayed fundoplication in LGEA patients managed with the FP remains unanswered. Our series demonstrates that it is possible to achieve good long-term outcomes when the operation is reserved for children with gastroesophageal reflux disease resistant to maximal medical therapy. LEVEL OF EVIDENCE: IV.

Outcomes of infants with abdominal wall defects over 18years.

BACKGROUND/PURPOSE: Infants with abdominal wall defects (AWD) are at risk of poor outcomes including prolonged hospitalization, infections and mortality. Our objective was to describe and compare the outcomes of infants admitted with gastroschisis and omphalocele over 18years. METHODS: Population-based study of clinical data and outcomes of live-born infants with AWD admitted to all tertiary-level neonatal intensive care units in New South Wales and Australian Capital Territory from 1992 to 2009. RESULT: There were 502 infants with AWD - 336 gastroschisis, 166 omphalocele. Infants with gastroschisis required a longer duration of total parenteral nutrition (19 vs 4days, p<0.05), longer hospitalization (28 vs 15days, p<0.05) and had a higher rate of systemic infection [23.5% vs 13.3%, OR 1.77 (1.15-2.74), p<0.05] compared to infants with omphalocele. Overall, omphalocele infants had higher mortality rate compared to gastroschisis infants [OR 2.77 (1.53, 5.04), p<0.05]. Gastroschisis mortality rates increased from epoch 1 to epoch 3 (4.2% to 8.8%). CONCLUSION: Compared to infants with omphalocele, infants with gastroschisis required significantly longer hospitalization and parenteral nutrition with higher rates of infection. Infants with omphalocele had higher overall mortality rates. However, there has been an increase in the gastroschisis mortality rates but the cause for this is unclear.

Perforation in a jejunal duplication lined by ectopic gastric mucosa.

We report a 3-year-old child with jejunal duplication with localized perforation and bleeding in the adjacent normal intestine. The entire duplicated jejunum was lined by gastric mucosa. This was successfully treated by mucosal excision of the duplicated intestine and resection of a short segment of normal jejunum.

Disseminated spina ventosa.

Spina Ventosa is a rare condition. A rare case of disseminated tuberculosis of bones and skin without primary foci is presented. Gross sclerosis of the short bones of hand and leg were noted.

Perineal canal: an uncommon entity with good prognosis.

This article reports two patients with perineal canal (anovestibular fistula) who were treated at Dept. of Pediatric Surgery, LTMG Hospital, Mumbai. Both the patients had normal anus and a fistula between the anal canal and the vestibule. They were treated by vestibulo anal pull through procedure. The pathogenesis of this condition and the pertinent literature is reviewed.

Pulmonary gangrene complicating bacterial pneumonia.

Two cases of pulmonary gangrene involving left lower lobe in an 18-month and 4-year-old female children are reported. The patients looked like having empyema following Klebsiella pneumonia. The diagnosis was made following computerized tomography scan and during decortication respectively.

Multiseptate gallbladder in an asymptomatic child.

A one-year-old child being investigated for urinary tract infection was diagnosed with a multiseptate gallbladder. The patient remains asymptomatic, and investigations demonstrate no associated anomalies. Forty-three cases, including 13 cases in children were identified in the literature. Their presentation and management were reviewed.

Propranolol: useful therapeutic agent for the treatment of ulcerated infantile hemangiomas.

Infantile hemangioma (IH) is the most common vascular tumor in early childhood. Ulceration is the most frequent complication, and its management can be challenging. We present 6 cases of ulcerated IH at a single pediatric center, which responded to oral propranolol within 2 to 6 weeks. We recommend that oral propranolol therapy be considered for the management of ulcerated IH as first-line treatment.

Successful treatment of recurrent epididymo-orchitis: Laparoscopic excision of the prostatic utricle.

Prostatic utricle presenting with recurrent epididymo-orchitis is not uncommon. Excision of prostatic utricle is the treatment of choice. The various techniques described in literature suffer from the disadvantages of incomplete excision due to poor view. We report the successful laparoscopic excision of prostatic utricle in childhood.