European Pediatric Surgeons' Association Survey on Timing of Inguinal Hernia Repair in Premature Infants.

AIM: This article evaluates the practice patterns of European Pediatric Surgeons' Association (EUPSA) members regarding the timing of inguinal hernia (IH) repair in premature infants. METHODS: Online survey containing 29 questions distributed to EUPSA members during January 2023. RESULTS: A total of 180 responds were received. Overall, IH repair prior to discharge was favored by 60% of respondents when there was a history of incarceration and 56% when there was not. In the case of very/extremely premature infants (< 32 weeks) with no history of incarceration, fewer (43%) respondents postpone the surgery until after discharge. The majority of respondents cited the risk of incarceration as the reason for advocating surgery prior to discharge, whereas a reduced risk of apnea was the most cited reason for respondents who prefer delayed surgery. Open approach under general anesthesia was favored by 54% of respondents, with 27% of them preferring open approach with spinal anesthesia. Laparoscopic surgery for premature infants is used in 11% while 7% of them preferred in all premature infants including extremely/very premature ones. Contralateral side evaluation was never done by 40% of respondents and 29% only performed it only during laparoscopic repair. The majority of respondents (77%) indicated that they have an overnight stay policy for premature infants < 45 weeks of gestation. CONCLUSION: There is variation in the practice patterns of pediatric surgeons in the treatment of IH in premature infants. Due to the concern for the high risk of incarceration, IH repair before discharge was the most prevalent practice. Lower risk of postoperative apnea was cited as the most common reason for delaying surgery. Randomized studies are required to establish the optimal timing for IH repair in premature infants.

Congenital lung malformations.

Congenital lung malformations (CLMs) are rare developmental anomalies of the lung, including congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and isolated congenital bronchial atresia. CLMs occur in 4 out of 10,000 live births. Postnatal presentation ranges from an asymptomatic infant to respiratory failure. CLMs are typically diagnosed with antenatal ultrasonography and confirmed by chest CT angiography in the first few months of life. Although surgical treatment is the gold standard for symptomatic CLMs, a consensus on asymptomatic cases has not been reached. Resection, either thoracoscopically or through thoracotomy, minimizes the risk of local morbidity, including recurrent infections and pneumothorax, and avoids the risk of malignancies that have been associated with CPAM, bronchopulmonary sequestration and bronchogenic cyst. However, some surgeons suggest expectant management as the incidence of adverse outcomes, including malignancy, remains unknown. In either case, a planned follow-up and a proper transition to adult care are needed. The biological mechanisms through which some CLMs may trigger malignant transformation are under investigation. KRAS has already been confirmed to be somatically mutated in CPAM and other genetic susceptibilities linked to tumour development have been explored. By summarizing current progress in CLM diagnosis, management and molecular understanding we hope to highlight open questions that require urgent attention.

Quality of life in flexible painful flatfoot treated by anterograde calcaneo-stop procedure: The patient's and family's perspective.

PURPOSE: This study aimed to evaluate the quality of life and satisfaction about the surgical treatment in patients with symptomatic flexible flatfoot. METHODS: The Oxford Ankle Foot Questionnaire for children (one to fill in before the surgical correction and another 6-12 months after the screw's removal), the PedsQLTM Healthcare Satisfaction Generic Module and the PedsQLTM General Well-Being Scale were administered to all patients who underwent the anterograde calcaneo-stop procedure for flexible painful flatfoot between January 2012 and December 2015. RESULTS: One hundred forty patients were sent the questionnaires and 74 (40 male and 34 female) of them responded. The surgical correction was performed at a medium age of 11,84±1,65 years. When the Oxford Ankle Foot Questionnaire for children scores before surgical correction and after the screw removal were compared, the latter scored significantly higher for all domains. Healthcare satisfaction was good in all families. Most of the patients scored medium-high on the PedQLTM General Well-Being both when asked about themselves (mean 86,50±7,44) and in general about their health (76,06±12,32). CONCLUSION: Our results confirmed that flexible painful flatfoot is significantly affecting the quality of life of children and that the anterograde calcaneo-stop procedure is a valuable technique, which improves their quality of life and the family wellbeing.

Are Pedobarographic and Gait Analyses Useful Tools to Evaluate Outcomes of Anterograde Calcaneo-Stop Procedure in Pediatric Symptomatic Flexible Flatfoot?

BACKGROUND: Flexible flatfoot is a frequent condition in childhood that needs to be treated when symptomatic. The aim of this study was to analyze pedobarographic and gait outcomes of patients with painful flexible flatfoot who underwent the anterograde calcaneo-stop procedure. METHODS: All patients scheduled for surgical correction of painful flexible flatfoot between April and September 2011 were offered to participate in a study of dynamic pedobarographic and gait analyses before surgery and 3, 12, and 24 months after surgery. A healthy control group of similar age and physical characteristics also underwent dynamic pedobarographic and gait analyses. RESULTS: Fifteen patients accepted to undergo dynamic pedobarography and gait analyses. The data were compared with fifteen controls of similar age and BMI. No significant differences were found on dynamic pedobarography within patients at different endpoints, except for a decreased percentage of plantigrade phase and increased percentage of digitigrade phase at 12 months post-op in comparison with 3 months post-op, nor when compared with control. Similarly, when range of motion was taken into consideration, no significant differences were found within patients at different endpoints, nor when compared with control, except for a decrease in ankle joint range of motion 24 months post-op in comparison with the controls. The stride was significantly decreased before surgery and became like controls 24 months after the procedure. The cadence, significantly decreased 24 months after surgery in comparison with the pre-surgical set, was similar to the controls. No significant differences were seen in the walking speed within patients at different endpoints and the controls. The cycle time significantly increased 24 months after surgery when compared to the pre-op situation, and was similar to the controls. CONCLUSION: Dynamic pedobarographic and gait analyses were useful not only to identify the gait impairment in patients with symptomatic flexible flatfoot, but also to measure the treatment outcome through the analysis of the surgery's impact on the gait quality.

Trauma in Children during Lockdown for SARS-CoV-2 Pandemic. A Brief Report.

PURPOSE: The national lockdown established by the Italian government began on the 11th of March 2020 as a means to control the spread of SARS-CoV-2 infections. The purpose of this brief report is to evaluate the effect of the national lockdown on the occurrence and characteristics of trauma in children during lockdown. METHODS: All children admitted to our paediatric orthopaedic unit with a diagnosis of fracture or trauma, including sprains and contusions, between 11 March 2020 and 11 April 2020, were retrospectively reviewed. Their demographic data, type of injury, anatomical location and need for hospitalisation were compared with the equivalent data of children admitted for trauma in the same period of 2018 and 2019. RESULTS: Sixty-nine patients with trauma were admitted in 2020, with a significant decrease in comparison with 2019 (n = 261) and 2018 (n = 289) (p < 0.01). The patients were significantly younger, and the rate of fractures significantly increased in 2020 (p < 0.01). CONCLUSIONS: Home confinement decreased admissions to the emergency department for trauma by shutting down outdoor activities, schools and sports activities. However, the rate of fractures increased in comparison with minor trauma, involved younger children and had a worse prognosis.

Foreign Body Ingestion in Neurologically Impaired Children: A Challenging Diagnosis and Management in Pediatric Surgery.

Children with intellectual disability/neurodevelopmental delay (ID-ND) commonly ingest foreign bodies (FB) and often present complications due to peculiar aspects of their condition. The aim of this paper is to report the experience of two centers in the management of ID-ND patients after FB ingestion and to discuss a possible algorithm for clinical practice. We retrospectively evaluated data of patients managed for FB ingestion (period: 2017-2021), focusing on those with ID-ND, specifically demographics and baseline diagnosis, elements related to the event, symptoms, time to endoscopy, FB location, endoscopic details, and follow-up. A total of 457 patients were managed in the study period and 19 had ID-ND (mean age 9.8 ± 3.5 years, 15 males). A total of 16/19 (84.2%) were symptomatic and required an operative approach. Recurrent ingestions and multiple FB were found in 2 and 11 patients, respectively. Endoscopy (mean time 65.6 ± 41 min) was effective in 14 cases (73.6%) and 6 patients (31.6%) developed a complication. FB ingestion in ID-ND patients represents a challenging condition for the clinician and a potentially dangerous situation. It should be addressed specifically by a multidisciplinary team considering a tailored diagnostic and management protocol.

Colonic Volvulus in Children: Surgical Management of a Challenging Condition.

Colonic volvulus (CV) is a rare but potentially life-threatening condition with unclear etiopathogenesis. To date, less than 80 pediatric cases have been described. Hirschsprung's disease (HD) is associated with CV in 17% of cases, representing a significant risk factor. Non-HD CV is an even more complex entity. The aim of this study is to describe a series of patients with CV to accentuate some peculiar aspects of this disease. We performed a retrospective study (period: 2012-2021) collecting information of patients with CV. Data analyzed included: demographics, medical history, presenting symptoms and radiological and surgical details. Eleven patients (12.5 ± 2.8 years; 7F/4M) had CV (eight sigmoid, two transverse colon, one total colon). Five patients had associated anomalies and three had HD. A two-step approach with volvulus endoscopic/radiological detorsion followed by intestinal resection was attempted in eight cases (one endoscopic approach failed). Three patients required surgery at admission. At follow-up, two patients developed recurrent intestinal obstruction, one of whom also had anastomotic stenosis. Colonic volvulus is a challenging condition that requires prompt patient care. A missed diagnosis could lead to severe complications. The evaluation of the patient should include a careful histological examination (searching for HD and alpha-actin deficiency), immunologic and metabolic screening, neurological tests and detection of chronic intestinal pseudo-obstruction (CIPO). Lifelong follow-up is mandatory for the early recognition and treatment of progressive diseases involving the proximal gastrointestinal tract.

The Histopathology of the Appendix in Children at Interval Appendectomy.

Whilst most surgeons agree that conservative treatment of appendiceal abscess in children is an adequate treatment, the need for subsequent interval appendectomy is still controversial. We analyzed the histopathology in interval appendectomy in search of signs of inflammation. All patients admitted between 2010 and 2017 with appendiceal abscess and scheduled for interval appendectomy were reviewed. The specimens were evaluated for grade of inflammation, type and distribution of cellular infiltrate, presence of necrosis or hemorrhage and infiltrate in the serosa. Forty-two patients had appendiceal abscess and were treated conservatively. Seven underwent emergent appendectomy. Thirty-three out of 35 patients underwent elective interval appendectomy. Thirty-two specimens were revised. Carcinoid tumor or other malignant lesions were not found. All of them presented some amount of inflammation, grade 1 to 2 in 53%, grade 3 to 4 in 47%. Twenty-five percent of the specimens had signs of necrosis accompanied by hemorrhage and in more than the half (53%) the infiltrate extended to the serosa. Conclusions: Although the appendix was mostly found not macroscopically inflamed intraoperatively, histology confirmed a certain grade of inflammation even months after the conservative treatment. No correlation was found between histopathologic findings and lapse of time between abscess treatment and interval appendectomy.

Whole-Genome Methylation Study of Congenital Lung Malformations in Children.

BACKGROUND AND OBJECTIVES: The treatment of asymptomatic patients with congenital pulmonary malformations (CPMs) remains controversial, partially because the relationship between congenital lung malformations and malignancy is still undefined. Change in methylation pattern is a crucial event in human cancer, including lung cancer. We therefore studied all differentially methylated regions (DMRs) in a series of CPMs in an attempt to find methylation anomalies in genes already described in association with malignancy. METHODS: The DNA extracted from resected congenital lung malformations and control lung tissue was screened using Illumina MethylationEPIC arrays. Comparisons between the group of malformed samples or the malformed samples of same histology or each malformed sample and the controls and between a pleuropulmonary blastoma (PPB) and controls were performed. Moreover, each malformed sample was pairwise compared with its respective control. All differentially methylated regions (DMRs) with an adjusted p-value <0,05 were studied. RESULTS: Every comparison highlighted a number of DMRs closed to genes involved either in cell proliferation or in embryonic development or included in the Cancer Gene Census. Their abnormal methylation had been already described in lung tumors. CONCLUSIONS: Methylation anomalies already described in lung tumors and also shared by the PPB were found in congenital lung malformations, regardless the histology. The presence of methylation abnormalities is suggestive of a correlation between congenital lung malformations and some step of malignant transformation.

MRI in Postreduction Evaluation of Developmental Dysplasia of the Hip: Our Experience.

BACKGROUND: Developmental dysplasia of the hip (DDH) is one of the most common congenital defects in the newborn. When its severe form is not corrected, it is associated with long-term morbidity. Closed reduction with casting is the standard primary treatment and reduction is confirmed by magnetic resonance imaging (MRI). We reported our experience on the reliability of MRI in postreduction assessment of DDH. METHODS: All children who underwent closed reduction for Graf type IV DDH at our institution between September 2010 and June 2016 were retrospectively reviewed. Since 2010 we assessed postreduction position of the femoral head by performing a MRI. RESULTS: Twenty-five (5 male, 20 female) patients presented with 29 (15 left sided, 6 right sided, 4 bilateral) Graf type IV DDH and underwent closed reduction at a mean age of 3.4 months. In all patients MRI studies performed within 24 hours were diagnostic, showing a concentric reduction of the femoral head within the acetabulum in 24/25 patients. In the patient with persistent hip instability, a subsequent open reduction was performed. In all the cases, there was no need of any contention or sedation during MRI. CONCLUSIONS: On the basis of our experience, MRI is an excellent, safe and, reliable modality to confirm maintenance of adequate femoral head position and to evaluate soft tissue interposition. We agree that MRI is the gold standard to early depict dislocation after closed reduction of DDH.

Ogilvie syndrome in a 8 year old girl after laparoscopic appendectomy.

BACKGROUND: Ogilvie's syndrome is described in the adult population, but rarely seen in children. CASE PRESENTATION: We present a case of a girl who suffered acute colonic pseudo-obstruction after laparoscopic appendectomy. CONCLUSIONS: Ogilvie's syndrome, although rare in the pediatric population, should be considered as possible diagnosis after a surgical procedure in presence of persisting subocclusive symptoms and radiological signs of massive colonic dilatation without mechanical obstruction.

Impact of Short Bowel Syndrome on Quality of Life and Family: The Patient's Perspective.

BACKGROUND: Short bowel syndrome (SBS) has an impact on children and their families not only physically, but also emotionally, mentally, and socially. This study aimed to evaluate quality of life and family impact in patients with SBS, using the Pediatric Quality of Life Inventory (PedsQL) measurement model. MATERIALS AND METHODS: PedsQL questionnaires were administered to patients with SBS followed at the Pediatric Surgery of Royal Manchester Children's Hospital. The scores were compared between two groups differing in age (children <5 y vs. >5 y) and with known-groups from literature published by Varni et al. RESULTS: Forty-three patients were sent the questionnaires, and 30 (70%, 17 < 5 and 13 > 5) responded. Family Impact Module failed to distinguish between families of children younger and older than 5 years of age. In contrast, it distinguished between families with SBS children and the ones with children in a long-term care facility. Healthcare satisfaction was worse in families of children over 5 years regarding information received, inclusion of the family, and communication. When compared with normal population, SBS children scored worse on Generic Core Scales for all domains except for emotional functioning. CONCLUSION: Although advances have been made on the treatment of children with SBS, and improvements have been reached on home PN, this condition is still significantly affecting the quality of life of children and their families. The future quality control of medical care must have greater focus on psychosocial and emotional functioning, aiming for the best possible quality of life.