Percutaneous Endoscopic Gastrostomy with T-Fasteners versus "Pull Technique": Analysis of Complications.

INTRODUCTION: The T-fasteners gastrostomy (T-PEG) has become increasingly popular over recent years as an alternative to the "pull-technique" gastrostomy (P-PEG). This study aimed to compare P-PEG and T-PEG complications. MATERIALS AND METHODS: A retrospective observational study of pediatric patients who underwent percutaneous endoscopic gastrostomy (PEG) placement. P-PEG was performed using the standard Ponsky technique and was replaced after 6 months by a balloon gastrostomy under sedation. T-PEG was performed using three percutaneous T-fasteners (that allow a primary insertion of a balloon gastrostomy). The balloon was replaced by a new one after 6 months without sedation. Complications were recorded. RESULTS: In total, 146 patients underwent PEG placement, 70 P-PEG and 76 T-PEG. The mean follow-up was 3.9 years (standard deviation = 9.6). Age, weight, and associated comorbidities were comparable (p > 0.05). The overall complications were 17 (24.2%) in the P-PEG group and 16 (21.0%) in the T-PEG group (p > 0.05). P-PEG was associated with more sedation for button replacement (97 vs. 2.6% [p < 0.05]). P-PEG was associated with more early tube dislodgement during the first replacement (7.2 vs. 1.4% [p = 0.092]). Two of the five dislodged gastrostomies in the P-PEG group underwent laparotomy due to peritonitis, whereas the only dislodged gastrostomy in the T-PEG group was solved endoscopically. Altogether, P-PEG was associated with more complications that required urgent endoscopy, laparotomy, or laparoscopy (18.6 vs. 6.6% [p < 0.05]). CONCLUSIONS: P-PEG was associated with more sedation, complications during first button replacement, and complications requiring urgent endoscopy, laparotomy, or laparoscopy compared with T-PEG.

Anatomical Variations of the External Genitalia in Posterior Cloaca: Clinical Consequences of Misdiagnosis-A Systematic Review of the Literature and the ARM-Net Consortium Experience.

PURPOSE: All types of cloacal malformations may be associated with anatomic variations of the external genitalia, including hypoplasia of the labia minora and enlarged clitoris; these variations could be even higher in posterior cloacas (PCs). If a careful physical examination is not performed, patients may be misdiagnosed with ambiguous genitalia (AG), leading to subsequent unnecessary testing, surgeries, or even wrong gender assignment. The aim was to analyze data of patients with PC within the ARM-Net registry, focusing on the description of the genitalia, gender assignment, and its consequences. Additionally, we investigated the presence of AG diagnosis in utero or at birth in patients with PC in the literature. METHODS: The ARM-Net registry was scanned for PC cases and data on diagnosis were collected. A systematic literature search was conducted using the PubMed, EMbase, and Web-of-Science databases. Descriptive statistics was used to report data. RESULTS: Nine patients with PC were identified in the ARM-Net registry. Five patients (55%) were diagnosed with AG, two (22%) were assigned as males and only two patients were correctly assigned as females and diagnosed with PC with respective variations of external genitalia. All patients diagnosed with AG had extensive blood testing including karyotype and hormonal studies. One of the patients who was diagnosed as a male, had surgery for pelvic cystic mass removal, which ultimately led to unaware salpingo-oophorectomy, hysterectomy, and vaginectomy. In the literature we identified 60 patients, 14 (23%) with AG, 1 with clitorolabial transposition and 1 with undeveloped vulva and vagina; 4 patients had normal anatomy. In 40 (67%) patients the anatomy of genitalia was not mentioned. CONCLUSION: Patients with PC are at high risk of being diagnosed with AG or even assigned the wrong gender at birth. In our series two patients were assigned as males, and consequently one of them underwent a highly mutilating surgery. A thorough physical examination together with a high index of suspicion and laboratory workup are mandatory to identify these variations, avoiding further investigations, unnecessary surgeries, and parental stress.

Esophageal Atresia and Gastric Ectopic Pancreas: Is There a Real Association?

OBJECTIVE: Heterotopic pancreas (HP) is a condition in which there is well-differentiated pancreatic tissue that lacks any anatomic or vascular contact with the pancreatic gland. It normally arises from the stomach but can be found in other locations. Although it is usually asymptomatic, obstructive symptoms, bleeding, or malignant degeneration can occur. The incidence is very low, but it is significantly more common in patients with esophageal atresia (EA). The aim of this study is to evaluate the incidence of HP in patients with and without EA and to compare the results in both groups. MATERIAL AND METHODS: We conducted a 2-year prospective study in pediatric patients who benefited from an upper gastrointestinal endoscopy. Patients were divided into two groups: group "A" comprised patients with EA and group "B" those without EA. The variables analyzed were the clinical presentation, presence of HP, location, associated malformations, genetic disorders, and management. RESULTS: A total of 192 consecutive patients were included in the study: 51 (26.6%) in group A and 141 (73.4%) in group B. Indications for endoscopy in group B were eosinophilic esophagitis in 37 (19.2%) patients, celiac disease in 23 (11.95%) patients, and other disorders in 81 (42.2%) patients. Gastric HP was found in seven patients, all of them in group A. All lesions were hosted in the prepyloric antrum. The prevalence of HP in groups A and B was 13.7 and 0%, respectively (p < 0.05). Female gender was predominant in patients with AE and HP, this result being statistically significant (p = 0.044). No other associated malformation or genetic syndrome studied showed association with HP. Only one patient debuted with upper gastrointestinal (GI) bleeding and required excision, while six patients were asymptomatic. The mean follow-up was 54 months (range: 45-78 months). CONCLUSION: The incidence of gastric HP is more common in patients with EA, with the female gender being a risk factor for their association. Active search and follow-up is recommended as it may become symptomatic anytime and need resection.

Transesophageal echocardiography during pectus excavatum correction in children: What happens to the heart?

OBJECTIVES: Cardiac compression in pectus excavatum remains difficult to evaluate. We describe the findings with intraoperative transesophageal echocardiography during pectus excavatum correction in pediatric patients. METHODS: We studied right heart changes during surgical correction of pectus excavatum by transesophageal echocardiograph. Four-D echo was associated to assess morphology of the tricuspid annulus. RESULTS: Twenty patients were included, mean age 13.5 (+/- 2.9). Mean preoperative Haller Index was 6.3 (+/- 2.63) and mean Correction Index 47.63% (+/- 12.4%). Preoperative transthoracic echocardiography at rest showed mild right heart compression in 6. Correction was gained by Nuss technique in 19, and Taulinoplasty in one. Initial transesophageal echocardiography showed compression of the right heart and deformation of the tricuspid annulus in all. During the sternal elevation, diameters of right atrium, ventricle and tricuspid annulus significantly improved: mean augmentation of right ventricle was 5.78 mm (+/- 3.56 p < 0.05), right atrium 6.64 mm (+/- 5.55 p < 0.05) and tricuspid annulus 6.02 mm (+/- 3.29 p < 0.05). The morphology of the tricuspid annulus in 4D normalized. CONCLUSIONS: Preoperative transthoracic echocardiography at rest underestimates right chamber compression in pediatric patients with pectus excavatum. Surgical correction improves diameters of the right ventricle, right atrium and tricuspid annulus and normalizes the morphology of the tricuspid annulus (4D). LEVEL OF EVIDENCE: Level III.

Inter- and Intraobserver Variation in the Assessment of Preoperative Colostograms in Male Anorectal Malformations: An ARM-Net Consortium Survey.

Aim: Male patients with anorectal malformations (ARM) are classified according to presence and level of the recto-urinary fistula. This is traditionally established by a preoperative high-pressure distal colostogram that may be variably interpreted by different surgeons. The aim of this study was to evaluate the inter- and intraobserver variation in the assessment by pediatric surgeons of preoperative colostograms with respect to the level of the recto-urinary fistula. Materials and Methods: Sixteen pediatric surgeons from 14 European centers belonging to the ARM-Net Consortium twice scored 130 images of distal colostograms taken in sagittal projection at a median age of 66 days of life (range: 4-1,106 days). Surgeons were asked to classify the fistula in bulbar, prostatic, bladder-neck, no fistula, and "unclear anatomy" example. Their assessments were compared with the intraoperative findings (kappa) for two scoring rounds with an interval of 6 months (intraobserver variation). Agreement among the surgeons' scores (interobserver variation) was also calculated using Krippendorff's alpha. A kappa over 0.75 is considered excellent, between 0.40 and 0.75 fair to good, and below 0.40 poor. Surgeons were asked to score the images in "poor" and "good" quality and to provide their years of experience in ARM treatment. Results: Agreement between the image-based rating of surgeons and the intraoperative findings ranges from 0.06 to 0.45 (mean 0.31). Interobserver variation is higher (Krippendorff's alpha between 0.40 and 0.45). Years of experience in ARM treatment does not seem to influence the scoring. The mean intraobserver variation between the two rounds is 0.64. Overall, the quality of the images is considered poor. Images categorized as having a good quality result in a statistically significant higher kappa (mean: 0.36 and 0.37 in the first and second round, respectively) than in the group of bad-quality images (mean: 0.25 and 0.23, respectively). Conclusions: There is poor agreement among experienced pediatric colorectal surgeons on preoperative colostograms. Techniques and analyses of images need to be improved in order to generate a homogeneous series of patients and make comparison of outcomes reliable.

Thoracoscopic Bullectomy for Persistent Air Leak in a 14-Year-Old Child with COVID-19 Bilateral Pulmonary Disease.

Introduction: Thoracic surgery in children with coronavirus disease-19 (COVID-19) pulmonary disease is rare, as very limited virus-related lung lesions require intervention. However, some patients may suffer from other pulmonary abnormalities that can be worsened by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and they may consequently require lung surgery. COVID-19 affects the indications, surgical procedure, and postsurgical care of these patients. Background: We present a case of a 14-year-old girl with COVID-19 pulmonary disease and persistent air leak due to right apical bullae that required resection. Clinical, surgical, and safety implications are discussed. The role of thoracic minimally invasive surgery under COVID-19 conditions is also analyzed. Materials and Methods: The thoracoscopic procedure was scheduled earlier than normally expected. The surgery was performed in a COVID-19 reserved theatre with neutral pressure and only the necessary personnel was allowed inside. The use of the required personal protective equipment was supervised by an expert nurse before and after the intervention. Results: The surgeons used a three-port technique to resect the bullae with an endostapler and no mechanical pleural abrasion was added to the procedure. Electrocautery and CO2 insufflation were avoided, and a chest drain with a closed-circuit aspiration system was installed before removing the ports. The child was discharged home 3 days later after the removal of the chest drain. Conclusions: COVID-19 has an impact on the standard indications, surgical strategies and postoperative care of some conditions requiring intervention. Extra safety measures are needed in the operating room to limit the chance of transmission. Minimally invasive surgery for thoracic surgery remains safe if the current safety guidelines are followed closely.

Anorectal malformations and perineal hemangiomas: The Arm-Net Consortium experience.

AIM: Perineal hemangiomas rarely occur in patients with anorectal malformations (ARMs), but they can pose a significant challenge and warrant special attention. Surgical incision of posterior sagittal anorectoplasty (PSARP) may involve the hemangioma site resulting in hemorrhage, damage to blood supply, leading to complications and adversely affecting outcome. The aim of this study was to review the experience of the ARM-Net Consortium in the management of perineal hemangioma associated with ARM and evaluate treatment strategies. MATERIALS AND METHODS: Data on all patients with ARM and a perineal hemangioma located in the planes of the PSARP dissection who were managed at participating ARM-Net centers were collected retrospectively by questionnaire, as follows: ARM type, hemangioma distribution and penetration, imaging findings, medical/surgical management, timing of definitive repair, complications and outcome. RESULTS: Ten patients from eight centers were included. Three patients each had a rectobulbar or rectovestibular fistula, 2 had a rectoperineal fistula, and one had a rectoprostatic fistula; in one patient, the hemangioma was too disfiguring to determine malformation type. Mean follow-up time was 36.6 months (median 29 months). Colostomies were performed before definitive repair in 8 patients. Five patients received systemic beta-blockers before PSARP: 3 were operated uneventfully following partial/complete involution of the hemangioma, and 2 are awaiting surgery. The two patients with rectoperineal fistula were managed expectantly. The remaining 3 patients underwent surgery with no preoperative medical treatment, and all had complications: mislocated neoanus in three and complete perineal dehiscence in one. CONCLUSION: Attempting PSARP in the presence of a perineal hemangioma may lead to complications and adversely affect outcome. This study confirms the benefits of beta blocker treatment before surgical reconstruction. LEVEL OF EVIDENCE: Treatment study, level III.

New Methods for Imaging Evaluation of Chest Wall Deformities.

AIM: The purpose of this study is to describe the development of an external 3-dimensional (3D) scanner as a noninvasive method for imaging chest wall deformities. It allows objective assessment, reconstruction of the area of interest, and evaluation of the severity of the deformity by using external indexes. EXTERNAL 3D SCANNING SYSTEM: The OrtenBodyOne scanner (Orten, Lyon, France) uses depth sensors to scan the entire 3D external body surface of a patient. The depth sensors combine structured light with two classic computer vision techniques: depth from focus and depth from stereo. The data acquired are processed and analyzed using the Orten-Clinic software. MATERIALS AND METHODS: To investigate the performance of the device, a preliminary prospective study (January 2015-March 2016) was carried out in patients attending our hospital chest wall deformities unit. In total, 100 patients (children and young adults) with pectus excavatum or pectus carinatum, treated by surgery or non-operative methods were included. In patients undergoing non-operative treatment, external 3D scanning was performed monthly until complete correction was achieved. In surgically treated patients, scanning was done before and after surgical correction. In 42 patients, computed tomography (CT) was additionally performed and correlations between the Haller index calculated by CT and the external Haller index using external scanning were investigated using a Student's test (r = 0.83). CONCLUSION: External scanning is an effective, objective, radiation-free means to diagnose and follow-up patients with chest wall deformities. Externally measured indexes can be used to evaluate the severity of these conditions and the treatment outcomes.

Two-Port Laparoscopic Descending Colostomy with Separated Stomas for Anorectal Malformations in Newborns.

Introduction We describe a two-port laparoscopic technique to create a colostomy in the descending colon with separated stomas for newborns with anorectal malformations. Material and Methods Six patients with an anorectal malformation underwent this procedure in the early-neonatal period. The surgical technique was performed with two ports, which allows for an accurate inspection of the abdominal contents. The first loop of the sigmoid colon is grasped through the first port and exteriorized while the attachments to the left retroperitoneum and direction of the loop are checked with the scope introduced in the second port. The division of the colon is performed extracorporally, the colon irrigated of meconium, and the distal colon moved to the second port incision. Both stomas are then fixed to the abdominal wall. Results The time of the procedure ranged from 50 to 90 minutes. A Mullerian duplication was noted in one case. Oral intake was started during the first 12 to 24 hours. No complications were seen during or after the procedure. Conclusions This technique allows for the precise localization of the colostomy with direct visualization, provides for the inspection of the internal genitalia, eliminates the incision between the two stomas and its complications, allows for painless stoma bag changes immediately after surgery, avoids twisting of the colostomy, and permits a cosmetically pleasing incision at the colostomy closure.

Duodenal lengthening in short bowel with dilated duodenum.

UNLABELLED: Although duodenal dilatation occurs in children with short bowel syndrome (SBS) facilitating dismotility and bacterial overgrowth, the duodenum has been an untouchable intestinal segment for lengthening procedures owing to its close relationship with bilio-pancreatic structures and blood supply shared with the pancreas. Three children (age range, 0.5-7 years) with SBS and dilated duodenum underwent a novel surgical procedure of duodenal lengthening combined with a technical modification of serial transverse enteroplasty (STEP). Pre-STEP, jejunum length was 5, 35 and 45cm, respectively. Duodenal lengthening was performed with sequential transverse applications of an endoscopic stapler on the anterior and posterior wall of the duodenum to avoid bilio-pancreatic structure injury. Two patients underwent 3 duodenal firings (stapler of 35mm) and the third 5 firings (stapler of 45mm). Duodenal firings were 17%, 21% and 83% of the total firings. RESULTS: No surgical complications occurred. One patient developed transient episodes of D-lactic acidosis. Two patients (5 and 45cm) were weaned off parenteral nutrition at 12months post-surgery and the remaining patient´s (35cm) parenteral calorie requirements have decreased by 60%. CONCLUSION: Duodenal lengthening is effective since it tailors and increases the absorptive surface of the duodenum, even in cases of extreme SBS.