Laparoscopic appendectomy with single port vs conventional access: systematic review and meta-analysis of randomized clinical trials.

BACKGROUND: Conventional three-access laparoscopic appendectomy (CLA) is currently the gold standard treatment, however, Single-Port Laparoscopic Appendectomy (SILA) has been proposed as an alternative. The aim of this systematic review/meta-analysis was to evaluate safety and efficacy of SILA compared with conventional approach. METHODS: Per PRISMA guidelines, we systematically reviewed randomised controlled trials (RCTs) comparing CLA vs SILA for acute appendicitis. The randomised Mantel-Haenszel method was used for the meta-analysis. Statistical data analysis was performed with the Review Manager software and the risk of bias was assessed with the Cochrane "Risk of Bias" assessment tool. RESULTS: Twenty-one studies (RCTs) were selected (2646 patients). The operative time was significantly longer in the SILA group (MD = 7,32), confirmed in both paediatric (MD = 9,80), (Q = 1,47) and adult subgroups (MD = 5,92), (Q = 55,85). Overall postoperative morbidity was higher in patients who underwent SILA, but the result was not statistically significant. In SILA group were assessed shorter hospital stays, fewer wound infections and higher conversion rate, but the results were not statistically significant. Meta-analysis was not performed about cosmetics of skin scars and postoperative pain because different scales were used in each study. CONCLUSIONS: This analysis show that SILA, although associated with fewer postoperative wound infection, has a significantly longer operative time. Furthermore, the risk of postoperative general complications is still present. Further studies will be required to analyse outcomes related to postoperative pain and the cosmetics of the surgical scar.

Intestinal Bowel Lengthening within the First 6 Months of Life: Institutional Experience and Review of the Literature.

Background: Management of short bowel syndrome in children has been surrounded by much debate with timing of the lengthening procedure still controversial. Early bowel lengthening procedure (EBLP) has been defined as any bowel lengthening procedure performed before 6 months of age. The purpose of this paper is to report the institutional experience in EBLP and to review the literature on this subject to identify common indications. Methods: An institutional retrospective analysis of all the intestinal lengthening procedures was performed. Furthermore, an Ovid/Embase search regarding children who underwent bowel lengthening in the past 38 years was conducted. Primary diagnosis, age at procedure, type of procedure, indication, and outcome were analyzed. Results: Ten EBLP were performed in Manchester from 2006 to 2017. Median age at surgery was 121 days (102-140), preoperative small bowel (SB) length was 30 cm (20-49) while postoperative SB length was 54 cm (40-70), with a median increased bowel length of 80%. Ninety-seven papers were reviewed, with more than 399 lengthening procedures performed. Twenty-nine papers matched criteria with more than 60 EBLP were observed of which 10 were performed in a single center from 2006 to 2017. EBLP was performed due to SB atresia, to excessive bowel dilatation or failure to enteral feeds, at a median age of 60 days (1-90). Serial transverse enteroplasty was the most frequent procedure used lengthening the bowel from 40 cm (29-62.5) to 63 cm (49-85), with a median increased bowel length of 57%. Conclusions: This study confirms that no clear consensus on indication or timing to perform early SB lengthening is reported. According to the gathered data, EBLP should be considered, only in cases of actual necessity after review of qualified intestinal failure center.

Let's (Not) Twist Again: Laparoscopic-Assisted Percutaneous Endoscopic Gastrostomy in Paediatric Gastric Volvulus.

INTRODUCTION: In the setting of intermittent abdominal pain accompanied by gastro-intestinal obstruction, gastric volvulus should be suspected. AREAS COVERED: A 10-year-old girl was admitted to our hospital for intermitted abdominal pain associated with recurrent non-bilious vomiting and acute weight loss. The radiological images performed at the district general hospital were reported as negative. After careful review of the imaging, a gastric volvulus was suspected. Exploratory laparoscopy confirmed the diagnosis; laparoscopic-assisted percutaneous endoscopic gastrostomy was performed. The postoperative upper gastro-intestinal study showed stomach in its normal shape and position. EXPERT COMMENTARY: Gastric volvulus is a potential life-threatening condition with a good outcome when promptly recognized. Laparoscopic-assisted percutaneous endoscopic gastrostomy is a safe and curative procedure in the case of suspected gastric volvulus, avoiding the need for a secondary procedure. In pediatric patients, it facilitates direct inspection of the stomach and abdominal cavity, reducing the need for a second procedure. This procedure may emerge as a 'gold standard' for gastric volvulus treatment.

Lrig2 and Hpse2, mutated in urofacial syndrome, pattern nerves in the urinary bladder.

Mutations in leucine-rich-repeats and immunoglobulin-like-domains 2 (LRIG2) or in heparanase 2 (HPSE2) cause urofacial syndrome, a devastating autosomal recessive disease of functional bladder outlet obstruction. It has been speculated that urofacial syndrome has a neural basis, but it is unknown whether defects in urinary bladder innervation are present. We hypothesized that urofacial syndrome features a peripheral neuropathy of the bladder. Mice with homozygous targeted Lrig2 mutations had urinary defects resembling those found in urofacial syndrome. There was no anatomical blockage of the outflow tract, consistent with a functional bladder outlet obstruction. Transcriptome analysis revealed differential expression of 12 known transcripts in addition to Lrig2, including 8 with established roles in neurobiology. Mice with homozygous mutations in either Lrig2 or Hpse2 had increased nerve density within the body of the urinary bladder and decreased nerve density around the urinary outflow tract. In a sample of 155 children with chronic kidney disease and urinary symptoms, we discovered novel homozygous missense LRIG2 variants that were predicted to be pathogenic in 2 individuals with non-syndromic bladder outlet obstruction. These observations provide evidence that a peripheral neuropathy is central to the pathobiology of functional bladder outlet obstruction in urofacial syndrome, and emphasize the importance of LRIG2 and heparanase 2 for nerve patterning in the urinary tract.

Combined procedures for surgical short bowel syndrome: experience from two European centres.

BACKGROUND: Autologous gastrointestinal reconstructive surgery (AGIR) has become a key component of intestinal rehabilitation programs. However, the best surgical option for short bowel syndrome (SBS) remains unknown. This paper presents our experience using combined procedures as primary treatment. METHODS: We collected data on SBS patients who underwent surgery from 2008 to 2021 in two tertiary European Centres. Combined procedures were defined as more than one technique used on the same patient. Charts were reviewed for demographics, type of procedures, complications, and outcomes. Data are presented as median and IQR. Wilcoxon signed rank was used for all paired analyses. RESULTS: Twenty-one children (12 females) underwent combined procedures. Preoperative median small bowel length was 20 cm (IQR: 15-35 cm); after lengthening, it was 35.5 cm (IQR: 30.75-50.50 cm) (P < 0.001). Combined procedures were simultaneous in 15 patients and sequential in 6. At a median of 9.2 years (IQR: 7.55-9.78 years) follow-up, complications were three bowel obstructions after strictures of anastomosis and two wound infections. Two patients achieved enteral autonomy, and others followed a weaning home parenteral nutrition regimen with a median of 4 nights off (IQR: 3-4 nights) starting with a median of 7 nights (IQR: 7-7 nights). CONCLUSIONS: Combined AGIR techniques are practical and safe in SBS treatment when tailored to meet patients' needs, combining lengthening, tailoring, and reducing transit time procedures. Therefore, combined AGIR may be considered a resource in intestinal rehabilitation units' armamentarium.

Intestinal twin-to-twin transplant for short gut: Review of the literature and discussion of a complex case.

Paediatric Intestinal Transplantation (IT) presents the highest mortality on the waiting-list due to anatomical disproportion. Living-Donor IT (LDIT) offers the best advantages and when performed among identical monozygotic twins, it also benefits from unique immunology. According to MEDLINE/Pubmed, twin-to-twin LDIT has been performed in seven cases (6:7 males, median age of 32 years). None of the patients received immunosuppression postoperatively. Only one paediatric twinto- twin LDIT was carried out with a 160-cm mid-ileum tract: an interposed 4/5-cm arterial graft was required to ensure a tensionfree anastomosis to the anterior wall of the infra-renal abdominal aorta. In contrast, venous anastomosis was done directly to the inferior cava vein. We present a case for debate of a 13- month-old SBS patient where a twin-LDIT was discussed with parents, who decided to wait after careful analysis and ethical considerations.

Total Esophagogastric and Cologastric Dissociation in Neurologically Normal Children: Systematic Review.

Total esophagogastric dissociation (TEGD) was first described by Bianchi as a definitive procedure for gastroesophageal reflux disease (GERD) in neurologically impaired children. In the last 20 years, different centers extended the indication to neurologically normal (NN) patients with GERD associated with congenital or acquired esophageal anomalies. The aim of this paper is to analyze the role of TEGD in this cluster of patients. A PubMed and Google Scholar search was conducted. All cases of NN children who underwent TEGD for GERD were collected. Patient characteristics and outcomes were analyzed. Complications were classified according to Clavien-Dindo classification. Forty-eight children were identified. In 56.25%, TEGD was the first anti-reflux procedure, while in 43.75% it was performed after failed fundoplications. Mean follow-up was 5.5 years. Mortality related to surgery was 2.08%. All of the survivors improved their condition, with resolution of GERD and weight gain. In addition, 50% of children weaned off enteral nutrition, with 14.6% having their gastrostomy removed, while 41.67% maintained partial enteral supplementation. Respiratory symptoms almost disappeared in 54.17% of patients. This review suggests that TEGD can also be considered for NN children where conventional methods seem insufficient to control reflux and preserve pulmonary function. Nevertheless, long-term follow-up is still required.

Difficult Vascular Access in Children with Short Bowel Syndrome: What to Do Next?

Short Bowel Syndrome and intestinal failure are chronic and severe conditions that may require life-long parenteral nutrition in children. Survival of these children rely on the correct functioning of central venous catheters; therefore, careful management, prevention, and treatment of complications is of paramount importance. Despite a growing awareness of preserving the vascular real estate, a certain number of patients still experience a progressive and life-threatening exhaustion of vascular access. We searched the literature to highlight the current management of children with vascular exhaustion, specifically focusing on vascular access salvage strategies and last-resource alternative routes to central veins. Given the paucity of data, results are reported in the form of a narrative review.

Minimizing Enterostomy Complication in Neonates, Lessons Learnt from Three European Tertiary Centres.

INTRODUCTION: Stoma formation in neonates is often a life-saving procedure across a variety of conditions but is still associated with significant morbidity. Tube stoma technique was originally described for short bowel patients, but in selected cases of neonates this approach could prevent the incidence of stoma-related complications. The aim of the study was to evaluate the safety and utility of tube stomas as an alternative to conventional enterostomy in the neonatal population. MATERIAL AND METHODS: A retrospective multicentre analysis of neonates undergoing emergency laparotomy and tube stoma formation between 2005 and 2017 was performed. Tube stoma complications were analysed. The investigation focused on stricture, skin lesion, enteric fistula and prolapse. RESULTS: Thirty-seven neonates underwent tube stoma fashioning during the study period. Tube-stoma complications were limited to three patients (8.1%), with two children (5.4%) requiring additional stoma surgery during the first 30 days because of an enterocutaneous fistula, and one child (2.7%) for bowel stenosis. CONCLUSIONS: In select neonates, such as those with proximal enteric stomas, the tube stoma avoids some of the commonly encountered complications (prolapse, skin excoriation). Further prospective studies are needed to validate these findings in order for us to recommend this technique as superior.

Early Bowel Lengthening Procedures: Bi-Institutional Experience and Review of the Literature.

Early bowel lengthening procedure (EBLP) has been defined as any bowel lengthening procedure performed before six months of age. The purpose of this paper is to compare our experience with literature on this subject to identify common indications. A bi-institutional retrospective analysis was performed. Diagnosis, type of surgery, age at procedure and outcomes were analysed. Eleven EBLP were performed in Manchester and Florence from 2006 to 2021. The median age at surgery was 126 days (102-180), pre-operative median short bowel (SB) length was 28 cm (17-49) with a post-operative median increase of 81%. Furthermore, a PubMed/Embase search was undertaken regarding bowel lengthening procedures performed in the last 40 years. Sixty-one EBLP were identified. The median age was 60 days (1-90). Serial transverse enteroplasty (STEP) was the most frequent procedure used, with a median increased bowel length of 57%. This study confirms that no clear consensus on indication or timing to perform early SB lengthening is reported. According to the gathered data, EBLP should be considered only in cases of actual necessity and performed in a qualified intestinal failure centre.

Uniportal Video-Assisted Thoracic Surgery in a Pediatric Hospital: Early Results and Review of the Literature.

Background: Uniportal video-assisted thoracic surgery (U-VATS) is an implemented technique in adult surgery that may aid to extend offer the benefits of thoracoscopy to a wide number of pediatric patients. Materials and Methods: Consecutive cases treated between July 2019 and July 2021 were retrospectively analyzed. Simultaneously, a MEDLINE systematic search was conducted. Results: Twelve patients (median age 13 years, median weight 44.5 kg) underwent 4 major procedures (n = 2 lobectomy, n = 2 segmentectomy) and 11 minor procedures (n = 1 bronchogenic cyst resection, n = 4 apical wedge resections and pleurodesis for pneumothorax, n = 4 wedge resections for lung nodules, and n = 2 debridement for empyema). The median observed operative time was 77 minutes. We recorded one conversion to biportal VATS. No intraoperative complications or 30-day morbidity-mortality was reported. A rate of 40% adverse postoperative events was observed (Clavien-Dindo grade I-IVa). Visual analog scale for postoperative pain recorded a median value of 0 on days 1, 2, and 3. The systematic review provided 15 full-text articles reporting 76 pediatric interventions (4 major and 72 minor procedures); among them, 1 biportal conversion, 3 mild postoperative complications, and 1 redo surgery are presented. Conclusions: As emerged from the literature review, U-VATS remains scarcely adopted by pediatric surgeons. Its feasibility is supported by the four reported major lung resections plus the four cases added on by our series. Thanks to a more rapid learning curve over conventional VATS, the uniportal technique could be accessible to a wider number of centers.

Treatment of Food Aversion and Eating Problems in Children with Short Bowel Syndrome: A Systematic Review.

Food Aversion (FA) is a strong refusing behaviour to the oral assumption of food that can affect children with Short Bowel Syndrome (SBS). Management includes behavioural and Messy Play treatments, with few reports on systematic strategies to return the patient to enjoyable eating. We conducted a systematic review to better understand this complex and vital issue. (1) Materials and Methods: We investigated publications using MEDLINE, Embase, and the Web of Science to include articles published up to July 2022. The inclusion criteria were original articles including paediatric patients (aged < 18 years old) affected by SBS and Intestinal Failure (IF) who underwent treatment for FA. (2) Results: A total of 24 patients received treatment­15 (62.5%) patients were male and 9 (37.5%) were female. The age range was from 1 month to 16 years. Treatment of FA was carried out by behavioural therapy in 2 patients and Messy Play Therapy in 12 patients already surgically and pharmacologically managed for SBS. The treatment results showed complete weaning from Parenteral Nutrition in 9/14 cases (64%) using the behavioural treatment and 7/12 cases using Messy Play Therapy. (3) Conclusions: FA is a rare but disabling condition that often affects SBS patients, worsening their overall health and quality of life. This condition should be addressed in an Intestinal Rehabilitation Centre context. Our review sheds light on the literature gap regarding FA, and further studies are required to understand better which treatment options best suit SBS paediatric patients.

Use of Teduglutide in Children With Intestinal Failure: A Systematic Review.

Background and Objectives: Short-bowel syndrome (SBS) results from the loss of a significant portion of the small intestine leading to a state of malabsorption. After an intestinal loss, there is a process of adaptation involving the Glucagon-Like Peptide-2 (GLP-2), an enteroendocrine peptide also involved in nutrient absorption. Teduglutide is a recombinant analog of GLP-2 approved in 2016 to treat selected SBS pediatric patients who are dependent on parenteral support. The present systematic review aims to evaluate the efficacy of Teduglutide in pediatric patients with SBS in reducing the need for parenteral nutrition (PN). Materials and Methods: We performed a literature search on MEDLINE and Embase to include articles up to November 2021. We included articles that involved using Teduglutide in the SBS pediatric population to define its efficacy in reducing the need for PN. The key words used were GLP-2, teduglutide, child. Results: Fourteen studies completely fulfilled the inclusion criteria. Two hundred 23 patients were treated with Teduglutide, and the median duration of treatment was 45 weeks (IQR: 36-52.5 weeks). One-hundred and fifty-two patients were treated with 0.05 mg/Kg/d of subcutaneous Teduglutide, 38 received 0.025 mg/Kg/d and 8 received either 0.125 mg/Kg/d or 0.20 mg/Kg/d. A total of 36 patients achieved enteral autonomy (EA) after a median of 24 weeks of treatment (IQR: 24-48 weeks) and 149 patients showed a reduction in PN needs in terms of volume, calories, or hours per day. Eleven studies reported complications: gastrointestinal were the most common, with 89 cases reported in treated patients and 11 in non-treated patients. Conclusion: Teduglutide appears safe and effective in reducing PN requirements and improving EA in the pediatric population. However, more studies are needed to understand its efficacy in the long term and after discontinuation and possible complications. Systematic Review Registration: [https://www.crd.york.ac.uk/prospero/], identifier [CRD42022301593].

A Unique Case of Primary Cutaneous Adenoid Cystic Carcinoma Associated with Aplasia Cutis Congenita in a Four-Year-Old Female: A Case Report.

INTRODUCTION: Primary cutaneous adenoid-cystic carcinoma (PCACC) is a rare malignant tumour reported in only about 450 cases in the literature, with only two adolescent cases reported. PCACC seems to occur between the fifth and seventh decade of life, and the most frequent regions involved are head and neck (46%). Aplasia cutis congenita (ACC) has an incidence of 1:10,000, and it seems to be rarely associated with neoplastic lesions. Interestingly, the association between PCACC and ACC has, so far, never been described. METHODS: We report a case of PCACC in the scalp associated with ACC in a four-year-old patient. DISCUSSION: The patient was under follow-up at the dermatology unit, but suddenly a red lesion appeared within the ACC. This red, ulcerated area increased rapidly over six months, so it was surgically removed, and the pathological examination results were suggestive for cribriform PCACC. According to the guidelines for skin tumours, the patient underwent widening resection, and an advancement-sliding skin flap was performed to recreate the scalp. After one year of follow-up, the patient has no local or widespread recurrence of the PCACC, and the surgical scar appears to have healed well. CONCLUSIONS: This clinical case is the first known patient with PCACC associated with ACC. A skin excision biopsy should be performed with wide margins to avoid a second widening resection of skin in a similar scenario. Genetic studies may help to identify the origin of this rare association.

Engineering Auxetic Cylinders and Intestine to Improve Longitudinal Intestinal Lengthening and Tailoring Procedure.

Auxetic materials can be exploited for coupling different types of tissues. Herein, we designed a material where the microorganism metabolic activity yields the formation of buckled/collapsed bubbles within gelling silicone cylinders thus providing auxetic properties. The finite element model of such hollow auxetic cylinders demonstrated the tubular structure to promote worm-like peristalsis. In this scenario, the described hybrid auxetic structures may be applied to the longitudinal intestinal lengthening and tailoring procedure to promote enteral autonomy in short bowel syndrome. The presented material and analytical design synergistic approach offer a pioneering step for the clinical translation of hybrid auxetic materials.

3D Printing Silk-Based Bioresorbable Piezoelectric Self-Adhesive Holey Structures for In Vivo Monitoring on Soft Tissues.

Flexible and biocompatible adhesives with sensing capabilities can be integrated onto human body and organ surfaces, characterized by complex geometries, thus having the potential to sense their physiological stimuli offering monitoring and diagnosis of a wide spectrum of diseases. The challenges in this innovative field are the following: (i) the coupling method between the smart adhesive and the soft human substrates, (ii) the bioresorbable behavior of the material, and (iii) the electrical exchange with the substrate. Here, we introduce a multifunctional composite by mixing silk fibroin, featuring piezoelectric properties, with a soluble plant-derived polyphenol (i.e., chestnut tannin) modified with graphene nanoplatelets. This material behaves as a glue on different substrates and gives rise to high elongation at break, conformability, and adhesive performances to gastrointestinal tissues in a rat model and favors the printability via extrusion-based 3D printing. Exploiting these properties, we designed a bioresorbable 3D printed flexible and self-adhesive piezoelectric device that senses the motility once applied onto a phantom intestine and the hand gesture by signal translation. Experimental results also include the biocompatibility study using gastrointestinal cells. These findings could have applicability in animal model studies, and, thanks to the bioresorbable behavior of the materials, such an adhesive device could be used for monitoring the motility of the gastrointestinal tract and for the diagnosis of motility disorders.

Modified Oesophago-Gastric Dissociation (M-OGD) - a technical modification.

Adhesions and fibrosis following failed primary surgery for severe gastro-oesophageal reflux (GOR) in neurologically impaired children (NI) can render mobilization of the lower oesophagus and oesophago-jejunal anastomosis a technically demanding exercise both at open surgery and laparoscopy. This paper presents the Modified Oesophago-Gastric Dissociation (M-OGD) as a less complex technical modification of the original Total Oesophago-Gastric Dissociation (TOGD). The stomach is detached from the oesophago-gastric junction with an articulated 5-mm stapler, leaving a 5-mm strip of stomach attached to the oesophagus. An end-to-side isoperistaltic oesophago-jejunostomy is created between the gastric stump and the isoperistaltic jejunal Roux loop. A jejuno-jejunal anastomosis restores bowel continuity. Between May 2018 and February 2020, M-OGD was performed on 3 NI patients with a weight of 9-27.3 kg (median = 14 kg). Median age at surgery was 60 months (18-180), median surgical time 170 min (146-280), median re-feeding time was 3 days (2-5), and median length of stay was 20 days (11-25). All patients healed primarily and after a median follow-up of 3 months, there were no problems related to the oesophago-jejunal anastomosis. M-OGD reduces the difficulties of redo oesophageal surgery following failed anti-reflux procedures, with a safer oesophago-jejunal anastomosis and a good long-term outcome.