Improving care and survival of newborns with surgical conditions in Tanzania (TINY Tanzania): a focus on gastroschisis.

PURPOSE: Gastroschisis is associated with over 90% mortality in many sub-Saharan African countries. The introduction of the Gastroschisis Care Bundle at Muhimbili National Hospital (MNH) increased survival up to 60%. We aim to explain the impact of using implementation science methods to decentralize the care of babies with gastroschisis to other parts of Tanzania. METHODS: We used a Step-Wedge Implementation Science design to scale up gastroschisis care through training of providers, dissemination and current revision of evidence-based care protocols, advocacy, and engagement with stakeholders. We used mixed methods for data collection. Anonymous patient and provider evaluation data were collected using a nationwide Gastroschisis Database via REDCap. We evaluated the implementation and effectiveness of the care bundle in different hospitals in Tanzania. RESULTS: Decentralizing care nationally was feasible, acceptable, and adaptable. A total of nine trainings have been conducted training 420 providers (14 Master Trainers) reaching seven regions of Tanzania. The three advocacy national campaigns have ensured community reach and patient engagement. A countrywide gastroschisis database was developed to collect data on patients with gastroschisis, hosted locally at MNH with 332 patients' data entered in 1 year. The majority (90.2%) were treated using preformed silo bags with an overall survival of 28.5% in all centers. Late presentation and infection remain to be the main challenge. CONCLUSION: To achieve quality and sustainable surgical care, there is a need to design, implement, evaluate, and continuously improve context-relevant strategies to achieve and sustain the survival of neonates with congenital anomalies. Decentralization enables clear connectedness of hospitals, bringing care closer to patients.

Improving outcomes for uncomplicated gastroschisis: clinical practice guidelines from the American Pediatric Surgical Association Outcomes and Evidence-based Practice Committee.

BACKGROUND: The authors sought better outcomes for uncomplicated gastroschisis through development of clinical practice guidelines. METHODS: The authors and the American Pediatric Surgical Association Outcomes and Evidenced-based Practice Committee used an iterative process and chose two questions to develop clinical practice guidelines regarding (1) standardized nutrition protocols and (2) postnatal management strategies. An English language search of PubMed, MEDLINE, OVID, SCOPUS, and the Cochrane Library Database identified literature published between January 1, 1970, and December 31, 2019, with snowballing to 2022. The Appraisal of Guideline, Research and Evaluation reporting checklist was followed. RESULTS: Thirty-three studies were included with a Level of Evidence that ranged from 2 to 5 and recommendation Grades B-D. Nine evaluated standardized nutrition protocols and 24 examined postnatal management strategies. The adherence to gastroschisis-specific nutrition protocols promotes intestinal feeding and reduces TPN administration. The implementation of a standardized postnatal clinical management protocol is often significantly associated with shorter hospital stays, less mechanical ventilation use, and fewer infections. CONCLUSIONS: There is a lack of comparative studies to guide practice changes that improve uncomplicated gastroschisis outcomes. The implementation of gastroschisis-specific feeding and clinical care protocols is recommended. Feeding protocols often significantly reduce TPN administration, although the length of hospital stay may not consistently decrease.

Congenital Hernia of the Umbilical Cord: A Retrospective Case Study.

Background: Congenital hernia of the umbilical cord (CHUC) is the rarest type of anterior abdominal wall defect, in which an intact umbilical ring is always present and viscera pass through the base of normal-looking umbilicus. Objectives: This study was conducted to document the intraoperative findings and postoperative outcomes of patients with congenital hernia of the umbilical cord up to discharge from a tertiary care center. Methods: This study was a retrospective observational study conducted for two years (August 2020 to July 2022) in the Department of Pediatric Surgery, at the tertiary health care center of UP, India. Results: During this two-year duration, a total of 10 cases with CHUC were seen in our department and were surgically managed. In this study, out of these 10 patients (male 7 and female 3), eight had normal gastrointestinal tract, one had accessory liver tissue on thin pedicle, and one had features of gangrenous bowel. Of these 10 cases, three patients developed postsurgical complications in which two patients developed superficial wound infection while one developed wound dehiscence. No mortality was noted. Conclusions: Congenital hernia of the umbilical cord induces stress on parents and relatives. In this study, we conclude that the majority of cases had normal gastrointestinal tract and had no serious postoperative complications up to discharge.

Long-term gastrointestinal morbidity in patients born with gastroschisis: A national register-based cohort study.

OBJECTIVES: In gastroschisis, the intestines are exposed to amniotic fluid during pregnancy. The defect in the abdominal wall may also compress the mesentery and impair the intestinal blood supply. There is a varying degree of intestinal damage at birth. Complex gastroschisis is defined as gastroschisis with severe complications such as intestinal atresia, volvulus, necrosis and perforation. The aim of this study was to investigate long-term gastrointestinal morbidity and compare simple and complex cases. METHODS: A nation-wide retrospective cohort study with data from national registers was conducted. All children born with gastroschisis in Sweden from 1 January 1997 to 31 December 2016 were included in the study. Exposure was complex gastroschisis and the primary outcomes were intestinal failure and bowel obstruction. RESULTS: The study included 315 cases with gastroschisis, 260 classifieds as simple gastroschisis and 55 as complex. The median time to follow was 8 years. A significantly higher risk of developing intestinal failure (hazard ratio: 11.7) was found in complex cases. Nine percent of the complex cases underwent autologous gastrointestinal reconstructive surgery for intestinal failure, none of the simple cases did. The complex cases had a higher risk for bowel obstruction (hazard ratio: 4.3) with a higher proportion requiring surgery (18.2% vs. 6.9%) compared to simple cases. CONCLUSIONS: This nationwide study showed that the risk for intestinal failure and bowel obstruction is significantly higher for children with complex gastroschisis compared to simple gastroschisis. Most of the events occurred during the first 2 years of life.

BODY STALK ANOMALY: CLINICAL AND HISTOPATHOLOGIC FINDINGS OF THIS RARE ANOMALY IN A NIGERIAN NEWBORN.

Introduction: Body stalk anomaly (BSA) is a rare and severe congenital malformation in which the exact pathophysiology is still unknown. The possible causes of body stalk anomaly include early amnion rupture with direct mechanical pressure and amniotic bands, vascular disruption of the early embryo, or an abnormality in the germinal disk. Case presentation: We report a case of sonologically delayed diagnosis of BSA which was confirmed post-delivery following histopathological examination and we reviewed relevant literature regarding this phenomenon. Sonographic features of the foetus included a wide anterior abdominal wall defect (omphalocele) with protrusion of the liver into the amniotic cavity. The umbilical arteries show normal calibre, flow, velocimetry, and spectral waveform. Conclusion: Body stalk anomaly is accepted as a fatal anomaly, so it is important to differentiate it from other anterior abdominal wall defects prenatally and this could guide the management options.

Unusual Presentation of Beckwith-Wiedemann Syndrome in an Extremely Low Birth Weight Infant.

Beckwith-Wiedemann syndrome (BWS) is a genetic disorder that affects fetal growth in which those afflicted present with features pertaining to that, such as macrosomia, macroglossia, hemihypertrophy, and abdominal wall defects. This case reports the presentation of an infant diagnosed with BWS who was born with an extremely low birth weight of 980 grams, in contrast to the typical presentation of overgrowth and macrosomia. As a result, reaching a diagnosis of BWS was delayed until the patient reached eight months of age, when other clinical features of BWS, such as hemihypertrophy, became apparent on follow-up visits. Although genetic testing can be used to diagnose this condition, a clinical scoring system consisting of a patient's clinical features is sufficient, allowing for a timely and precise diagnosis, which is of great significance to allow for early screening and detection of the associated embryonal tumors with such a syndrome.

Prune-Belly Syndrome: An Update.

The Prune-Belly (Eagle-Barrett) syndrome (PBS) is a congenital and genetically heterogeneous disease, more prevalent in males, defined by the clinical triad (1) deficiency of abdominal muscles, (2) bilateral cryptorchidism, and (3) urinary tract abnormalities. The abdomen of an infant with PBS has a typical appearance, similar to the aspect of a prune, which gives it its name. Although the etiology of this disorder is still unknown, numerous theories, mutations, and genetic disturbances have been proposed to explain the origin of PBS. Prognosis can differ a lot from one patient to another, since this condition has a wide spectrum of clinical presentation. Despite being a rare condition, the importance of PBS should not be underestimated, in the light of the potential of the disorder to lead to chronic kidney disease and other severe complications. In that regard, this review gathers the most up-to-date knowledge about the etiopathogenesis, clinical features, diagnosis, management and prognosis of PBS.

Management of Gastroschisis: Timing of Delivery, Antibiotic Usage, and Closure Considerations (A Systematic Review From the American Pediatric Surgical Association Outcomes & Evidence Based Practice Committee).

BACKGROUND: No consensus exists for the initial management of infants with gastroschisis. METHODS: The American Pediatric Surgical Association (APSA) Outcomes and Evidenced-based Practice Committee (OEBPC) developed three a priori questions about gastroschisis for a qualitative systematic review. We reviewed English-language publications between January 1, 1970, and December 31, 2019. This project describes the findings of a systematic review of the three questions regarding: 1) optimal delivery timing, 2) antibiotic use, and 3) closure considerations. RESULTS: 1339 articles were screened for eligibility; 92 manuscripts were selected and reviewed. The included studies had a Level of Evidence that ranged from 2 to 4 and recommendation Grades B-D. Twenty-eight addressed optimal timing of delivery, 5 pertained to antibiotic use, and 59 discussed closure considerations (Figure 1). Delivery after 37 weeks post-conceptual age is considered optimal. Prophylactic antibiotics covering skin flora are adequate to reduce infection risk until definitive closure. Studies support primary fascial repair, without staged silo reduction, when abdominal domain and hemodynamics permit. A sutureless repair is safe, effective, and does not delay feeding or extend length of stay. Sedation and intubation are not routinely required for a sutureless closure. CONCLUSIONS: Despite the large number of studies addressing the above-mentioned facets of gastroschisis management, the data quality is poor. A wide variation in gastroschisis management was documented, indicating a need for high quality RCTs to provide an evidence-based approach when caring for these infants. TYPE OF STUDY: Qualitative systematic review of Level 1-4 studies.

Remodeling and Regenerative Properties of Fully Absorbable Meshes for Abdominal Wall Defect Repair: A Systematic Review and Meta-Analysis of Animal Studies.

Fully absorbable meshes can repair abdominal wall defects and effectively reduce the incidence of complications, but different types of fully absorbable meshes have different remodeling and regeneration effects. In order to investigate and compare the effects of different fully absorbable meshes on remodeling and regeneration in animals and reduce the biological risk of clinical translation, SYRCLE was adopted to evaluate the methodological quality of the included studies, and GRADE and ConQual were used to evaluate the quality of evidence. According to the inclusion and exclusion criteria, a total of 22 studies related to fully absorbable meshes were included in this systematic review. These results showed that fiber-based synthetic materials and fiber-based natural materials exhibited better restorative and regenerative effects indicated by infiltration and neovascularization, when compared with a porcine acellular dermal matrix. In addition, the human acellular dermal matrix was found to have a similar regenerative effect on the host extracellular matrix and scaffold degradation compared to the porcine acellular dermal matrix, porcine intestinal submucosa, and fiber-based natural materials, but it offered higher tensile strength than the other three. The quality of the evidence in this field was found to be poor. The reasons for downgrading were analyzed, and recommendations for future research included more rigor in study design, more transparency in result reporting, more standardization of animal models and follow-up time for better evaluation of the remodeling and regenerative performance of abdominal wall hernia repair meshes, and less biological risk in clinical translation.

Estimating fetal weight in gastroschisis: A 10 year audit of outcomes at the National Maternity Hospital.

OBJECTIVE: To identify whether conventional methods of estimating fetal growth (Hadlock's formula), which relies heavily on abdominal circumference measurements, are accurate in fetuses with gastroschisis. METHODS: A retrospective cohort study was performed between the period January 1, 2011 and December 31, 2021 in a tertiary referral maternity hospital identifying all pregnancies with a diagnosis of gastroschisis. Projected fetal weight was obtained using the formula (EFW [Hadlock's formula] + 185 g × [X/7]) where X was the number of days to delivery. RESULTS: During the study period 41 cases were identified. The median maternal age was 25. The median BMI was 25 and 63% were primiparous women (n = 26). Median gestation at diagnosis was 21 weeks. Median gestation at delivery was 36 weeks. A total of 4.8% of mothers had a history of drug use (n = 2). The rate of maternal tobacco use was 21.9% (n = 9). A total of 4.8% of fetuses had additional congenital anomalies including amniotic band syndrome and myelomeningocele (n = 2). Estimated fetal weight (EFW) and birth weight data were available for 34 cases. A Wilcoxon signed-rank test showed projected EFW using Hadlock's formula did not result in a statistically significant different birth weight (Z = -1.3, P = 0.169). Median projected weight and actual birth weight were 2241.35 and 2415 g respectively. Median difference was 0.64 g (95% CI: -148 to -28.5). CONCLUSION: Our data showed accuracy using standard formulae for EFW in fetuses with gastroschisis.

European reference network for rare inherited congenital anomalies (ERNICA) evidence based guideline on the management of gastroschisis.

BACKGROUND: The European Reference Network for rare Inherited Congenital Anomalies, ERNICA, guidelines for gastroschisis cover perinatal period to help teams to improve care. METHOD: A systematic literature search including 136 publications was conducted. Research findings were assessed following the GRADE methodology. The evidence to decision framework was used to determine the strength and direction of recommendations. RESULTS: The mode or timing of delivery do not impact neonatal mortality, risk of NEC or time on parenteral nutrition (PN). Intra or extra abdominal bowel dilatation predict complex gastroschisis and longer length of hospital stay but not increased perinatal mortality. Outcomes after Bianchi procedure and primary fascia closure under anesthesia are similar. Sutureless closure decreases the rate of surgical site infections and duration of ventilation compared to surgical closure. Silo-staged closure with or without intubation results in similar outcomes. Outcomes of complex gastroschisis (CG) undergoing early or delayed surgical repair are similar. Early enteral feeds starting within 14 days is associated with lower risk of surgical site infection. RECOMMENDATIONS: The panel suggests vaginal birth between 37 and 39 w in cases of uncomplicated gastroschisis. Bianchi's approach is an option in simple gastroschisis. Sutureless closure is suggested when general anesthesia can be avoided, sutured closure. If anesthesia is required. Silo treatment without ventilation and general anesthesia can be considered. In CG with atresia primary intestinal repair can be attempted if the condition of patient and intestine allows. Enteral feeds for simple gastroschisis should start within 14 days.

Designing a bi-layer multifunctional hydrogel patch based on polyvinyl alcohol, quaternized chitosan and gallic acid for abdominal wall defect repair.

In abdominal wall defect repair, surgical site infection (SSI) remains the primary cause of failure, while complications like visceral adhesions present significant challenges following patch implantation. We designed a Janus multifunctional hydrogel patch (JMP) with antibacterial, anti-inflammatory, and anti-adhesive properties. The patch comprises two distinct layers: a pro-healing layer and an anti-adhesion layer. The pro-healing layer was created by a simple mixture of polyvinyl alcohol (PVA), quaternized chitosan (QCS), and gallic acid (GA), crosslinked to form PVA/QCS/GA (PQG) hydrogels through GA's self-assembly effect and hydrogen bonding. Additionally, the PVA anti-adhesive layer was constructed using a drying-assisted salting method, providing a smooth and dense physical barrier to prevent visceral adhesion while offering essential mechanical support to the abdominal wall. The hydrogel patch demonstrates widely adjustable mechanical properties, exceptional biocompatibility, and potent antimicrobial properties, along with a sustained and stable release of antioxidants. In rat models of skin and abdominal wall defects, the JMP effectively promoted tissue healing by controlling infection, inhibiting inflammation, stimulating neovascularization, and successfully preventing the formation of visceral adhesions. These compelling results highlight the JMP's potential to improve the success rate of abdominal wall defect repair and reduce surgical complications.

Double-Layer Asymmetric Porous Mesh with Dynamic Mechanical Support Properties Enables Efficient Single-Stage Repair of Contaminated Abdominal Wall Defect.

Contamination tolerance and long-term mechanical support are the two critical properties of meshes for contaminated abdominal wall defect repair. However, biological meshes with excellent pollution tolerance fail to provide bio-adaptive long-term mechanical support due to their rapid degradation. Here, a novel double-layer asymmetric porous mesh (SIS/PVA-EXO) is designed by simple and efficient in situ freeze-thaw of sticky polyvinyl alcohol (PVA) solution on the loosely porous surface of small intestinal submucosal decellularized matrix (SIS), which can successfully repair the contaminated abdominal wall defect with bio-adaptive dynamic mechanical support through only single-stage surgery. The exosome-loaded degradable loosely porous SIS layer accelerates the tissue healing; meanwhile, the exosome-loaded densely porous PVA layer can maintain long-term mechanical support without any abdominal adhesion. In addition, the tensile strength and strain at break of SIS/PVA-EXO mesh change gradually from 0.37 MPa and 210% to 0.10 MPa and 385% with the degradation of SIS layer. This unique performance can dynamically adapt to the variable mechanical demands during different periods of contaminated abdominal wall reconstruction. As a result, this SIS/PVA-EXO mesh shows an attractive prospect in the treatment of contaminated abdominal wall defect without recurrence by integrating local immune regulation, tissue remodeling, and dynamic mechanical supporting.

Ibuprofen-loaded bilayer electrospun mesh modulates host response toward promoting full-thickness abdominal wall defect repair.

Pro-inflammatory response impairs the constructive repair of abdominal wall defects after mesh implantation. Electrospinning-aid functionalization has the potential to improve the highly orchestrated response by attenuating the over-activation of foreign body reactions. Herein, we combined poly(L-lactic acid-co-caprolactone) (PLLA-CL) with gelatin proportionally via electrospinning, with Ibuprofen (IBU) incorporation to fabricate a bilayer mesh for the repair improvement. The PLLA-CL/gelatin/IBU (PGI) mesh was characterized in vitro and implanted into the rat model with a full-thickness defect for a comprehensive evaluation in comparison to the PLLA-CL/gelatin (PG) and off-the-shelf small intestinal submucosa (SIS) meshes. The bilayer PGI mesh presented a sustained release of IBU over 21 days with degradation in vitro and developed less-intensive intraperitoneal adhesion along with a histologically weaker inflammatory response than the PG mesh after 28 days. It elicited an M2 macrophage-dominant foreign body reaction within the process, leading to a pro-remodeling response similar to the biological SIS mesh, which was superior to the PG mesh. The PGI mesh provided preponderant mechanical supports over the SIS mesh and the native abdominal wall with similar compliance. Collectively, the newly developed mesh advances the intraperitoneal applicability of electrospun meshes by guiding a pro-remodeling response and offers a feasible functionalization approach upon immunomodulation.

Severe High-Voltage Electrical Injury: A Rare Case Report.

High-voltage electrical injury usually causes severe tissue damage and serious secondary complications. We report a case of treatment of severe high-voltage electrical injury. A series of personalized and effective treatment plans were created through repeated discussions, we successfully handled a series of acute and critical conditions, including severe limb damage, a very large area of full-thickness abdominal wall defect, abdominal viscera (stomach and liver) necrosis, abdominal infection, renal insufficiency, myocardial damage, and malignant arrhythmia (atrial fibrillation). Finally, the wounds were all closed, the functions of the abdominal organs were restored, and the course of the disease was successfully transitioned into the rehabilitation stage. It took a lot of twists and turns but ultimately saved the patient's life. The successful treatment of this patient provides an important reference for similar patients with serious electrical injury in the future.

Enhancing Omphalocele Care: Navigating Complications and Innovative Treatment Approaches.

Congenital abdominal wall abnormalities in infants present an interesting and difficult management problem for surgeons. Congenital malformations of the ventral abdominal wall can be diagnosed by their distinctive anatomical presentations. Among them, omphalocele, gastroschisis and umbilical cord hernias are the most frequently observed clinically detected abdominal wall anomalies. Omphalocele refers to the herniation of abdominal contents through a defect in the abdominal wall at the umbilicus with or without the presence of a sac. This article gives an insight into the embryology, risk factors, treatment, investigations and complications of omphalocele, a common congenital abdominal wall defect. There is minimal proof that environmental factors contribute to the development of omphalocele. However, there is a considerable amount of evidence which points to the importance of genetic or familial risk factors. Newborns and infants with prenatal diagnoses are the most frequently presenting patients with omphalocele to paediatric surgeons. This article describes the problems and the steps of management for handling each circumstance, as well as any further complications. Omphalocele and gastroschisis are frequently described together in many research papers. However, it's crucial to consider that they are two different conditions which vary in anatomy, pathology and associated conditions which account for the difference in their treatments and noticeably varied outcomes. Additionally, there is evidence that each has a different set of factors associated with risk for occurrence. There are no known etiologic causes that cause these abnormalities to develop. The size of the baby, the extent of the lesion, and any other disorders all affect how individuals with these congenital abdominal wall anomalies are treated.

The increased angiogenic capacity and decreased inflammatory response when a mesh is used in combination with an omental flap. A prospective experimental study.

BACKGROUND: The use of a surgical mesh for abdominal wall reconstruction is well established and has been used for long with minor complications, whereas the omental flap has been used for decades in reconstructive surgery. AIM: To demonstrate the increased angiogenic capacity and the reduced inflammatory markers of a synthetic mesh when used in combination with an omental flap. Furthermore, we compare two independent meshes when used alone or in combination with the omental flap. MATERIALS AND METHODS: Twenty-eight rats were included in the study. To determine the effect of using an omental flap under two different meshes, the animals were separated into four groups, i.e., group A (flap + mesh 1), group B (flap + mesh 1 + silicone), group C (flap + mesh 2), and group D (flap + mesh 2 + silicone). A silicone sheet was placed as a barrier between the mesh and the flap. All groups were sacrificed 8 weeks post-operatively. RESULTS: The use of a silicone sheet barrier between any of the two synthetic meshes and the omental flap in an abdominal wall defect is accompanied by a markedly reduced angiogenesis in terms of a cluster of differentiation (CD)-34 (p < 0.001) and factor VIII (p = 0.0012) and by increased inflammatory response CD-68 (p = 0.0024) and visual scoring (p < 0.001). CONCLUSIONS: Τhe increased angiogenic capacity and the reduced inflammatory markers of a synthetic surgical mesh when used in combination with an omental flap make it a useful option in the reconstruction of an abdominal wall defect on a large or contaminated wound.

Contrasting Presentations of Spigelian Hernia in a Single Surgical Unit of a Tertiary Healthcare Center: A Case Series.

A Spigelian hernia is a very rare type of anterior abdominal wall hernia. The etiology revolves around the dehiscence of the transverse abdominis and internal oblique aponeurosis. The majority of Spigelian hernias develop in the lower abdomen where the posterior sheath is absent. This condition is also known as a spontaneous lateral ventral hernia or hernia of the semilunar line. It is mostly asymptomatic and is generally proven by radiological diagnosis. In the worst-case scenario, strangulation of the Spigelian hernia can occur. Here, we report a case series of contrasting presentations of Spigelian hernia in a single surgical unit of a tertiary healthcare center, considering the rarity and associated complications of Spigelian hernia.

Prevalence of Beckwith Wiedemann Syndrome and Risk of Embryonal Tumors in Children Born with Omphalocele.

AIM OF THE STUDY: Children with omphalocele have an increased prevalence of Beckwith Wiedemann syndrome (BWS) and thus a suspected increased risk of developing embryonal tumors, e.g. Wilms tumor, hepatoblastoma, neuroblastoma and rhabdomyosarcoma. The aim of this study was to examine the prevalence of BWS and the risk of embryonal tumors amongst patients born with omphalocele. METHODS: A population-based cohort was used, including all children born in Sweden 1/1 1997-31/12 2016. Patients with omphalocele were identified through the Swedish National Patient Register and the Swedish Medical Birth Register. For each case of omphalocele ten age and sex matched individuals unexposed for omphalocele were randomly selected for comparison. Data on BWS and embryonal tumors were collected from the Swedish National Patient Register and the Swedish National Cancer Register. MAIN RESULTS: Out of 207 cases of omphalocele, 15 (7.2%) were diagnosed with BWS. None of the children with omphalocele had yet developed any kind of embryonal tumor (median follow-up time 8 years). None of the 2070 controls were diagnosed with BWS but 3 (0.1%) of them had developed embryonal tumors during a median follow-up time of 10 years. CONCLUSIONS: In this study the prevalence of BWS amongst children born with omphalocele is in the lower range of previously reported figures. Also, the prevalence of embryonal tumors amongst children with BWS is lower than expected and the risk of embryonal tumors in children with omphalocele and BWS might not be as high as previously stated. This must be taken into consideration when counseling parents prenatally. TYPE OF STUDY: National register cohort study. LEVEL OF EVIDENCE: II.