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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Short and Medium-term Outcomes of Omphalocele and Gastroschisis: A Survey from a Tertiary Center / Resultados a curto e médio prazo do onfalocelo e gastrosquisis: uma investigação de um centro terciário

Abstract Objective To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center. Methods This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019. Results There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p=0.033), were born earlier (36 versus 37 weeks, p=0.006), had lower birth weight (2365±430.4 versus 2944.2±571.9 g; p=0.001), and had a longer hospital stay (24 versus 9 days, p=0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%). Conclusion Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.

Resumo Objetivo Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. Métodos Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. Resultados Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p=0.033), nasceram mais precocemente (36 versus 37 semanas, p=0.006), com menor peso ao nascimento (2,365±430.4 versus 2,944.2±571.9 g; p=0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p=0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante umtempo mediano de seguimento de 24meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). Conclusão Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrermorbidade substancial no médio prazo.

Gastrosquisis en Hospital IESS "Manuel Ygnacio Monteros". Presentación de caso clínico y revisión bibliográfica / Gastroschisis in Hospital del IESS "Manuel Ygnacio Ygnacio". Case Report and Literature Reviview.

INTRODUCCIÓN: La gastrosquisis es una anomalía que expone los órganos abdominales hacia el exterior. La prevalencia media global es de 1.36 por cada 10 000 nacidos vivos. En el Ecuador, según el Instituto Nacional de Estadísticas y Censos (INEC), se evidencia un aumento en el número de casos de egreso hospitalario alcanzando los 52 pacientes en el año 2010, los 85 en el 2012 y los 112 pacientes en el 2015. El objetivo del presente caso es realizar una descripción de esta patología y dar a conocer la importancia que tiene su diagnóstico prenatal con el fin de planificar su resolución quirúrgica para la atención inmediata al recién nacido luego del nacimiento. CASO CLÍNICO: recién nacido de madre primigesta de 16 años, sin más antecedentes prenatales, se realizó cuatro ultrasonidos durante el embarazo en casa de salud particular, los cuales no reportaron alteraciones. A las 37.4 semanas nace por parto eutócico, evidenciándose gastrosquisis. Se realizó laparotomía más peritoneostomía a las 24 horas de vida, y se programó cierre diferido. EVOLUCIÓN: recibió manejo integral en cuidados intensivos neonatales. Se realizó cierre definitivo a los 8 días. Permaneció hospitalizado por 22 días, recibió nutrición parenteral y se inició alimentación trófica a los 15 días de vida; se inició antibioticoterapia de primera línea, sin embargo por evolución tórpida, requirió rotación a antibiótico de amplio espectro. CONCLUSIÓN: la gastrosquisis se puede ser detectada por ecografía desde la semana doce de gestación con el objetivo de minimizar las complicaciones y planificar su resolución quirúrgica inmediatamente luego de su nacimiento.(au)

BACKGROUND: Gastroschisis is an anomaly in which abdominal organs are exposed outside the abdominal wall. The global average prevalence is 1.36 for each 10 000 newborns. In Ecuador, according to the National Institute of Statistics and Censuses (INEC), there is evidence of an increase in the number of hospital discharges with this diagnosis, reaching 52 in 2010, 85 in 2012, and 112 patients in 2015. The aim of this publication is to describe this pathology and expose the importance of prenatal diagnosis to plan prompt surgical intervention for the newborn after birth. CASE REPORT: newborn from a 16-year-old primigravid mother, with no other prenatal history, four ultrasounds were performed during pregnancy in a private health facility, which reported no alterations. At 37.4 weeks of pregnancy, the newborn was delivered by eutocic birth, showing gastroschisis. The patient underwent laparotomy and peritoneostomy at 24 hours of age, delayed closure was scheduled. EVOLUTION: the newborn received comprehensive care in the neonatal intensive care unit. Delayed closure was performed eight days later. He remained hospitalized for 22 days, he received parenteral nutrition and started trophic feeding at 15 days of age; first-line antibiotic therapy was initiated, however due to torpid evolution, he required a change to a broad-spectrum antibiotic. CONCLUSION: gastroschisis can be detected in ultrasound from the twelve week of pregnancy to minimize complications and program the surgical procedure immediately after birth.(au)

Estado actual de la gastrosquisis en recién nacidos en el Hospital Nacional Daniel A. Carrión: Serie de casos de 5 años / Gastroschisis in newborns at Hospital Nacional Daniel A. Carrión: Case series in 5 years

La gastrosquisis es un defecto congénito de la pared abdominal que permite la exteriorización del contenido abdominal, cuyo pronóstico dependerá de las complicaciones intestinales asociadas. Es importante la detección prenatal de aquellos casos con riesgo de gastrosquisis compleja. Objetivo. Describir las características epidemiológicas de los recién nacidos con gastrosquisis atendidos en un hospital nacional. Diseño. Serie de casos. Institución. Hospital Nacional Daniel A. Carrión (HNDAC), Lima, Perú. Pacientes. Recién nacidos vivos. Metodología. Estudio de todos los recién nacidos vivos con el diagnóstico de gastrosquisis, en un periodo de 5 años. Se excluyó aquellos con nacimiento extrainstitucional o con datos insuficientes. Principales medidas de resultados. Características y manejo de los recién nacidos con gastrosquisis. Resultados. Se reportaron 17 casos. La incidencia fue 9,7/1 000 nacidos vivos y la mortalidad perinatal 14%; 64,3% nacieron de madres adolescentes, 78% primigestas. El nacimiento ocurrió principalmente por cesárea (71,4%). Existió un pobre registro de predictores ecográficos de complicaciones intestinales. Se realizó cierre primario en 28,6% de los casos. El primer caso de mortalidad ocurrió luego de manejo quirúrgico con silo, falleciendo por sepsis. El segundo caso presentó atresia intestinal requiriendo múltiples cirugías y falleció por atelectasia masiva. Conclusiones. Es necesario estandarizar la valoración y registro de los diversos marcadores pronósticos en gastrosquisis fetal. Es necesario establecer series más largas y mejor documentadas.

Gastroschisis is a congenital defect of the abdominal wall that allows externalization of the abdominal contents; prognosis depends on the associated intestinal complications. Prenatal detection of cases with risk of complex gastroschisis is important. Objective: To describe the epidemiological characteristics of newborns with gastroschisis occurring at a national hospital. Design: Case series. Institution: Hospital Nacional Daniel A. Carrion (HNDAC), Lima, Peru. Patients: Newborns with gastroschisis. Methodology: All live newborns with the diagnosis of gastroschisis in a period of 5 years were studied. Cases born in other institutions or with insufficient data were excluded. Main outcome measures: Characteristics of newborns with gastroschisis and their management. Results: 17 cases were reported with an incidence of 9.7/1 000 live newborns and with a perinatal mortality of 14%; 64.3% were born to an adolescent mother, of which 78% carried their first pregnancy. The main delivery mode was cesarean section (71.4%). There were poor records of ultrasonographic predictors of intestinal complications. Primary closure was performed in 28.6% of cases. The first case of death occurred after surgery with silo and died from sepsis. The second case had intestinal atresia requiring multiple surgeries and died due to massive atelectasis. Conclusions: It is necessary to standardize the assessment and registration of various prognostic markers in fetal gastroschisis. More and better documented series are necessary.

Pentalogía de Cantrell: presentación de un caso clínico / Pentalogy of Cantrell: clinical case

La pentalogía de Cantrell es una enfermedad poco frecuente que resulta de alteraciones embriológicas del mesodermo, caracterizada por defecto en la porción baja esternal, defecto de la pared abdominal supraumbilical, defecto diafragmático anterior, defecto del pericardio diafragmático y anomalía cardiaca. Caso clínico: Se presenta el caso de un neonato de sexo masculino con diagnóstico prenatal de defecto de la pared abdominal, en quien posteriormente se realizó el diagnóstico de Pentalogía de Cantrell. Al presentar este caso se destaca la posibilidad diagnóstica en la vida fetal, y el pobre pronóstico de estos pacientes cuando su presentación clínica se asocia a hipoplasia pulmonar severa.

Pentalogy of Cantrell is a rare disease that results from alterations in embryo development. It is characterized by defects of the lower sternum, midline supraumbilical abdominal wall defect, deficiency of the anterior diaphragm, defects in the diaphragmatic pericardium and cardiac anomaly. Case Report: The case of a male neonate with a prenatal diagnosis of abdominal wall defect is presented, who was subsequently diagnosed with Pentalogy of Cantrell. This study emphasizes the prenatal diagnosis possibilities and the poor prognosis for these patients when their clinical presentation is associated with severe lung hypoplasia.