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World Journal of Clinical Cases May 2024Morgagni hernia (MH) is a form of congenital diaphragmatic hernia (CDH) characterized by an incomplete formation of diaphragm, resulting in the protrusion of abdominal...
BACKGROUND
Morgagni hernia (MH) is a form of congenital diaphragmatic hernia (CDH) characterized by an incomplete formation of diaphragm, resulting in the protrusion of abdominal organs into the thoracic cavity. The estimated incidence of CDH is between 1 in 2000 and 1 in 5000 live births, although the true incidence is unknown. MH typically presents in childhood and can be diagnosed either prenatally or postnatally. However, it can also be asymptomatic and carry the risk of developing into a life-threatening condition in adulthood.
CASE SUMMARY
A 76-year-old female with no history of prior abdominal surgeries presented for an elective colonoscopy for polyp surveillance. During the procedure, when approaching the hepatic flexure, the scope could not be advanced further despite multiple attempts. The patient experienced mild abdominal discomfort, leading to the abortion of the procedure. While in the recovery area, she developed increasing abdominal pains and hypotension. Urgent abdominal imaging revealed herniation of the proximal transverse colon through a MH into the chest with evidence of perforation. The patient underwent laparoscopic urgent colonic resection and primary hernia repair and was discharged uneventfully 2 d later.
CONCLUSION
A MH is a rare condition in adults that can present as a life-threatening complication of colonoscopy, even in patients with a history of uneventful colonoscopies. This case highlights the importance of considering congenital and internal hernias when faced with sudden and unexplained difficulties during colonoscopy. If there is a suspicion of MH, the endoscopist should halt the procedure and immediately obtain abdominal imaging to confirm the diagnosis.
PubMed: 38765745
DOI: 10.12998/wjcc.v12.i14.2389 -
JPGN Reports May 2024Congenital mixed hiatal hernia is a disorder that combines features of both sliding and paraoesophageal hernias. The precise incidence of congenital mixed hiatal hernia...
Congenital mixed hiatal hernia is a disorder that combines features of both sliding and paraoesophageal hernias. The precise incidence of congenital mixed hiatal hernia during the pediatric and neonatal period remains uncertain, making diagnosis challenging within this age cohort. This case presents a 15-day-old female with an 8% postnatal weight loss and apost-feeding vomiting. An upper gastrointestinal series, computer tomography, and upper endoscopy revealed a mixed hiatal hernia. The patient underwent a laparoscopic herniorrhaphy and Nissen fundoplication achieving successful resumption of complete oral feeding before discharge. Diagnosis and management of this condition in neonates remain challenging due to its rarity and variable clinical presentations. This report emphasizes the importance of early recognition, accurate diagnosis, and tailored management strategies in the neonatal period. Further research, with a collaborative effort between pediatricians and surgeons, is needed to refine diagnostic criteria, establish evidence-based management approaches, and improve outcomes for affected children.
PubMed: 38756129
DOI: 10.1002/jpr3.12042 -
World Journal of Pediatric Surgery 2024Congenital diaphragmatic hernia (CDH) is a major congenital anomaly, resulting from the herniation of abdominal contents into the thoracic cavity, thereby impeding the... (Review)
Review
Congenital diaphragmatic hernia (CDH) is a major congenital anomaly, resulting from the herniation of abdominal contents into the thoracic cavity, thereby impeding the proper development of the lungs and pulmonary vasculature. CDH severity correlates with a spectrum of pulmonary hypoplasia, pulmonary hypertension (PHT), and cardiac dysfunction, constituting the pathophysiological triad of this complex condition. The accurate diagnosis and effective management of PHT and cardiac dysfunction is pivotal to optimizing patient outcomes. Targeted neonatal echocardiography is instrumental in delivering real-time data crucial for the bespoke, pathophysiology-targeted hemodynamic management of CDH-associated PHT.
PubMed: 38737963
DOI: 10.1136/wjps-2024-000790 -
Surgical Endoscopy Jun 2024The introduction of the functional lumen imaging probe (FLIP) has provided objective, real-time feedback on the geometric variations with each component of a hiatal...
INTRODUCTION
The introduction of the functional lumen imaging probe (FLIP) has provided objective, real-time feedback on the geometric variations with each component of a hiatal hernia repair (HHR). The utility of this technology in altering intraoperative decision-making has been scarcely reported. Herein, we report a single-center series of intraoperative FLIP during HHR.
METHODS
A retrospective review of electronic medical records between 2020 and 2022 was conducted and all patients undergoing non-recurrent HHR with FLIP were queried. Patient and hernia characteristics, intraoperative FLIP values and changes in decision-making, as well as early post-operative outcomes were reported. Both diameter and distensibility index (DI) were measured at 40 ml and 50 ml balloon inflation after hiatal dissection, after hiatal closure, and after fundoplication when indicated.
RESULTS
Thirty-three patients met inclusion criteria. Mean age was 62 ± 14 years and mean BMI was 28 ± 6 kg/m. The majority (53%) were type I hiatal hernias. The largest drop in DI occurred after hiatal closure, with minimal change seen after fundoplication (mean DI of 4.3 ± 2. after completion of HH dissection, vs 2.7 ± 1.2 after hiatal closure and 2.3 ± 1 after fundoplication when performed). In 13 (39%) of cases, FLIP values directly impacted intraoperative decision-making. Fundoplication was deferred in 4/13 (31%) patients, the wrap was loosened in 2/13 (15%); the type of fundoplication was altered to achieve adequate anti-reflux values in 2/13 (15%) patients, and in 1/13 (3%) the wrap was tightened.
CONCLUSION
FLIP measurements can be used intraoperatively to guide decision-making and alter management plan based on objective values. Long-term outcomes and further prospective studies are required to better delineate the value of this technology.
Topics: Hernia, Hiatal; Humans; Middle Aged; Female; Retrospective Studies; Male; Herniorrhaphy; Aged; Fundoplication
PubMed: 38722379
DOI: 10.1007/s00464-024-10851-6 -
Gastroenterology Report 2024
PubMed: 38716309
DOI: 10.1093/gastro/goae043 -
Translational Pediatrics Apr 2024In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development... (Review)
Review
In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development of pulmonary hypoplasia and hypertension, which is the primary determinant of morbidity and mortality for affected newborns. The severity is determined using prenatal imaging as early as the first trimester and is related to the laterality of the defect, extent of lung compression, and degree of liver herniation. Comprehensive evaluation of fetal CDH includes imaging-based severity assessment, severity assessment, and evaluation for structural or genetic abnormalities to differentiate isolated from complex cases. Prenatal management involves multispecialty counseling, consideration for fetal therapy with fetoscopic endoluminal tracheal occlusion (FETO) for severe cases, monitoring and intervention for associated polyhydramnios or signs of preterm labor if indicated, administration of antenatal corticosteroids in the appropriate setting, and planned delivery to optimize the fetal condition at birth. Integrated programs that provide a smooth transition from prenatal to postnatal care produce better outcomes. Neonatal care involves gentle ventilation to avoid hyperinflation and must account for transitional physiology to avoid exacerbating cardiac dysfunction and decompensation. Infants who have undergone and responded to FETO have greater pulmonary capacity than expected, but cardiac dysfunction seems unaffected. In about 25-30% of CDH neonates extracorporeal life support is utilized, and this provides a survival benefit for patients with the highest predicted mortality, including those who underwent FETO. Surgical repair after initial medical management for the first 24-48 hours of life is preferred since later repair is associated with delayed oral feeding, increased need for tube feeds, and increased post-repair ventilation requirement and supplemental oxygen at discharge. With overall survival rates >70%, contemporary care involves management of chronic morbidities in the context of a multidisciplinary clinic setting.
PubMed: 38715680
DOI: 10.21037/tp-23-602 -
Multidisciplinary follow-up in a patient with Morgagni hernia leads to diagnosis of Marfan syndrome.Italian Journal of Pediatrics May 2024congenital diaphragmatic hernia (CDH) is a birth defect occurring in isolated or syndromic (chromosomal or monogenic) conditions. The diaphragmatic defect can be the...
BACKGROUND
congenital diaphragmatic hernia (CDH) is a birth defect occurring in isolated or syndromic (chromosomal or monogenic) conditions. The diaphragmatic defect can be the most common one: left-sided posterolateral, named Bochdalek hernia; or it can be an anterior-retrosternal defect, named Morgagni hernia. Marfan syndrome (MFS) is a rare autosomal dominant inherited condition that affects connective tissue, caused by mutations in fibrillin-1 gene on chromosome 15. To date various types of diaphragmatic defects (about 30 types) have been reported in association with MFS, but they are heterogeneous, including CDH and paraesophageal hernia.
CASE PRESENTATION
We describe the case of a child incidentally diagnosed with Morgagni hernia through a chest X-ray performed due to recurrent respiratory tract infections. Since the diagnosis of CDH, the patient underwent a clinical multidisciplinary follow-up leading to the diagnosis of MFS in accordance with revised Ghent Criteria: the child had typical clinical features and a novel heterozygous de novo single-base deletion in exon 26 of the FBN1 gene, identified by Whole-Exome Sequencing. MFS diagnosis permitted to look for cardiovascular complications and treat them, though asymptomatic, in order to prevent major cardiovascular life-threatening events.
CONCLUSION
Our case shows the importance of a long-term and multidisciplinary follow-up in all children with diagnosis of CDH.
Topics: Humans; Adipokines; Fibrillin-1; Follow-Up Studies; Hernias, Diaphragmatic, Congenital; Marfan Syndrome; Child
PubMed: 38715046
DOI: 10.1186/s13052-024-01643-8 -
Pediatric Surgery International May 2024Postoperative pulmonary growth in congenital diaphragmatic hernias (CDH) remains unclear. We investigated postoperative pulmonary vascular growth using serial lung...
BACKGROUND
Postoperative pulmonary growth in congenital diaphragmatic hernias (CDH) remains unclear. We investigated postoperative pulmonary vascular growth using serial lung perfusion scintigraphy in patients with CDH.
METHODS
Neonates with left CDH who underwent surgery and postoperative lung perfusion scintigraphy at our institution between 2001 and 2020 were included. Patient demographics, clinical courses, and lung scintigraphy data were retrospectively analyzed by reviewing medical records.
RESULTS
Twenty-one patients with CDH were included. Of these, 10 underwent serial lung scintigraphy. The ipsilateral perfusion rate and median age on the 1st and serial lung scintigraphy were 32% (34 days) and 33% (3.6 years), respectively. Gestational age at prenatal diagnosis (p = 0.02), alveolar-arterial oxygen difference (A-aDO) at birth (p = 0.007), and preoperative nitric oxide (NO) use (p = 0.014) significantly correlated with the 1st lung scintigraphy. No other variables, including operative approach, were significantly correlated with the 1st or serial scintigraphy findings. All patients improved lung perfusion with serial studies [Difference: + 7.0 (4.3-13.25) %, p = 0.001, paired t-test]. This improvement was not significantly correlated with preoperative A-aDO (p = 0.96), NO use (p = 0.28), or liver up (p = 0.90). The difference was significantly larger in patients who underwent thoracoscopic repair than in those who underwent open abdominal repair [+ 10.6 (5.0-17.1) % vs. + 4.25 (1.2-7.9) %, p = 0.042].
CONCLUSION
Our study indicated a postoperative improvement in ipsilateral lung vascular growth, which is possibly enhanced by a minimally invasive approach, in patients with CDH.
Topics: Humans; Hernias, Diaphragmatic, Congenital; Retrospective Studies; Female; Male; Infant, Newborn; Lung; Postoperative Period; Perfusion Imaging; Child, Preschool
PubMed: 38714568
DOI: 10.1007/s00383-024-05706-z -
Langenbeck's Archives of Surgery May 2024Paraoesophageal hernias (PEH) are associated with a high complication rate and often occur in elderly and fragile patients. Surgical gastropexy without fundoplication is...
PURPOSE
Paraoesophageal hernias (PEH) are associated with a high complication rate and often occur in elderly and fragile patients. Surgical gastropexy without fundoplication is an accepted alternative procedure; however, outcomes and functional results are rarely described. Our study aims to evaluate short-term outcomes and the long-term quality of life after gastropexy as treatment for PEH.
METHODS
Single center cohort analysis of all consecutive patients who underwent gastropexy for PEH without fundoplication. Postoperative outcomes and functional results were retrospectively collected. Reflux symptoms developed postoperatively were reported using the validated quality of life questionnaire: GERD-Health Related Quality of Life Qestionnaire (GERD-HRQL).
RESULTS
Thirty patients (median age: 72 years (65-80)) were included, 40% classified as ASA III. Main PEH symptoms were reflux (63%), abdominal/thoracic pain (47%), pyrosis (33%), anorexia (30%), and food blockage (26%). Twenty-six laparoscopies were performed (86%). Major complications (III-IVb) occurred in 9 patients (30%). Seven patients (23%) had PEH recurrence, all re-operated, performing a new gastropexy. Median follow-up was 38 (17-50) months. Twenty-two patients (75%) reported symptoms resolution with median GERD-HRQL scale of 4 (1-6). 72% (n = 21) reported operation satisfaction. GERD-HRQL was comparable between patients who were re-operated for recurrence and others: 5 (2-19) versus 3 (0-6), p = 0.100.
CONCLUSION
Gastropexy without fundoplication was performed by laparoscopy in most cases with acceptable complications rates. Two-thirds of patients reported symptoms resolution, and long-term quality-of-live associated to reflux symptoms is good. Although the rate of PEH recurrence requiring a new re-intervention remained increased (23%), it does not seem to affect long-term functional results.
Topics: Humans; Hernia, Hiatal; Female; Male; Aged; Quality of Life; Aged, 80 and over; Retrospective Studies; Gastropexy; Treatment Outcome; Herniorrhaphy; Fundoplication; Postoperative Complications; Gastroesophageal Reflux; Laparoscopy; Cohort Studies; Recurrence
PubMed: 38702556
DOI: 10.1007/s00423-024-03340-w -
Turkish Journal of Surgery Dec 2023In hospital attendance, 75% of diaphragmatic hernias occur on left as opposed to 25% on the right side. Right side hernias are associated with abdominal injuries, mainly...
OBJECTIVES
In hospital attendance, 75% of diaphragmatic hernias occur on left as opposed to 25% on the right side. Right side hernias are associated with abdominal injuries, mainly the liver. However, right-side injuries are frequently underdiagnosed due to the complexity of associated injuries and high mortality rates. The aim of this study was to perform a retrospective analysis of records from our clinical experience to investigate demographics, TM, diagnosis, morbidity, and mortality associated with right sided TDH. These findings may provide insights into improving the clinical management of patients with this serious injury, potentially reducing morbidity and mortality rates.
MATERIAL AND METHODS
Retrospective analysis of the medical records of patients from the trauma database of the Division of Trauma Surgery at University of Campinas in 32-year period was performed. Only records of patients with right sided TDH were included in the analysis.
RESULTS
Blunt trauma was the most common mechanism. Diagnoses were made by laparotomy in eight cases, all these cases were hemodynamically unstable. TDH grade III injury occurred in most cases followed by grade IV. Liver injuries were present in almost all cases, most of them high grade, followed by colon and small bowel. Extra-abdominal associated injuries with a predominance of femur fractures, pelvic fractures and hemothorax. Post-operative complications were associated with length of stay in intensive care unit. Pneumonia was the most frequent complication. The overall mortality rate was 16%.
CONCLUSION
Most diagnoses were performed through laparotomy and not by radiologic exams, due to hemodynamic instability on admission. There is underdiagnosis of right-side TDH due to the high-energy trauma mechanism with high grade associated injuries and mortality on pre-hospital.
PubMed: 38694525
DOI: 10.47717/turkjsurg.2023.6271