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World Journal of Clinical Cases May 2024A Bochdalek hernia (BH) is a congenital diaphragmatic hernia that often develops in the neonatal period. BH typically occurs on the left side of the diaphragm. A...
BACKGROUND
A Bochdalek hernia (BH) is a congenital diaphragmatic hernia that often develops in the neonatal period. BH typically occurs on the left side of the diaphragm. A right-sided BH in an adult is rare.
CASE SUMMARY
A 45-year-old man was referred to our hospital because of an abnormal shadow seen on chest radiography during a medical check-up. A chest radiograph showed elevation of the right hemidiaphragm. Computed tomography showed prolapse of multiple intraabdominal organs into the right thoracic cavity, corresponding to a right-sided BH. The herniated contents included the stomach, transverse colon, and left lobe of the liver. The left lobe of the liver was enlarged, particularly the medial segment. Laparoscopic surgery was performed. However, the left lobe of the liver was completely trapped in the thoracic cavity. Therefore, thoracoscopic manipulation had to be performed to return the liver to the abdominal cavity. The hernia was repaired with interrupted nonabsorbable sutures and reinforced with mesh.
CONCLUSION
Combined laparoscopic and thoracoscopic surgery was successfully performed for right-sided BH with massive liver prolapse and abnormal liver morphology.
PubMed: 38765754
DOI: 10.12998/wjcc.v12.i14.2420 -
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 -
Ultrasound in Obstetrics & Gynecology :... May 2024
PubMed: 38764162
DOI: 10.1002/uog.27700 -
Statewide Hospital Admissions for Adult Survivors of Infant Surgical Diseases Over a 10-Year Period.The Journal of Surgical Research Jul 2024The number of patients with congenital disease living to adulthood continues to grow. Often undergoing surgical correction in infancy, they continue to require lifelong...
INTRODUCTION
The number of patients with congenital disease living to adulthood continues to grow. Often undergoing surgical correction in infancy, they continue to require lifelong care. Their numbers are largely unknown. We sought to evaluate hospital admissions of adult patients with esophageal atresia with tracheoesophageal fistula (EA/TEF), congenital diaphragmatic hernia (CDH), and Hirschsprung disease (HD).
METHODS
The Florida Agency for Healthcare Administration inpatient database was merged with the Distressed Communities Index and Centers for Medicare and Medicaid Services Hospital and Physician Compare datasets. The dataset was queried for adult patients (≥18 y, born after 1970) with EA/TEF, CDH, and HD in their problem list from 2010 to 2020. Patient demographics, hospitalization characteristics, and discharge information were obtained.
RESULTS
In total, 1140 admissions were identified (266 EA/TEF, 135 CDH, 739 HD). Patients were mostly female (53%), had a mean age of 31.6 y, and often admitted to an adult internist in a general hospital under emergency. Principal diagnoses and procedures (when performed) varied with diagnosis and age at admission. EA patients were admitted with dysphagia and foregut symptoms and often underwent upper endoscopy with dilation. CDH patients were often admitted for diaphragmatic hernias and underwent adult diaphragm repair. Hirschsprung patients were often admitted for intestinal obstructive issues and frequently underwent colonoscopy but trended toward operative intervention with increasing age.
CONCLUSIONS
Adults with congenital disease continue to require hospital admission and invasive procedures. As age increases, diagnoses and performed procedures for each diagnoses evolve. These data could guide the formulation of multispecialty disease-specific follow-up programs for these patients.
Topics: Humans; Female; Male; Adult; Hirschsprung Disease; Hernias, Diaphragmatic, Congenital; Florida; Esophageal Atresia; Young Adult; Tracheoesophageal Fistula; Middle Aged; Survivors; Hospitalization; Adolescent; Retrospective Studies; Infant; Databases, Factual
PubMed: 38759333
DOI: 10.1016/j.jss.2024.04.018 -
Current Medical Imaging May 2024This case report describes a case of Müllerian duct cyst that occurred in a male retroperitoneum. The cyst lesion is rare and complicated with diaphragmatic hernia....
BACKGROUND
This case report describes a case of Müllerian duct cyst that occurred in a male retroperitoneum. The cyst lesion is rare and complicated with diaphragmatic hernia. Müllerian duct-derived cyst is a rare developmental disorder that is more common in male pelvic tissues and rare in the retroperitoneum. We investigated the important role of computerized tomography (CT) and magnetic resonance imaging (MRI) in preoperative diagnosis and disease prediction of this condition.
CASE PRESENTATION
A 25-year-old male was found to have an abnormal occupying lesion in the left diaphragm in imaging examinations, usually healthy with no obvious clinical symptoms. X-ray examination showed a circular, high-density shadow near the left diaphragm. CT scan showed a soft tissue density shadow resembling a tumor in the left adrenal area, irregularly protruding into the chest cavity, with uneven density. MRI examination showed an irregular elongated T1 and T2 signal shadow in the left adrenal area. T2 fat suppression showed high signal intensity with unrestricted diffusion. Robotic-assisted laparoscopic surgery showed left retroperitoneal tumor resection. The patient recovered well postoperatively and had no recurrence after discharge follow-up.
CONCLUSION
The preclinical symptoms of retroperitoneal Müllerian cysts complicated by diaphragmatic hernia in young men are difficult to distinguish, and it is difficult to diagnose other similar cysts with imaging. The method of combined CT and MRI diagnosis guides the endoscopic robot-assisted minimally invasive surgery for excision of cysts to achieve accurate diagnosis and treatment of such diseases.
PubMed: 38757328
DOI: 10.2174/0115734056303445240509061437 -
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 -
Pediatric Radiology May 2024Right atrial isomerism is a rare and severe isomerism. It is frequently associated with complex congenital heart disease and various extracardiac anomalies. Imaging...
Ultrasound and computed tomography angiography diagnosis of fetal right atrial isomerism with cardiac total anomalous pulmonary venous connection and intestinal malrotation: a case report and literature review.
Right atrial isomerism is a rare and severe isomerism. It is frequently associated with complex congenital heart disease and various extracardiac anomalies. Imaging diagnosis of right atrial isomerism is a challenge. Multisystem and complex anomalies in a 24-week-old fetus were diagnosed with prenatal ultrasound, postnatal computed tomography angiography (CTA), and autopsy. The ultrasound detected most major cardiovascular anomalies including right atrial isomerism and total anomalous pulmonary venous connection. The CTA further detected thoracic and abdominal malformations such as bilateral morphologically right bronchus, diaphragmatic hernia, asplenia, midline liver, and intestinal malrotation. The autopsy confirmed both ultrasound and CTA findings with additional findings, namely, bilateral trilobed lungs and bilateral morphological right auricles. Prenatal ultrasound and postnatal CTA can be complementary to each other in detecting multi-system complex anomalies. Their combined use can be useful for prenatal counseling and postpartum management.
PubMed: 38750327
DOI: 10.1007/s00247-024-05938-0 -
BJOG : An International Journal of... May 2024To evaluate medium-term self-reported respiratory and gastrointestinal (GI) outcomes in children with congenital diaphragmatic hernia (CDH).
OBJECTIVE
To evaluate medium-term self-reported respiratory and gastrointestinal (GI) outcomes in children with congenital diaphragmatic hernia (CDH).
DESIGN
Self-reported respiratory and GI outcomes correlated with prenatal severity indicators.
SETTING
Prospective study at three fetal medicine units.
POPULATION
Families of children prenatally diagnosed with isolated, left-sided CDH surviving for >1 year.
METHODS
Families received validated questionnaires for GI outcomes (Infant Gastroesophageal Reflux Questionnaire Revised, I-GERQ-R, for infants aged <2 years, or Paediatric Gastro-oesophageal Symptom and Quality of Life Questionnaire, PGSQ, for children aged aged 2-8 years or >9 years) and respiratory outcomes (preschool respiratory outcome questionnaire, for children aged ≤5 years, or the International Study of Asthma and Allergies in Childhood asthma questionnaire, for children aged 6-8 years or ≥9 years). Prenatal data collected from the medical records included lung size (percentage observed/expected lung-to-head ratio, O/E LHR %), liver position, fetal endoluminal tracheal occlusion (FETO) gestational age (GA) at delivery, and perinatal data included birthweight, location, patch repair and respiratory support.
MAIN OUTCOME MEASURES
The GI and respiratory scores were correlated with O/E LHR using linear and logistic regression models. Univariate analysis was used to evaluate associations with perinatal variables.
RESULTS
We obtained 142 responses from 342 families (representing a response rate of 45%). The baseline characteristics of participants and non-participants were comparable. No correlations between perinatal variables and respiratory or GI scores were identified. Children aged ≤5 years with lower O/E LHR values reported higher respiratory scores (P = 0.0175); this finding was not reported in older children. Overall, the children who underwent FETO (n = 51) had GI (P = 0.290) and respiratory (P = 0.052) scores that were comparable with those of children who were expectantly managed.
CONCLUSIONS
Families and children with prenatally diagnosed CDH reported fewer respiratory symptoms with increasing age. There was no correlation between O/E LHR or the use of FETO and self-reported outcomes.
PubMed: 38747110
DOI: 10.1111/1471-0528.17836 -
Gastroenterologia Y Hepatologia May 2024
PubMed: 38740328
DOI: 10.1016/j.gastrohep.2024.502209 -
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