Anterior Diaphragmatic Hernia Research Articles

Single-Incision Pediatric Endosurgical Morgagni Diaphragmatic Hernia Repair

Abstract Introduction: Anterior diaphragmatic hernias of the Morgagni type are the least common type of congenital diaphragmatic hernia and account for about 6% of all cases. Morgagni hernias often present as an incidental finding on a chest radiograph. Most pediatric surgeons would advocate for elective repair in asymptomatic patients. Laparoscopic repair of Morgagni hernia in children has been previously described.1–3 As the hernia itself produces minimal symptoms, postoperative cosmesis is often of great concern to the parents of the affected child. Percutaneous suturing techniques have been previously described for single-incision laparoscopic surgery. We present our technique of virtually scarless single-incision pediatric endosurgical (SIPES) repair of a Morgagni hernia. Case: The patient was a 20-month-old female child who presented to our hospital with fever and persistent cough. A chest radiograph was obtained, showing an anterior diaphragmatic hernia. The finding was confirmed on computed tomogr...

Concurrent Laparoscopic Morgagni Hernia Repair and Sleeve Gastrectomy

The Morgagni-type anterior diaphragmatic hernia is a congenital defect that is a very uncommon hernia presenting in an adult. Surgical repair is usually recommended upon diagnosis and often requires synthetic mesh for a durable, tension-free repair. The use of synthetic mesh concurrently with several of bariatric operations is controversial owing to the potential for mesh infection. In this report we describe a laparoscopic repair of a symptomatic Morgagni hernia with synthetic mesh, concurrently with sleeve gastrectomy, in a morbidly obese man. The patient was a 58-year-old man with a body mass index of 48 kg/m(2) and associated co-morbid conditions that included obstructive sleep apnea, hypertension, hyperlipidemia, impaired fasting glucose, and osteoarthritis. He was diagnosed with Morgagni hernia with exertional dyspnia. He underwent concurrent laparoscopic Morgagni hernia repair with mesh and sleeve gastrectomy. At 2 months after surgery the patient was doing well and tolerating solid foods, and his percentage excess weight loss was 35%. He was exercising regularly and had no exertional dyspnea. Laparoscopy is an attractive approach to performing multiple intra-abdominal procedures concurrently. The Morgagni hernia repair with mesh can be performed safely and effectively using a laparoscopic approach. This can be performed concurrently with bariatric surgery in the morbidly obese.

Stage IV Gastrointestinal Stromal Tumor of Colonic Interposition in Esophagus

Purpose: Gastrointestinal stromal tumors (GISTs) is the most common mesenchymal tumor of the gastrointestinal tract. GISTs predominant in the stomach (60-70%) and intestine (20-25%), but rarely occur in the esophagus (5%) and colorectum. A 47-year-old man with a history of recurrent intermittent chest pain for >6 months who was found to have a widened mediastinum on chest film. He has a history of lye ingestion in 1981 with gastric pull up and colonic interposition. He reported a 40 lbs weight loss with decreased appetite for 8 months. Review of systems was otherwise negative. Patient had fever of 101°F. Blood pressure, pulses and respiratory rate were normal. Physical exam is non-contributory. Laboratory evaluation was normal except for urine toxicology which was positive for cocaine. Chest film showed prominence of the left hilum and multiple retrosternal lucencies are likely represent bowel containing anterior diaphragmatic hernia. CT scan of chest showed a giant mediastinal mass with vessels running through it in continuity with the stomach, heart, aorta and pulmonary artery. Multiple rounded enhancing hypodensities were found in the liver in CT. Upper endoscopy revealed a large, ulcerated and cratered mass measuring 7cm in diameter and involving 75% of luminal circumference of the colonic interposition was seen 29-36cm from the incisors. EUS of the liver lesions showed well circumscribed hypoechoic masses easily accessible for FNA. FNA biopsy revealed malignant cells positive for CD34, CD117 and DOG1, consistent with GIST. Most GISTs occur in the sub diaphragmatic gastrointestinal tract; only a few cases have been reported in the esophagus. Our patient has GIST in a segment of colon that was used as an esophageal graft. Patient has been treated with Gleevec (a targeted therapy that is directed to the exact molecular abnormality that drives the GIST tumor) and followed up with CT and PET scans, which are sensitive for evaluating the extent of tumor progression. This is the first case of a giant metastatic GIST that involved a colonic interposition.Figure: [577] CT scan of chest.

Incarcerated Morgagni hernia mimicking acute cholecystitis

A 64-year-old man presented with sharp, constant right upper quadrant pain, nausea, and constipation for 2 days. The patient reported intermittent right upper quadrant pain for the prior 6 years, which was exacerbated by eating, but noted that over the past 2 days the pain had become more severe. The patient had previously undergone three right upper quadrant ultrasound studies that were all negative for acute cholecystitis. On presentation, examination was notable for focal tenderness to palpation over the xiphoid process. A right upper quadrant ultrasound study was completed, and demonstrated a single hepatic cyst and gallbladder sludge without evidence of gallstones, gallbladder wall thickening, pericholecystic fluid, or a sonographic Murphy’s sign (Fig. 1). A chest radiograph was obtained, and demonstrated elevation of the right hemidiaphragm with colonic interposition in the right upper quadrant (Fig. 2). Computed tomography of the abdomen and pelvis with intravenous and oral contrast was performed, and demonstrated a large right-sided anterior diaphragmatic hernia containing distended loops of colon with short segment narrowing at the entrance as well as soft tissue stranding and simple fluid within the herniation sac, consistent with an incarcerated Morgagni hernia (Fig. 3). The patient underwent laparoscopic diaphragmatic hernia repair with mesh, which was complicated by postoperative development of a right pneumothorax, but with eventual normalization of the chest radiograph (Fig. 4) and resolution of the right upper quadrant pain.

Laparoscopic repair of iatrogenic anterior diaphragmatic hernia

Congenital diaphragmatic hernia is a relatively common condition with an incidence of about 1 in 2000 to 1 in 5000 live births. Most cases occur posterolaterally through the foramen of Bochdalek and usually present immediately after birth with respiratory distress. Congenital anterior diaphragmatic hernia (Morgagni's hernia) is rare, accounting for less than 5% of all types of congenital diaphragmatic hernias. Most of these cases are seen in older children and are usually asymptomatically discovered incidentally or during investigations for some other unrelated condition. Iatrogenic anterior diaphragmatic hernia on the other hand is extremely rare. This report describes an iatrogenic anterior diaphragmatic hernia in a 6-month-old child following cardiac surgery that was repaired laparoscopically. The literature on the subject is also reviewed. Key words: Anterior diaphragmatic hernia, Iatrogenic, laparoscopic repair.

Cantrell‘s Syndrome

Cantrell s syndrome is characterized by: 1) Omphalocele 2) Sternal cleft 3) Anterior diaphragmatic hernia 4) Ectopia cordis 5) Intracardiac defects With an incidence of 5 to 8 cases per million of newborn babies, this syndrome is caused by mesodermal defects in early stages of the embrionary period. There are two ways of presentation: complete and incomplete. The incomplete way is when the heart is covered by skin, pericardium or both of them. In the complete way, the sternum is absent or it has a wide defect. There is no parietal pericardium and the heart is totally out of the thorax with its apex upwards and there is an important reduction in the size of the thoracic cavity. Generally, with omphalocele. 80% of the cases with incomplete way present congenital heart defect and in the complete way this percentage is 100%. Truncoconal abnormalities are the most usual. The studied case had interventricular communication (IVC) and the emergence of the great vessels was difficult to observe.

Intrapericardial diaphragmatic hernia: a rare type of congenital diaphragmatic hernia

An extremely rare case of congenital intrapericardial diaphragmatic hernia is presented. Since 1981, only 14 cases have been reported in the literature. A 5-year-old girl presented with dyspnea on exertion and easy fatigability. Computed tomography was suggestive of an anterior diaphragmatic hernia. Laparoscopy followed by successful open repair of hernia was performed.

Co‐occurrence of severe Goltz–Gorlin syndrome and pentalogy of Cantrell – Case report and review of the literature

Goltz-Gorlin syndrome is a highly variable disorder affecting many body parts of meso-ectodermal origin. Mutations in X-linked PORCN have been identified in almost all patients with a classical Goltz-Gorlin phenotype. The pentalogy of Cantrell is an infrequently described congenital disorder characterized by the combination of five anomalies: a midline supra-umbilical abdominal wall defect; absent or cleft lower part of the sternum; deficiency of the diaphragmatic pericardium; deficiency of the anterior diaphragm; and congenital heart anomalies. Etiology and pathogenesis are unknown. We report on an infant with findings fitting both Goltz-Gorlin syndrome (sparse hair; anophthalmia; clefting; bifid nose; irregular vermillion of both lips; asymmetrical limb malformations; caudal appendage; linear aplastic skin defects; unilateral hearing loss) and the pentalogy of Cantrell (absent lower sternum; anterior diaphragmatic hernia; ectopia cordis; omphalocele). The clinical diagnosis Goltz-Gorlin syndrome was confirmed molecularly by a point mutation in PORCN (c.727C>T). The presence of molecularly confirmed Goltz-Gorlin syndrome and pentalogy of Cantrell in a single patient has been reported twice before. The present patient confirms that the pentalogy of Cantrell can be caused in some patients by a PORCN mutation. It remains at present uncertain whether this can be explained by the type or localization of the mutation within PORCN, or whether the co-occurrence of the two entities is additionally determined by mutations or polymorphisms in other genes, environmental factors, and/or epigenetic influences.

A maternally inherited chromosome 18q22.1 deletion in a male with late‐presenting diaphragmatic hernia and microphthalmia–evaluation of DSEL as a candidate gene for the diaphragmatic defect

Using an Affymetrix GeneChip(R) Human Mapping 100K Set to study a patient with a late-presenting, right-sided diaphragmatic hernia and microphthalmia, we found a maternally inherited deletion that was 2.7 Mb in size at chromosome 18q22.1. Mapping of this deletion using fluorescence in situ hybridization revealed three deleted genes-CDH19, DSEL, and TXNDC10, and one gene that contained the deletion breakpoint, CCDC102B. We selected DSEL for further study in 125 patients with diaphragmatic hernias, as it is involved in the synthesis of decorin, a protein that is required for normal collagen formation and that is upregulated during myogenesis. We found p.Met14Ile in an unrelated patient with a late-presenting, anterior diaphragmatic hernia. In the murine diaphragm, Dsel was only weakly expressed at the time of diaphragm closure and its expression in C2C12 myoblast cells did not change significantly during myoblast differentiation, thus reducing the likelihood that the gene is involved in myogenesis of the diaphragm. Although it is possible that the 18q22.1 deletion and haploinsufficiency for DSEL contributed to the diaphragmatic defect in the patient, a definite role for DSEL and decorin in the formation of the collagen-containing, central tendon of the diaphragm has not yet been established.

Pentalogy of Cantrell: six new cases

Cantrell's pentalogy is a rare multiple malformation syndrome consisting of supraumbilical abdominal wall defect, sternal defect, pericardial defect, anterior diaphragmatic defect and heart malformation. Prenatal ultrasound findings and malformations described during autopsy of the Cantrell's pentalogy cases diagnosed between January 1991 and June 2009 in our institute were reviewed. A literature research was conducted to analyze the prevalence and prenatal detection rate of the five previously described malformations and ectopia cordis in the Cantrell's pentalogy cases. Six cases of Cantrell's pentalogy were diagnosed during the study period in our department. Sonography detected multiple malformations in 3 cases, and isolated malformation in 3 cases. Analyzing the data of 49 Cantrell's pentalogy cases altogether showed that, beside abdominal wall defect and ectopia cordis which had the highest prenatal detection rate (83% and 91% with a prevalence of 94% and 69%, respectively), sternal defect and anterior diaphragmatic hernia were also present in a large number of the cases (80% and 73% respectively). Sonographic identification of the sternal defect or diaphragmatic hernia may help to differentiate Cantrell's pentalogy from malformations part of the syndrome but occurring as isolated defects.

Concomitant Exencephaly and Limb Defects Associated with Pentalogy of Cantrell

A woman, aged 42, gravida 5, para 2, presented with a dichorionic twin pregnancy and an abnormal fetus at 16 weeks’ gestation. The parents were non-consanguineous and healthy. The father was aged 46 years. The mother denied any recent infections or exposure to teratogens and had not experienced any assisted reproductive technology. She had two healthy sons and had suffered two spontaneous abortions. There was no family history of congenital malformations or of diabetes mellitus. Amniocentesis revealed a 46,XX karyotype in the abnormal fetus and a 46,XY karyotype in the normal twin. Sonographic examination of the abnormal fetus revealed scoliosis, ectopia cordis, a ventricular septal defect, a large ventral wall defect with protruding heart, liver, spleen and bowel loops, exencephaly, acranium, shortening of the left upper limb, and arthrogryposis of the left wrist and bilateral ankles. The umbilical cord appeared normal, and there was no amniotic band. A diagnosis of pentalogy of Cantrell with exencephaly and limb defects was established. The co-twin was normal. The parents elected to continue the pregnancy. Preterm labor occurred at 32 weeks’ gestation. The 1,760-g normal male co-twin survived, while the 1,098-g abnormal female fetus (Figure) died after delivery. An autopsy confirmed a defect of the lower sternum, a deficiency of the diaphragmatic pericardium, an anterior diaphragmatic hernia, a ventricular septal defect, scoliosis, hypoplasia of the left upper limb, a deficiency of the left thumb, and arthrogryposis of the left wrist and bilateral ankles in the abnormal fetus. Her face, toes and other fingers, except the left thumb, were normal. No amniotic band could be found in the fetus and placenta.

Iatrogenic Anterior Diaphragmatic Hernia in Childhood

Congenital anterior diaphragmatic hernias (ADH) account for 2 - 6 % of diaphragmatic defects, whereas acquired ADH are rare. These hernias are most often the result of blunt or penetrating trauma. This is the first report of iatrogenic ADH in childhood. Two children with asymptomatic, iatrogenic ADH were diagnosed at 6 and 12 months of age, respectively. Both had undergone previous cardiac surgery requiring pericardial drainage. The ADH was seen on an incidental chest X-ray in one patient, and during a laparoscopic-assisted gastrostomy in the other. Both were successfully repaired laparoscopically. Subxiphoid pericardial drains may result in iatrogenic ADH. A high index of suspicion after cardiac surgery is necessary to avoid missing this potentially serious complication. Laparoscopy is a useful tool in both the diagnosis and repair of this condition.

Sternal cleft, Morgagni hernia, and ectopic liver: a unique chest wall anomaly

Sternal clefts are rare chest wall anomalies which may occur in isolation or in association with cardiac, pericardial, and diaphragmatic defects. We present the first report of a unique chest wall anomaly consisting of a triad of sternal cleft, anterior diaphragmatic hernia, and ectopic, exophytic liver tissue. A partial diagnosis was made on prenatal ultrasound imaging and computed tomography imaging of the baby's chest, but the full extent of the anomaly was only identified at operation. Full repair was completed on the third day of life with excellent outcome.

Asymptomatic Congenital Intrapericardial Diaphragmatic Hernia and Epigastric Hernia in the Adult

The congenital intrapericardial hernia is a rare kind of diaphragmatic hernia. It is due to an embryologic defect of the central tendon of the diaphragm, often accompanied by other congenital malformations. This work presents a unique case report in the literature of the congenital association between intrapericardial diaphragmatic hernia and epigastric hernia in an adult woman. In spite of herniation of the colon and omentum the patient was completely asymptomatic, requesting surgery for an epigastric hernia for aesthetic reasons. The defect of the diaphragm was sutured and the abdominal wall was repaired with a prosthetic mesh.

Phenotype resembling Donnai–Barrow syndrome in a patient with 9qter;16qter unbalanced translocation

We describe a 3-year-old boy with complete agenesis of corpus callosum, developmental delay/mental retardation, anterior diaphragmatic hernia, Morgagni type, severe hypermetropia, and facial dysmorphism suggesting the diagnosis of Donnai-Barrow syndrome. Subtelomeric FISH analysis revealed a paternally-derived t(9;16) (q34.3;q24.3) translocation with partial 9q monosomy and partial 16q trisomy. As some facial features resemble the 9q emerging phenotype, we suggest the hypothesis that some patients with Donnai-Barrow syndrome might be abscribed to 9q terminal deletion.

Sternotomy Reconstruction with Omentum Followed by Large Diaphragmatic Hernia

Poststernotomy mediastinitis carries significant morbidity and mortality. Aggressive wound debridement combined with a pedicled omental flap, with or without a pedicled muscle flap, has gained acceptance in the management of difficult sternal wound infections. Two cases of poststernotomy mediastinitis and sternal wound reconstruction with a pedicled omental flap were complicated by a large anterior diaphragmatic hernia containing the large bowel.

Massive Recurrent Anterior Diaphragmatic Hernia, Coronary Artery Disease, and Valvular Heart Disease

We report the case of a 76-year-old woman found on preoperative evaluation for vaginal prolapse to have coronary artery disease, aortic stenosis, and a large recurrent anterior diaphragmatic hernia. The clinical presentation and surgical management of this case, and the review of the literature are discussed.

A Newborn Who Has an Abdominal Wall Defect

A newborn female born at 35 weeks gestational age presents with an abdominal wall defect. ### Prenatal History: ### Presentation: Figure 1. Figure 2. The appearance of the abdominal wall was grossly abnormal and …

Anterolateral Diaphragmatic Hernia: Is it a Morgagni Hernia?

Three unusual cases of large anterolateral congenital diaphragmatic hernia are described with emphasis on its embryogenesis. This type of diaphragmatic defect is rare and underemphasized. Its clinical spectrum ranges from the asymptomatic case to those with acute respiratory distress. It may present itself as a solitary defect or as part of a more complex association such as the thoracoabdominal syndrome. This defect should be differentiated from Morgagni (and other anterior) diaphragmatic hernias by its size and lateral extension and the presence of left pulmonary hypoplasia. Treatment is by primary operative repair, and prognosis depends on the presence and severity of associated malformations.

Diaphragmatic hernia in Down's syndrome

Three Saudi children with Down's syndrome were found to have anterior diaphragmatic hernias. They all needed surgical correction. In Down's syndrome, besides congenital heart disease and recurrent chest infection, a diaphragmatic hernia should be looked for as a possible cause of respiratory distress and chest deformity.