Congenital Diaphragmatic Eventration Research Articles

Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study

Congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH) with or without hernia sac are three different types of congenital diaphragmatic malformations, which this study evaluates. All surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016 were included in this retrospective analysis. Demographics, CDH-characteristics, treatment, and clinical outcome were evaluated. In total, 200 patients were included. Patients with an eventration or hernia sac had no significant differences and were compared as patients without a true defect to patients with a true defect. The 1-year survival of patients with a true defect was significantly lower than patients with no true defect (76% versus 97%, p = 0.001). CDH with no true defect had significantly better short-term outcomes than CDH with true defect requiring patch repair. However, at 30 days, they more often required oxygen supplementation (46% versus 26%, p = 0.03) and had a higher recurrence rate (8% versus 0%, p = 0.006) (three eventration and two hernia sac patients). Conclusion: Patients without a true defect seem to have a more similar clinical outcome than CDH patients with a true defect, with a better survival. However, the recurrence rate and duration of oxygen supplementation at 30 days are higher than CDH patients with a true defect.What is Known:• Congenital diaphragmatic hernia with or without hernia sac and congenital diaphragmatic eventration (incomplete muscularization) are often treated similarly.• Patients with hernia sac and eventration are thought to have a relatively good outcome, but exact numbers are not described.What is New:• Congenital diaphragmatic eventration and patients with hernia sac seem to have a more similar clinical outcome than Bochdalek type CDH with a true defect.• Patients without a true defect (eventration or hernia sac) have a high recurrence rate.

Thoracoscopic repair of diaphragmatic eventration in children: a comparison of two repair techniques

BackgroundThoracoscopic plication has gained popularity in the management of diaphragmatic eventration, and several suturing techniques have been described. However, the superiority of one technique over the other has not been demonstrated. The purpose of this study is to report our experience with diaphragmatic plication and to compare the thoracoscopic interrupted and pleated suture techniques in pediatric patients with diaphragmatic eventration. MethodsThis is a retrospective cohort study (level of evidence: 3) performed on 14 patients with diaphragmatic eventration. All patients were symptomatic and had diaphragmatic plication via thoracoscopy. The patients were further divided into two groups according to the repair technique; interrupted repair (n = 9) and pleated repair (n = 5). Preoperative, operative and postoperative data were compared between the two groups. ResultsThe median age was 9.5 months (25th- 75th percentiles: 6 to 15 months), and 8 (57%) were males. Twelve patients (85.71%) had right side eventration, and nine patients (64.29%) had congenital diaphragmatic eventration. One case was converted to open thoracotomy because of adhesions. There was no difference in the preoperative characteristics between both groups. Median operative time was 117 min (25th- 75th percentiles: 101–129 min) and 77 min (25th- 75th percentiles: 73–83 min) in the interrupted and pleated groups, respectively (p = 0.004). One patient had a postoperative elevation of the diaphragm (incomplete repair) in the pleated group (p = 0.357). No recurrence was reported during the follow-up. ConclusionThoracoscopic plication is an effective technique for management of diaphragmatic eventration in children. Pleating technique is easy, fast, and associated with a marked reduction in operative time. Type of the studyRetrospective cohort study. Levels of EvidenceLevel of evidence: 3

Congenital diaphragmatic eventration with absent left phrenic nerve in the fetal pig.

We encountered a fetal pig with eventration of the diaphragm and pulmonary hypoplasia accompanied by phrenic nerve agenesis. The fetal pig was female measuring 34cm in crown-rump length and about 1500g in body weight. The diaphragm was a complete continuous sheet, but comprised a translucent membrane with residual muscular tissue only at the dorsolateral area of the right leaf of the diaphragm. The left leaf protruded extraordinarily toward the thoracic cavity. The left phrenic nerve was completely absent, while there was a slight remnant of the right phrenic nerve that supplied the dorsolateral muscular area of the right leaf. Both lungs were small, and the number of smaller bronchioles arising from the bronchioles was decreased to about half of that of the normal lung. Additionally, the right and left subclavius muscles and nerves could not be identified. These findings imply that the diaphragm, the subclavius muscle and nerves innervating them comprise a developmental module, which would secondarily affect lung development. It is considered that the present case is analogous to the animal model of congenital eventration of the diaphragm in humans.

Congenital Cystic Diaphragm with Diaphragmatic Eventration in a Fetus: A Case Presentation

Introduction: Congenital diaphragmatic eventration (CDE) is defined as the abnormal elevation of the diaphragm, due to incomplete muscularization of the diaphragm with a thin membranous sheet replacing normal diaphragmatic muscle. Case report: We report a prenatal case with a diaphragmatic mesothelial cyst combined with CDE. Conclusion: A large cystic mass between the thoracic wall and the liver in early pregnancy is highly suggestive of cystic diaphragm.

Ultrasound-guided perioperative management of 28-month-old patient with congenital diaphragmatic eventration

Diaphragmatic eventration is a rare anomaly. When patients with this condition undergo general anesthesia, anesthetic management should be performed with particular care owing to the risk of diaphragmatic rupture. Such a rupture can be perioperatively diagnosed using multiple tools including lung ultrasonography. This case report describes the anesthetic management of a male infant with osteochondroma in the distal ulna, presenting with diaphragmatic eventration on the right side.

An Unusual Presentation of an Adult with Diffuse Abdominal Pain Related to Congenital Diaphragmatic Hepatic Eventration

This case report identifies a uncommon presentation of a congenital diaphragmatic hepatic eventration in a middle aged female adult with an acute onset of diffuse abdominal pain. This case highlights a significant congenital event which is not commonly found as an adult. However, this case is interesting, as many clinicians may be puzzled over the radiologic presence, leading to numerable diagnostic studies and unnecessary consultations. A 43 year old Hispanic female with history of hypertension, diabetes and dyslipidemia presented to ER with complaint of diffuse abdominal pain, all quadrants of abdomen with no guarding or rigidity, normal bowel sounds. Routine labs showed no leukocytosis, normal metabolic panel except an elevated Cr of 1.7. CT Abdomen was done without any contrast in light of acute kidney injury, which showed a soft tissue mass at right lung base measuring 4.4 cm in diameter. Thought to be part of the hepatic dome pushing the diaphragm to the lung base versus, albeit less likely, a pulmonary mass, a CT with contrast was recommended. The patient was given symptomatic treatment in ER and was discharged with outpatient follow up. After her kidney function returned back to baseline CT abdomen with contrast showed focal eventration of the right hemidiaphragm with liver tissue extending through the eventration and no other suspicious mass. The patient was seen in GI clinic with impression of likely congenital anomaly. The patient was completely asymptomatic at that time and was conservatively managed with regular follow up. Congenital diaphragmatic eventration has been frequently cited in literature as a common condition in infancy, requiring urgent care and observation. However is a rare anomaly found in an adult. Eventation of the diaphragm is defined anatomically by thinning of the diaphragmatic tissue, characterized by the cephalic displacement an intact diaphragm. This entity is frequently asymptomatic and initially detected by chest x-ray. Adults can occasionally complain of progressive dyspnea, chest pain, abdominal discomfort, nausea/vomiting, heartburn. A differential must still be considered, including; diaphragmatic hernia, paraesophageal hernia, hiatus hernia, bronchogenic cyst, teratoma, cardiac sarcoma, lymphosarcoma, schwannoma, or metastatic tumor. Asymptomatic patients are usually managed with surveillance, and if persistently symptomatic managed with plication of the diaphragm with thoracotomy.2340_A Figure 1. Chest CT with IV Contrast- Sagittal view with hepatic eventration extending into thoracic cavity.2340_B Figure 2. Chest CT with IV Contrast- Coronal view with hepatic eventration extending into thoracic cavity.

A Rare Case of Partial Diaphragmatic Eventration with Hepatic Herniation

A 43 year old Female with past history of hypertension, diabetes mellitus presented to the Emergency room with a chief complaint of left flank pain. Her physical examination was notable for left sided flank tenderness with no abdominal rigidity or guarding. Routine blood work showed normal complete blood count, with normal comprehensive metabolic profile. A chest xray showed a soft tissue mass at the right lung base. Further contrast Computed Tomography(CT) of the chest and abdomen showed an incidental soft tissue mass at right lung base that measured 4.4 cm in diameter representing a focal eventration of the right hemi-diaphragm with liver tissue extending through the eventration.(Fig 1,2).2343_A Figure 1. Arrow depicting hepatic herniation2343_B Figure 2. Coronal CT showing hepatic mass extending through diaphragamDiaphragmatic eventration was first recognized by Jean Louis Petit in 1774 and the term was coined by Beclard in 1916. It is defined anatomically by thinning of the diaphragmatic tissue, characterized by the cephalic displacement of all or a part of an intact diaphragm. While complete eventration in more common on left, partial eventration is seen majorly on the right side. Congenital diaphragmatic eventration has been frequently cited as common in infancy, requiring urgent care and observation but it is rarely seen in adults. Typically this entity is frequently asymptomatic but can rarely manifest as progressive dyspnea, chest pain, abdominal discomfort, nausea, vomiting, heartburn and recurrent pneumonia. Partial eventration are usually detected incidentally on chest x-ray as a smooth homogeneous mass in continuation with the diaphragm.Ultrasound may reveal more anatomical detail about the diaphragmatic integrity, specifically eventration content or other diaphragmatic pathologies.If a contrast CT scan is performed, hepatic or omental vascular enhancements are seen as hyper-dense areas. Other modalities such as fluoroscopy with sniff testing and Magnetic resonance imaging are done if the diagnosis is still in doubt. Once diagnosed asymptomatic patients are usually managed conservatively and diaphragmatic plication is done in symptomatic patient. Our patient's left sided pain was musculoskeletal and not related to the diaphragmatic eventration. Her pain resolved with analgesics and she has remained asymptomatic on follow up visits. In summary we present a rare case of partial diaphragmatic eventration with hepatic herniation. Our case highlights conservative management in asymptomatic patients with diaphragmatic eventration.

Repair of Congenital Diaphragmatic Hernia and Eventration Of Diaphragm Without Ventilatory Support

Background: Congenital Diaphragmatic Hernia (CDH) and eventration of Diaphragm(ED) are important causes of respiratory distress in children. CDH and ED are more a medical emergency than a surgical one and after birth, confirmation of the diagnosis should be followed by treatment in a Neonatal Intensive Care Unit (NICU) or Special Care Neonatal Unit (SCANU), Paediatric Intensive Care Unit (PICU) to stabilize the cardiovascular system. Preoperative resuscitation and delayed surgical repair with or without the use of ExtraCorporeal Membrane Oxygenation (ECMO) improves the survivality.
 Methods: The medical records of all patients with CDH and ED between January 2010 and December 2015 were retrospectively reviewed. Patients’ presentation, management, operative findings and complications were evaluated.
 Results: 22 patients were diagnosed as CDH or ED. Male to female ratio was 4.5:1. Age range was 2 days to 7 years, median 82.5 days. Eighteen patients were CDH and 4 patients were ED. Six patients had associated malrotation of the gut. 3 patients had Congenital Heart Disease, 1 patient had Gastroschisis and another had Hiatus hernia. Four patients expired.
 Conclusion: Surgery preceded by a preoperative respiratory resuscitation and stabilization reduces postoperative mortality and increases the survival rate. Outcome was not very unsatisfactory without adequate ventilatory support.
 Journal of Paediatric Surgeons of Bangladesh (2016) Vol. 7 (1): 3-10

Spontaneous Rupture of a Congenital Diaphragmatic Eventration in an Infant

Rupture of eventration of diaphragm is a rare entity that commonly presents as sudden onset respiratory distress. We report a case of a 7-month-old infant with congenital diaphragmatic eventration having spontaneous diaphragmatic rupture which led to secondary diaphragmatic herniation with gastric volvulus. Clinical diagnosis of gastric volvulus was suspected on the basis of imaging and failed entry of nasogastric tube into stomach. Exploratory laparotomy revealed rupture of eventration of diaphragm and gastrothorax with volvulus. Anatomical repair of diaphragmatic perforation followed by diaphragmatic plication resulted in successful outcome in our patient.

OP04.02: Congenital diaphragmatic hernia and diaphragmatic eventration: separate entities or two sides of the same coin?

OP04.02: Congenital diaphragmatic hernia and diaphragmatic eventration: separate entities or two sides of the same coin?

Intrathoracic Gastric Volvulus presenting with GIT Bleed.

Intrathoracic gastric volvulus in neonatal period is a life-threatening surgical emergency. We report a case of neonate with respiratory distress and GI bleeding who was diagnosed to have congenital diaphragmatic eventration with Intrathoracic gastric volvulus.

Synchronous Morgagni and Bochdalek Hernias: A Case Report of a Unique Approach to a Rare Finding

Introduction: Multiple unilateral congenital diaphragmatic hernias (CDH) are extremely rare, described only five times in the medical literature. Concurrent ipsilateral Bochdalek and Morgagni hernias are rarer still with only two cases previously described. In all reported cases of multiple concurrent defects, the hernias were repaired in an open fashion, either via a thoracotomy or laparotomy with both of the two combined Bochdalek and Morgagni hernias repaired via laparotomy. Case Presentation: In this case report we have a 2-day-old who developed respiratory distress and on CT scan was found to have a congenital diaphragmatic hernia (CDH) or eventration. This patient is ideal for this case report because he meets a lot of the previously established criteria for minimally invasive repair of congenital diaphragmatic hernias - minimal respiratory compromise, no congenital heart defects - and he has synchronous defects which have very rarely been seen before. Here we present the first reported case of concurrent ipsilateral Bochdalek and Morgagni hernias repaired in a one-stage minimally invasive fashion, approaching the Bochdalek hernia thoracoscopically and the Morgagni laparoscopically. The patient had a quick recovery post-operatively and he continues to do well. Conclusions: From this experience, we argue that in the right circumstances a completely minimally invasive approach can be taken for synchronous congenital diaphragmatic hernias.

Congenital diaphragmatic eventration in children: 12 years' experience with 177 cases in a single institution

ObjectiveThis study sought to summarize the diagnostic and treatment aspects of congenital diaphragmatic eventration (CDE) in children by retrospectively analyzing their medical records to identify and understand the complications of CDE, its treatment, and to evaluate the long-term outcomes of diaphragmatic plication. MethodsThe medical records of children who received treatment for CDE from January 2000 to December 2011 at the Children's Hospital of Chongqing Medical University, China were analyzed. Data analyzed included the following: age, sex, symptom, location of eventration, associated anomalies, surgical procedures, complications, and survival and follow up details after diaphragmatic plication. ResultsThe medical records of 177 children (boys: 128, girls: 49, mean age: 10.28±2.35months) with CDE were included in this study. Specific symptoms of eventration of the diaphragm were reported for 86 cases; and the typical symptoms included rapid breathing, vomiting, and recurrent respiratory infections. Except for a bilateral case, all the other patients had unilateral CDE. Associated malformations were observed in 31 cases (17.5%), hypoplastic lung (10 cases) was the most common followed by congenital heart disease (9 cases), and cryptorchidism (3 cases). Interestingly, 91 patients were asymptomatic. Diaphragmatic plication was performed in all symptomatic patients (86 cases, 48.5%) and none had recurrence. ConclusionsClinical symptoms of CDE varied in severity, ranging from asymptomatic conditions to life-threatening respiratory distress. Timely accurate diagnosis and treatment of symptomatic CDE could effectively resolve respiratory morbidity and reduce complications. The diaphragm plication surgery provided good results among the study population with no recurrence.

Thoracoscopic Treatment of Congenital Diaphragmatic Eventration in Children: Lessons Learned After 15 Years of Experience

We present our experience with the thoracoscopic treatment of congenital diaphragmatic eventration (CDE) in children through 15 years to evaluate the efficiency of the procedure and the potential risk of recurrence. Materials and We reviewed the medical files of patients treated for CDE through thoracoscopy from 2000 to 2011. Age at surgery, sex, side of the lesion, procedure's details, postoperative course, and complications were analyzed. Mean follow-up was 12 months. In this study, eight patients (five males and three females) aged from 6 months to 7 years underwent thoracoscopic plication for six right and two left eventrations; one conversion was necessary due to a too small operative field. Mean operative time was 60.5 minutes. A chest drainage was placed in six patients. We observed two recurrences from which the first one was treated thoracoscopically by endostapler resection/suturing and the other one by laparotomy. At follow-up, all patients were asymptomatic with a correct level of the diaphragm. Thoracoscopic plication is feasible and safe, and we consider this approach as the gold standard for the treatment of CDE. However, we still need to carefully consider the possibility of introducing certain modifications to reduce the potential risk of recurrence.

Bilateral eventration of the diaphragm: report of a case and reviw of the literature

Introduction. Diaphragmatic eventration is a congenital malforma- tion which could result in respiratory distress. A chest X-ray, shows an elevated diaphragm. Most case reports present unilateral dia- phragmatic eventration. We report a case of an infant with bilateral diaphragmatic elevation. Case report. A 17 day old infant presented with respiratory distress. With the aid of an Xray study we diagnosed a bilateral congenital eventration of the diaphragm (BCED). A bilateral thoracotomy was performed. Discussion. Congenital diaphragmatic eventration represents 5% of all diaphragmatic pathology. There have been few reports of bilateral congenital diaphragmatic eventration. We found only nine reported cases since 1973, and only one in Mexico and Latin America. A bilateral diaphragmatic placation in our patient was successful.

Congenital Eventration of Hemidiaphragm in an Adult

Congenital diaphragmatic eventration is a common condition in infancy but is a rare anomaly in an adult. The muscle is permanently elevated, but keeps its continuity and attachments to the costal margins, which differentiates diaphragmatic eventration from diaphragmatic hernia. We describe a case of congenital eventration of the left diaphragm in a 35-year-old male who underwent successful surgical repair.

Prenatal diagnosis of right diaphragmatic eventration associated with fetal hydrops

Congenital diaphragmatic eventration (CDE) is a rare diaphragmatic abnormality. Clinical manifestations of CDE may mimic congenital diaphragmatic hernia. Prenatal differential diagnosis of eventration is critical because postnatal managing and prognosis of these conditions vary significantly. Sonographic features of CDE involve presence of abdominal organs in the thorax, shift of cardiac axis and mediastinum. Non-immune hydrops fetalis (NIHF) has been previously reported to be associated with intrathoracic masses as well as CDE. In this report, we present a case of congenital right diaphragmatic eventration associated with NIHF.

Anesthetic management of a patient with congenital diaphragmatic eventration

Congenital diaphragmatic eventration is uncommon in adults and is caused by paralysis, aplasia or atrophy of the muscular fibers of the diaphragm. It may cause severe dyspnea, orthopnea and hypoxia in adult patients. Most symptomatic patients may be managed efficiently without the need for surgical correction, although any event that leads to an increase in intra-abdominal pressure puts them at the risk of spontaneous diaphragmatic rupture. This case report presents the successful anesthetic management of an adult female with congenital diaphragmatic eventration undergoing diagnostic laparoscopy and hysteroscopy using a total intravenous anesthesia technique. Essential steps to prevent any rise in intrathoracic and intra-abdominal pressures along with care to minimize intragastric volume were taken.

Left Diaphragmatic Eventration Associated with Ipsilateral Pulmonary Sequestration and Intrathoracic Kidney in a Fetus: Reviewing the Prenatal Diagnosis and Etiopathogenesis

The prenatal diagnosis of congenital diaphragmatic eventration, pulmonary extralobar sequestration and intrathoracic ectopic kidney in a single fetus is not reported yet according to the pubmed. Congenital diaphragmatic eventration is an abnormal elevation of the diaphragm. Differential diagnosis from hernia is essential for the perinatal management. Extralobar sequestrations are usually asymptomatic and detected incidentally. Intrathoracic kidney is an extremely rare congenital anomaly. Genitourinary and cardiac anomalies should be searched as common co-existing malformations. Besides prenatal ultrasound, fetal magnetic resonance imaging has a substantial support in counselling the family, planning the follow-up of the pregnancy and decision-making for the perinatal management.

Congenital diaphragmatic eventration in two closely related British Shorthair cats

Two closely related British Shorthair cats, which died after surgery performed in the dorsal position, were presented for dissection. In both, a thin, flaccid, enlarged transparent tendinous diaphragmatic portion protruded cranially into the thoracic cavity, forming a cupola in which left, right medial and quadrate hepatic lobes were encased in both cats and the stomach in one cat. Microscopically, no muscle fibres were observed in the membrane, but numerous hepatocytes and bile ducts were incorporated in its central part. The anomaly was diagnosed as a congenital diaphragmatic eventration.