Bochdalek hernia is a rare congenital condition. Read this lesson to learn more about this specific type of hernia, common characteristics of the condition, and how to treat it. What is a Hernia? Joan, a middle school teacher, has recently started suffering from abdominal pain. Eager to find some relief from the ongoing and increasing discomfort, she makes an appointment with her doctor.
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Last Update: September 21, Introduction Bochdalek hernia is a developmental defect in the posterolateral diaphragm, allowing herniation of abdominal contents into the thorax causing mechanical compression of the developing lung parenchyma and sometimes causing lung hypoplasia.
As such, symptoms typically manifest in the pediatric age group and tend to be respiratory. Exact etiology is unknown, though, both environmental and genetic components are favored.
Surgical repair of the diaphragmatic defect is often the treatment. There are multiple chronic conditions associated with Bochadelk hernia and congenital diaphragmatic hernia CDH which require surveillance and long-term follow-up from a team of clinicians.
Etiology It is postulated that both environmental and genetic factors contribute to the etiology of a Bochdalek hernia.
The aforementioned affected genes discovered include multiple transcription factors and the retinoic acid pathway. Most of these appear to have pleiotropic effects and variable penetrance causing a range of phenotypes.
A Bochdalek hernia is a type of congenital hernia due to a developmental defect in the formation of the diaphragm. At around 4 weeks of gestation in humans, the diaphragm starts to form from several infoldings. Anteriorly, the central tendon is proposed to form from the septum transversum.
Posterolateral infoldings form the pleuroperitoneal membranes. In the pediatric age group, the presentation is often with respiratory symptoms from lung hypoplasia after mechanical compression of developing lung parenchyma in utero.
In contrast, Bochdalek hernias in adults are usually asymptomatic and incidentally found. Symptoms in adults often manifest as vague or nonspecific gastrointestinal complaints.
More severe cases could show a scaphoid abdomen, as bowel contents herniate into the thorax. Evaluation Ultrasonography, plain film chest radiography, and thin section CT with multiplanar reformats all play a role in the diagnosis of a Bochdalek hernia. In fact, a congenital diaphragmatic hernia is the most commonly reported indication for thoracic prenatal MRI. Post-natally, ultrasonography can delineate the entire diaphragm, assessing for discontinuity, as well as evaluating the viscera that has herniated.
However, sensitivity on plain film chest radiography is low, and congenital diaphragmatic hernias can be confused for other thoracic pathology.
On Chest CT, one can more accurately assess for focal defects in the diaphragm. Sometimes, in severe herniation, Fetoscopic endoluminal tracheal occlusion FETO therapy can be considered. This procedure temporarily blocks the trachea, and preliminary research suggests it improves lung development. PPH is also a sequela of congenital diaphragmatic hernia and mechanical compression of the lung during development.
However, there are other considerations such as congenital diaphragmatic eventration, diaphragmatic paralysis, and extralobar pulmonary sequestration, which are detectable during pregnancy.
Congenital diaphragmatic eventration is thought to be secondary to defective development of the diaphragmatic musculature with subsequent focal thinning. Whereas, extralobar pulmonary sequestration is lung parenchyma with separate pleural and vascular supply. Some research suggests there is an overall increase in patient survival, thought secondary to advances in critical care.
Other pulmonary morbidities include reactive airway disease, reduced airway disease, and recurrent lung infections. Many of these patients demonstrate improved pulmonary function to near normal in adulthood, though some adult survivors show persistent impairment on pulmonary function testing.
Musculoskeletal abnormalities such as pectus deformities and scoliosis have associations with CDH. Hernia recurrence has also been reported, with an increased size of the defect correlating with increased risk for recurrence. In fact, most adult patients are asymptomatic and require no intervention. Majority of symptomatic patients obtain their diagnosis at a young age, and increasingly, antenatally.
Increased attention and continued surveillance in utero are now possible. Vigilance on screening, and subsequently, birth at a tertiary care center to ensure the appropriate support is essential. Multiple comorbidities have been identified, and long-term follow-up is necessary as the population of CDH survivor grows. The care and monitoring of these patients are by ICU nurses who work with the team to evaluate the progression of improvement and identify untoward changes as quickly as possible.
Appropriate surveillance, follow-up and possibly postsurgical care are imperative for the management of this condition. References 1. Congenital diaphragmatic hernias: from genes to mechanisms to therapies. Dis Model Mech. Leeuwen L, Fitzgerald DA. Congenital diaphragmatic hernia. J Paediatr Child Health. Birth Defects Res. Symptomatic Bochdalek hernia in an adult. Gale ME. Bochdalek hernia: prevalence and CT characteristics. Keijzer R, Puri P. Alam A, Chander BN. Adult Bochdalek Hernia.
Med J Armed Forces India. Imaging findings in fetal diaphragmatic abnormalities. Pediatr Radiol. Helical CT of diaphragmatic rupture caused by blunt trauma. Congenital Diaphragmatic Hernia and Diaphragmatic Eventration. Clin Perinatol.
Eur J Pediatr Surg. Long-term follow-up of congenital diaphragmatic hernia. This book is distributed under the terms of the Creative Commons Attribution 4.
Hernia de Bochdalek
Introduction Bochdalek hernia is a type of congenital diaphragmatic hernia that primarily manifests in children. It is rare in adults and accounts for about 0. Bochdalek hernia affects approximately 1 in to 12, live births and was first described by Vincent Alexander Bochdalek in [ 3 ]. Presentation of a Bochdalek hernia in an adult is exceptionally rare, Kirkland in published the first review of 34 cases of adult Bochdalek hernia and as of only cases of symptomatic adult Bochdalek hernia have been reported in world literature [ 4 ], however with the growing use of abdominal CT this abnormality is being increasingly detected in asymptomatic individuals.
Bochdalek Hernia: Symptoms & Repair
A few of the symptoms of a Bochdalek Hernia include difficulty breathing, fast respiration and increased heart rate. Also, if the baby appears to have cyanosis blue-tinted skin this can also be a sign. If the baby has a Bochdalek Hernia it may appear that one side of the chest cavity is larger than the other and or the abdomen seems to be scaphoid caved in. During fetal development, the diaphragm is formed between the seventh and tenth week. Also, during this time, the esophagus, stomach, and intestines are formed. Therefore, a Bochdalek hernia forms either from malformation of the diaphragm, or the intestines become locked into the chest cavity during the construction of the diaphragm.
Adult Bochdalek Hernia
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