Bochdalek hernia

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Bochdalek hernia
Diaphragma.png
"Bochdalek", at lumbocostal triangle, labeled in bottom left (Captions in German.)
Specialty Thoracic surgery
Differential diagnosis Diaphragmatic rupture [1]
FrequencyRare [2]

Bochdalek hernia is one of two forms of a congenital diaphragmatic hernia, the other form being Morgagni hernia. A Bochdalek hernia is a congenital abnormality in which an opening exists in the infant's diaphragm, allowing normally intra-abdominal organs (particularly the stomach and intestines) to enter into the thoracic cavity. In the majority of people, the affected lung will be deformed, [3] and the resulting lung compression can be life-threatening. Bochdalek hernias occur more commonly on the posterior left side (85%, versus right side 15%).

Contents

Bochdalek hernias are rare. [2] This type of hernia was first described in 1754 by McCauley and subsequently studied and named after the Czech pathologist Vincenz Alexander Bochdalek (1801–1883). [2]

Signs and symptoms

Children

In normal Bochdalek hernia cases, the symptoms are often observable simultaneously with the baby's birth. 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. [4] Another way to identify a baby with Bochdalek hernia, is to look at the chest immediately after birth. 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 caved in. [5]

Adults

Symptoms rarely develop in adults. [1] If symptoms do occur, they may be vague and related to the gastrointestinal tract. [1] This may include pain or signs of a bowel obstruction. [2]

Causes

Most likely, Bochdalek hernias are formed throughout the growth process and organ construction during fetal development. 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. Although these are some factors that contribute to a Bochdalek hernia, it does not take all variables into account. Bochdalek hernias, along with Morgagni hernias, are both multifactor conditions, meaning that there are many reasons and multiple variables that contribute to the malformations. For example, in each case, there could be genetic and or environmental condition(s) that can add to the probability of this birth defect. [5]

Diagnosis

One way to determine if a baby does in fact have a Bochdalek hernia, would be to have a pediatrician perform a physical on the infant. A chest X-ray can also be done to examine the abnormalities of not only the lungs but also the diaphragm and the intestine. In addition to these, a doctor can also take a blood test, drawing arterial blood to check and determine how well the baby is breathing and his or her ability to breathe. A chromosomal test (done by testing the blood) can also be performed to determine whether or not the problem was genetic. The doctors can also take an ultrasound of the heart (echocardiogram) to evaluate the health of the heart.[ citation needed ]

Treatment

Children

There are different pathways for treatment available. The first step in treatment includes the baby's admission into the NICU (Neonatal Intensive Care Unit). [5] The preferred path of treatment for most Bochdalek Hernia is that babies who are admitted in the NICU, are placed on a mechanical ventilator to help breathing. Very rarely will the hernia be small enough that it is not noticed. It is more likely to be asymptomatic if it is on the right side of the chest. If the infant cannot be stabilized using medical treatments and mechanical ventilation, the alternate pathway involves putting the infants on a temporary heart/lung bypass machine, called ECMO, which stands for extra-corporeal membrane oxygenation. [5] ECMO allows oxygen to be regulated into the blood and then pumps the blood throughout the entire body. Normally, this machine is used to stabilize the baby's condition. It is preferred to stabilize the infant prior to surgery, to minimize complications, if possible. The only treatment is surgery. [6]

After the baby is stable and his or her state has improved, the diaphragm can be fixed and the misplaced organs can be relocated to their correct position. That is either done via primary repair where the remaining diaphragm is stretched to cover the hole, or an abdominal muscle is split so it can be used to stretch over the hole, and the last option is with a Gore-Tex patch, which has higher risks of later infection or re-herniation. [5] Although these are various treatments for Bochdalek Hernias, it does not guarantee the baby will survive. [5] Since the baby must go through some or all of the previous treatments, the baby's hospital stay is usually longer than that of a "normal" newborn. The average infants born with a Bochdalek Hernia stay in the hospital between 23.1 and 26.8 days. [7] An infant needing to go on ECMO will have a longer stay and likely worse outcome than one who was stabilized without ECMO.[ citation needed ]

Adults

If the disorder is diagnosed in an adult and they remain without symptoms no specific treatment is required. [1] In those who are symptomatic surgery may be carried out by an open or closed approach. [2]

Prognosis

Bochdalek hernia can be a life-threatening condition. Approximately 85.3% of newborns born with a Bochdalek hernia are immediately high risk. [8] Infants born with a Bochdalek hernia have a "high mortality rate due to respiratory insufficiency". [9] Between 25 and 60% of infants with a Bochdalek hernia die. [7] The lungs, diaphragm, and digestive system are all forming at the same time, so when a Bochdalek hernia permits the abdominal organs to invade the chest cavity rather than remain under the diaphragm in the correct position, it puts the infant in critical condition. These "foreign bodies" in the chest cavity compress the lungs, impairing their proper development and causing pulmonary hypoplasia. [5] Since the lungs of infants suffering from a Bochdalek hernia have fewer alveoli than normal lungs, Bochdalek hernias are life-threatening conditions due to respiratory distress. [5] Like the lungs, the intestines may also have trouble developing correctly. If the intestines are trapped within the thoracic cavity, then the lungs and intestines may not be receiving the amount of blood they need to stay healthy and function properly.[ citation needed ]

More individualized prognosis for infants depends on several factors, including where the infant is born, the amount of herniation into the thoracic cavity, whether the liver is in the thorax as well, and other malformations including heart defects. [10]

For best outcomes, the fetus should be diagnosed prenatally and delivered at a tertiary hospital with ECMO capabilities. [14] Different centers manage differently and have different outcomes. Some families temporarily relocate to be at the CDH surgical center of choice.

Epidemiology

Bochdalek hernias make up about 0.17% to 6% of all diaphragmatic hernia cases when they appear spontaneously in adults and about one in every 2200 to 12,500 births every year. [4] Babies who are born with a Bochdalek hernia are more than likely to have another birth defect caused by the hernia. About twenty percent of those children born with a Bochdalek hernia, also have a congenital heart defect. In addition, infants born with this condition may also have other abnormalities. "Between five and sixteen [percent of infants] have a chromosomal abnormality." [5] In most cases, left-sided hernias or Bochdalek hernias have a ratio of 3:2 of males to females. In other words, Bochdalek hernias are more common in men.

Mnemonic

A useful way to remember the localization of this hernia vs. Morgagni is "Bochdalek is back and to the left" (re the postero-lateral localization).

Related Research Articles

Hernia Abnormal exit of tissues or organs from the cavity they usually reside in

A hernia is the abnormal exit of tissue or an organ, such as the bowel, through the wall of the cavity in which it normally resides. Various types of hernias can occur, most commonly involving the abdomen, and specifically the groin. Groin hernias are most commonly of the inguinal type but may also be femoral. Other types of hernias include hiatus, incisional, and umbilical hernias. Symptoms are present in about 66% of people with groin hernias. This may include pain or discomfort in the lower abdomen, especially with coughing, exercise, or urinating or defecating. Often, it gets worse throughout the day and improves when lying down. A bulge may appear at the site of hernia, that becomes larger when bending down. Groin hernias occur more often on the right than left side. The main concern is bowel strangulation, where the blood supply to part of the bowel is blocked. This usually produces severe pain and tenderness in the area. Hiatus, or hiatal hernias often result in heartburn but may also cause chest pain or pain while eating.

Hiatal hernia Type of hernia

A hiatal hernia or hiatus hernia is a type of hernia in which abdominal organs slip through the diaphragm into the middle compartment of the chest. This may result in gastroesophageal reflux disease (GERD) or laryngopharyngeal reflux (LPR) with symptoms such as a taste of acid in the back of the mouth or heartburn. Other symptoms may include trouble swallowing and chest pains. Complications may include iron deficiency anemia, volvulus, or bowel obstruction.

Thoracic diaphragm Sheet of internal skeletal muscle

The thoracic diaphragm, or simply the diaphragm, is a sheet of internal skeletal muscle in humans and other mammals that extends across the bottom of the thoracic cavity. The diaphragm is the most important muscle of respiration, and separates the thoracic cavity, containing the heart and lungs, from the abdominal cavity: as the diaphragm contracts, the volume of the thoracic cavity increases, creating a negative pressure there, which draws air into the lungs. Its high oxygen consumption is noted by the many mitochondria and capillaries present; more than in any other skeletal muscle.

Omphalocele Rare abdominal wall defect in which internal organs remain outside of the abdomen in a sac

Omphalocele or omphalocoele also called exomphalos, is a rare abdominal wall defect. Beginning at the 6th week of development, rapid elongation of the gut and increased liver size reduces intra abdominal space, which pushes intestinal loops out of the abdominal cavity. Around 10th week, the intestine returns to the abdominal cavity and the process is completed by the 12th week. Persistence of intestine or the presence of other abdominal viscera in the umbilical cord results in an omphalocele.

Polyhydramnios Excess of amniotic fluid in the amniotic sac

Polyhydramnios is a medical condition describing an excess of amniotic fluid in the amniotic sac. It is seen in about 1% of pregnancies. It is typically diagnosed when the amniotic fluid index (AFI) is greater than 24 cm. There are two clinical varieties of polyhydramnios: chronic polyhydramnios where excess amniotic fluid accumulates gradually, and acute polyhydramnios where excess amniotic fluid collects rapidly.

Pulmonary sequestration Medical condition

A pulmonary sequestration is a medical condition wherein a piece of tissue that ultimately develops into lung tissue is not attached to the pulmonary arterial blood supply, as is the case in normally developing lung. This sequestered tissue is therefore not connected to the normal bronchial airway architecture, and fails to function in, and contribute to, respiration of the organism.

Congenital diaphragmatic hernia Medical condition

Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm. The most common type of CDH is a Bochdalek hernia; other types include Morgagni hernia, diaphragm eventration and central tendon defects of the diaphragm. Malformation of the diaphragm allows the abdominal organs to push into the chest cavity, hindering proper lung formation.

Umbilical hernia Medical condition

An umbilical hernia is a health condition where the abdominal wall behind the navel is damaged. It may cause the navel to bulge outwards—the bulge consisting of abdominal fat from the greater omentum or occasionally parts of the small intestine. The bulge can often be pressed back through the hole in the abdominal wall, and may "pop out" when coughing or otherwise acting to increase intra-abdominal pressure. Treatment is surgical, and surgery may be performed for cosmetic as well as health-related reasons.

Vincent Bochdalek Czech anatomist

Vincent Alexander Bochdalek was a Bohemian anatomist and pathologist. His first name has also been given as Vincenc and Vincenz. Bochdalek was elected as member of the German Academy of Sciences Leopoldina.

Diaphragmatic breathing Form of breathing done by contracting the diaphragm

Diaphragmatic breathing, abdominal breathing or deep breathing, is breathing that is done by contracting the diaphragm, a muscle located horizontally between the thoracic cavity and abdominal cavity. Air enters the lungs as the diaphragm strongly contracts, but unlike during traditional relaxed breathing (eupnea) the intercostal muscles of the chest do minimal work in this process. The belly also expands during this type of breathing to make room for the contraction of the diaphragm.

Diaphragmatic hernia Medical condition

Diaphragmatic hernia is a defect or hole in the diaphragm that allows the abdominal contents to move into the chest cavity. Treatment is usually surgical.

EXIT procedure

The EXIT procedure, or ex utero intrapartum treatment procedure, is a specialized surgical delivery procedure used to deliver babies who have airway compression. Causes of airway compression in newborn babies result from a number of rare congenital disorders, including bronchopulmonary sequestration, congenital cystic adenomatoid malformation, mouth or neck tumor such as teratoma, and lung or pleural tumor such as pleuropulmonary blastoma. Airway compression discovered at birth is a medical emergency. In many cases, however, the airway compression is discovered during prenatal ultrasound exams, permitting time to plan a safe delivery using the EXIT procedure or other means.

Catamenial pneumothorax Medical condition

Catamenial pneumothorax is a spontaneous pneumothorax that recurs during menstruation, within 72 hours before or after the onset of a cycle. It usually involves the right side of the chest and right lung, and is associated with thoracic endometriosis. A third to a half of patients have pelvic endometriosis as well. Despite this association, CP is still considered to be misunderstood as is endometriosis considered to be underdiagnosed. The lack of a clear cause means that diagnosis and treatment is difficult. The disease is believed to be largely undiagnosed or misdiagnosed, leaving the true frequency unknown in the general population.

Diaphragmatic rupture Tear in the thoracic diaphragm, usually caused by physical trauma

Diaphragmatic rupture is a tear of the diaphragm, the muscle across the bottom of the ribcage that plays a crucial role in breathing. Most commonly, acquired diaphragmatic tears result from physical trauma. Diaphragmatic rupture can result from blunt or penetrating trauma and occurs in about 0.5% of all people with trauma.

Pulmonary hypoplasia Congenital disorder of respiratory system

Pulmonary hypoplasia is incomplete development of the lungs, resulting in an abnormally low number or size of bronchopulmonary segments or alveoli. A congenital malformation, it most often occurs secondary to other fetal abnormalities that interfere with normal development of the lungs. Primary (idiopathic) pulmonary hypoplasia is rare and usually not associated with other maternal or fetal abnormalities.

Alveolar capillary dysplasia (ACD) is a rare, congenital diffuse lung disease characterized by abnormal blood vessels in the lungs that cause highly elevated pulmonary blood pressure and an inability to effectively oxygenate and remove carbon dioxide from the blood. ACD typically presents in newborn babies within hours of birth as rapid and labored breathing, blue-colored lips or skin, quickly leading to respiratory failure and death. Atypical forms of ACD have been reported with initially milder symptoms and survival of many months before the onset of respiratory failure or lung transplantation.

Fryns syndrome Medical condition

Fryns syndrome is an autosomal recessive multiple congenital anomaly syndrome that is usually lethal in the neonatal period. Fryns (1987) reviewed the syndrome.

Donnai–Barrow syndrome Medical condition

Donnai–Barrow syndrome is a genetic disorder first described by Dian Donnai and Margaret Barrow in 1993. It is associated with LRP2. It is an inherited (genetic) disorder that affects many parts of the body.

Abdominal wall defects are a type of congenital defect that allows the stomach, the intestines, or other organs to protrude through an unusual opening that forms on the abdomen.

Pulmonary agenesis Medical condition

Pulmonary agenesis is an inborn lung underdevelopment that is rare and potentially lethal. The disorder is caused by a complete developmental arrest of the primitive lung during embryonic life, and it is often associated with other developmental defects. Bilateral and unilateral pulmonary agenesis are classified, depending on whether one side of the lung or both sides are affected. Bilateral pulmonary agenesis is lethal, while the mortality rate of unilateral pulmonary agenesis is higher than 50%. Depending on the severity, the symptom ranges from none to various respiratory complaints. It is detectable prenatally, however, its nonspecific clinical features act as the obstacle for diagnosing. The exact cause of pulmonary agenesis is still obscure. However, theories have been raised regarding the vascular, iatrogenic, viral and genetic causes of pulmonary agenesis in an attempt to explain the pathogenesis of the disorder. In most cases of pulmonary agenesis, surgical resection is performed to remove the malformed lobe or the entire defected lung of the patient depending on the severity of the respiratory impairment.

References

  1. 1 2 3 4 Gue, RS; Winkler, L (January 2020). "Bochdalek Hernia". PMID   30969589.{{cite journal}}: Cite journal requires |journal= (help)
  2. 1 2 3 4 5 Brown, SR; Horton, JD; Trivette, E; Hofmann, LJ; Johnson, JM (February 2011). "Bochdalek hernia in the adult: demographics, presentation, and surgical management". Hernia: The Journal of Hernias and Abdominal Wall Surgery. 15 (1): 23–30. doi:10.1007/s10029-010-0699-3. PMID   20614149. S2CID   23777788.
  3. ↑ Diaphragmatic Hernia. 2007. Children's Hospital of Wisconsin, Wisconsin. 3 Feb. 2007 <http://www.chw.org/display/PPF/DocID/22791/router.asp>
  4. 1 2 Jeffrey, Mark E., and Wilbur A. Gorodetsky. "Adult Bochdalek Hernia." Medind. 10, Sept. 2004, accessed 3 Feb. 2007 <http://medind.nic.in/maa/t05/i3/maat05i3p284.pdf> Archived October 11, 2010, at the Wayback Machine .
  5. 1 2 3 4 5 6 7 8 9 "Diaphragmatic Hernia." HealthSystems. 24 Nov. 2006. UVA Health. 3 Feb. 2007. <http://www.healthsystem.virginia.edu/UVAHealth/peds_digest/diaphrag.cfm>.
  6. ↑ Larrazábal, Natasha. Diaphragmatic Hernia, Left. 2003. Caracas-Venezuela. Diaphragmatic hernia. 6 Feb. 2007 <http://www.thefetus.net/page.php?id=1218> Archived July 27, 2009, at the Wayback Machine .
  7. 1 2 Hekmatnia, Ali, and Kieran McHugh. "Congenital Diaphragmatic Hernia." EMedicine (2003). 8 Feb. 2007 <http://www.emedicine.com/RADIO/topic187.htm>.
  8. ↑ Klein, Jaquier M. "Hospital Stays, Hospital Charges, and in-Hospital Deaths Among Infants with Selected Birth Defects --- United States, 2003." CDC. 19 Jan. 2007. 3 Feb. 2007 <https://www.cdc.gov/mmwr/preview/mmwrhtml/mm5602a1.htm>.
  9. ↑ "Hernia." Encarta. 2006. Microsoft Corporation. 8 Feb. 2007 < "Hernia - MSN Encarta". Archived from the original on October 29, 2009. Retrieved October 31, 2009.>. 2009-10-31.
  10. ↑ Chou HC, Tang JR, Lai HS, Tsao PN, Yau KI. Prognostic indicators of survival in infants with congenital diaphragmatic hernia. J Formos Med Assoc. 2001;100(3):173-175.
  11. 1 2 http://www.perinatology.com/calculators/LHRnrew.htm
  12. 1 2 3 "Archived copy" (PDF). Archived from the original (PDF) on 2020-07-26. Retrieved 2020-07-26.{{cite web}}: CS1 maint: archived copy as title (link)
  13. ↑ "Congenital Diaphragmatic Hernia (CDH)".
  14. 1 2 https://www.texaschildrens.org/sites/default/files/uploads/documents/outcomes/standards/CDH_Dec2017.pdf [ bare URL PDF ]
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