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Encyclopedia > Congenital diaphragmatic hernia
Congenital diaphragmatic hernia
Classification and external resources
ICD-10 Q79.0
ICD-9 756.6
OMIM 142340 222400
DiseasesDB 32489
MedlinePlus 001135
eMedicine ped/2603 

Congenital diaphragmatic hernia (CDH) is a term applied to a variety of congenital birth defects that involve abnormal development of the diaphragm. The term is used most commonly in reference to Bochdalek hernia, however it can also apply to Morgagni's hernia, diaphragm eventration and central tendon defects of the diaphragm. A common theme in all scenarios is malformation of the diaphragm; this allows the abdominal contents to protrude into the chest thereby impeding proper lung formation. Newborns with CDH often have severe respiratory distress which can be life-threatening unless treated appropriately. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // Q00-Q99 - Congenital malformations, deformations and chromosomal abnormalities (Q00-Q07) Congenital malformations of the nervous system (Q00) Anencephaly and similar malformations (Q01) Encephalocele (Q02) Microcephaly (Q03) Congenital hydrocephalus (Q04) Other congenital malformations of brain (Q05) Spina bifida (Q06) Other congenital malformations of spinal cord (Q07) Other congenital malformations of nervous... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... A congenital disorder is any medical condition that is present at birth. ... In the anatomy of mammals, the diaphragm is a shelf of muscle extending across the bottom of the ribcage. ... // A Bochdalek Hernia is one of two forms of a diaphragmatic hernia (the other, Morgagnis hernia. ... The abdomen in a human and an ant. ... Male Chest The chest is a part of the anatomy of humans and various other animals. ... For the village in Tibet, see Lung, Tibet. ... There are two forms of respiratory distress syndrome: ARDS, which is acute (or adult) respiratory distress syndrome or infant respiratory distress syndrome which is a complication of premature birth. ...

Contents

Bochdalek hernia

The Bochdalek hernia, also known as a postero-lateral diaphragmatic hernia, is the most common manifestation of CDH, accounting for more than 95% of cases.[1][2] In this instance the diaphragm abnormality is characterized by a hole in the postero-lateral corner of the diaphragm which allows passage of the abdominal viscera into the chest cavity. The majority of Bochdalek hernias (80-85%) occur on the left side of the diaphragm, a large proportion of the remaining cases occur on the right side, and a small fraction are bilateral i.e., left and right sided defects. [3][4]


Pathophysiology

It involves three major defects.

In the anatomy of mammals, the diaphragm is a shelf of muscle extending across the bottom of the ribcage. ... Morphogenesis (from the Greek morphê shape and genesis creation) is one of three fundamental aspects of developmental biology along with the control of cell growth and cellular differentiation. ... Look up hernia in Wiktionary, the free dictionary. ... The abdomen in a human and an ant. ... Male Chest The chest is a part of the anatomy of humans and various other animals. ... The heart and lungs (from an older edition of Grays Anatomy) The lung is an organ belonging to the respiratory system and interfacing to the circulatory system of air-breathing vertebrates. ... Hypoplasia is an incomplete or arrested development of an organ or a part [1]. It is descriptive of many medical conditions such as: Underdeveloped breasts during puberty. ...

Presentation and diagnosis

This condition can often be diagnosed before birth and fetal intervention can sometimes help, depending on the severity of the condition.[5] Infants born with diaphragmatic hernia experience respiratory failure due to both pulmonary hypertension and pulmonary hypoplasia. The first condition is a restriction of blood flow through the lungs thought to be caused by defects in the lung. Pulmonary hypoplasia or decreased lung volume is directly related to the abdominal organs presence in the chest cavity which causes the lungs to be severely undersized, especially on the side of the hernia. Fetal intervention involves in-utero medical treatment for a fetus—usually one suffering from some form of birth defect. ... In medicine, pulmonary hypertension (PH) is an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, together known as the lung vasculature, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion. ...


Survival rates for infants with this condition vary, but have generally been increasing through advances in neonatal medicine. Work has been done to correlate survival rates to ultrasound measurements of the lung volume as compared to the baby's head circumference. This figure known as the lung to head ratio (LHR).


Treatment

ECMO has been used as part of the treatment strategy at some hospitals.[6][7] In intensive care medicine, extracorporeal membrane oxygenation (ECMO) is a technique of providing oxygen to patients whose lungs are so severely diseased that they can no longer serve their function. ...


Morbidity and mortality

Congenital Diaphragmatic Hernia has a morbidity rate of 50%. This is an average rate of success, individual rates very greatly dependent upon multiple factors; size of hernia, organs involved, additional birth defects or genetic problems, amount of lung growth, age and size at birth, type of treatments, timing of treatments, complications such as infections and lack of lung function. Each patient is different, therefore each patient has different chances of survival.


Morgagni's hernia

Morgagni's Hernia
Morgagni's Hernia

This rare anterior defect of the diaphragm is variably referred to as Morgagni’s, retrosternal, or parasternal hernia. Accounting for approximately 2% of all CDH cases, it is characterised by herniation through the foramina of Morgagni which are located immediately adjacent to the xyphoid process of the sternum.[8] The majority of hernias occur on the right side of the body and are generally asymptomatic. However, newborns may present with respiratory distress at birth similar to Bochdalek hernia. Additionally, recurrent chest infections and gastrointestinal symptoms have been reported in those with previously undiagnosed Morgagni's hernia.[9][10] In asymptomatic individuals laparoscopic surgical repair is still recommended as they are at risk of a strangulated intestine. The foramina of Morgagni (sing. ... The xiphoid process is a small cartilaginous extension to the lower part of the sternum which is usually ossified in the adult human. ... The sternum (from Greek στέρνον, sternon, chest) or breastbone is a long, flat bone located in the center of the thorax (chest). ...

Diaphragm eventration

The diagnosis of congenital diaphragmatic eventration is used when there is abnormal displacement (i.e. elevation) of part or all of an otherwise intact diaphragm into the chest cavity. This rare type of CDH occurs because in the region of eventration the diaphragm is thinner, allowing the abdominal viscera to protrude upwards. This thinning is thought to occur because of incomplete muscularisation of the diaphragm, and can be found unilaterally or bilaterally. [11] Minor forms of diaphragm eventration are asymptomatic, however in severe cases infants will present with respiratory distress similar to Bochdalek hernia.[12] Diaphragm eventration is typically repaired thoracoscopically, by a technique called plication of the diaphragm. [13] Plication basically involves a folding of the eventrated diaphragm which is then sutured in order to “take up the slack” of the excess diaphragm tissue.


References

  1. ^ Torfs CP, Curry CJ, Bateson TF, Honoré LH (1992). "A population-based study of congenital diaphragmatic hernia". Teratology 46 (6): 555–65. doi:10.1002/tera.1420460605. PMID 1290156. 
  2. ^ Yang W, Carmichael SL, Harris JA, Shaw GM (2006). "Epidemiologic characteristics of congenital diaphragmatic hernia among 2.5 million California births, 1989-1997". Birth Defects Res. Part A Clin. Mol. Teratol. 76 (3): 170–4. doi:10.1002/bdra.20230. PMID 16511883. 
  3. ^ Torfs CP, Curry CJ, Bateson TF, Honoré LH (1992). "A population-based study of congenital diaphragmatic hernia". Teratology 46 (6): 555–65. doi:10.1002/tera.1420460605. PMID 1290156. 
  4. ^ Lally KP, Lally PA, Lasky RE, et al (2007). "Defect size determines survival in infants with congenital diaphragmatic hernia". Pediatrics 120 (3): e651–7. doi:10.1542/peds.2006-3040. PMID 17766505. 
  5. ^ "Deadly hernia corrected in womb - Surgeons have developed an operation to repair a potentially fatal abnormality in babies before they are born.", BBC news, 2004-07-26. Retrieved on 2006-07-14.  - report of new operation, pioneered at London's King's College Hospital which reduced death rates in the most at risk by 50%
  6. ^ Tiruvoipati R, Vinogradova Y, Faulkner G, Sosnowski AW, Firmin RK, Peek GJ (2007). "Predictors of outcome in patients with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation". J. Pediatr. Surg. 42 (8): 1345–50. doi:10.1016/j.jpedsurg.2007.03.031. PMID 17706494. 
  7. ^ Logan JW, Rice HE, Goldberg RN, Cotten CM (2007). "Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies". Journal of perinatology : official journal of the California Perinatal Association 27 (9): 535–49. doi:10.1038/sj.jp.7211794. PMID 17637787. 
  8. ^ Torfs CP, Curry CJ, Bateson TF, Honoré LH (1992). "A population-based study of congenital diaphragmatic hernia". Teratology 46 (6): 555–65. doi:10.1002/tera.1420460605. PMID 1290156. 
  9. ^ Thomas GG, Clitherow NR (1977). "Herniation through the foramen of Morgagni in children". The British journal of surgery 64 (3): 215–7. PMID 890271. 
  10. ^ Eren S, Ciriş F (2005). "Diaphragmatic hernia: diagnostic approaches with review of the literature". European journal of radiology 54 (3): 448–59. doi:10.1016/j.ejrad.2004.09.008. PMID 15899350. 
  11. ^ Thomas TV (1970). "Congenital eventration of the diaphragm". Ann. Thorac. Surg. 10 (2): 180–92. PMID 4913762. 
  12. ^ Rais-Bahrami K, Gilbert JC, Hartman GE, Chandra RS, Short BL (1996). "Right diaphragmatic eventration simulating a congenital diaphragmatic hernia". American journal of perinatology 13 (4): 241–3. PMID 8724727. 
  13. ^ Becmeur F, Talon I, Schaarschmidt K, et al (2005). "Thoracoscopic diaphragmatic eventration repair in children: about 10 cases". J. Pediatr. Surg. 40 (11): 1712–5. doi:10.1016/j.jpedsurg.2005.07.008. PMID 16291157. 

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Arthrogryposis (IPA pronunciation: /ËŒar. ... Carpenters syndrome is an extremely rare craniofacial disorder. ... Craniodiaphyseal dysplasia (also known as CDD or lionitis) is an extremely rare autosomal recessive bone disorder that causes calcium to build up in the skull, disfiguring the facial features and reducing life expectancy. ... Craniosynostosis is a medical medical condition in which some or all of the sutures in the skull of an infant close too early, causing problems with normal brain and skull growth. ... Scaphocephaly is a type of cephalic disorder. ... Macrocephaly (from the greek words μακρύς, meaning long, and κεφάλη, meaning head), a type of cephalic disorder, is a condition in which the head circumference is larger than average for the age and sex of the infant or child. ... Oxycephaly is a type of cephalic disorder. ... Platybasia is a spinal disease of a malformed relationship between the occipital bone and cervical spine. ... Saethre-Chotzen syndrome, also known as acrocephalosyndactyly type 3 (ACS III) and Chotzen syndrome is a very rare congenital syndrome characterised by craniosynostosis (premature closure of one or more of the sutures between the bones of the skull). ... Treacher Collins syndrome (also known as Franceschetti-Zwahlen-Klein syndrome or mandibulofacial dysostosis) is a rare genetic disorder characterized by craniofacial deformities. ... Trigonocephaly is a type of cephalic disorder. ... Crouzon Syndrome is a type of genetic disorder known as a branchial arch syndrome. ... Crouzon Syndrome is a genetic disorder known as a branchial arch syndrome. ... Hypertelorism is an abnormally increased distance between two organs or bodily parts, usually referring to an increased distance between the eyes (orbital hypertelorism), seen in a variety of syndromes, including DiGeorge syndrome and Loeys-Dietz syndrome. ... The Hallermann-Streiff syndrome (also known as the François Dyscephalic Syndrome, Hallermann-Streiff-François syndrome, Oculomandibulodyscephaly with hypotrichosis and the Oculomandibulofacial Syndrome) is a disorder that effects the bodys stature, head structure and hair growth. ... Klippel-Feil syndrome is a rare disorder characterized by the congenital fusion of any 2 of the 7 cervical (neck) vertebrae. ... Not to be confused with spondylosis or spondylolysis. ... A cervical rib is a supernumerary (extra) rib which arises from the seventh cervical vertebra. ... Bifid rib, bifurcated rib Bifid rib or bifurcated rib. ... Osteochondrodysplasia is a general term for a disorder of the development (dysplasia) of bone (osteo) and cartilage (chondro). Examples include: Achondroplasia Cleidocranial dysostosis Fibrous dysplasia Langer-Giedion syndrome Mafucci syndrome Osteosclerosis Category: ... Achondrogenesis is a lethal bone disease in utero or a few days after the birth. ... Hypochondrogenesis is a severe, inherited disorder of bone growth. ... Thanatophoric dysplasia is a severe inherited skeletal disorder characterized by extremely short limbs and folds of extra skin on the arms and legs. ... Short rib-polydactyly syndrome is a family of four closely related dysplasias: I - Saldino-Noonan type II - Majewski type III - Verma-Naumoff type IV - Beemer-Langer type Category: ... Chondrodysplasia punctata is a punctiform osteochondrodysplasia. ... Rhizomelic chondrodysplasia punctata is a form of chondrodysplasia punctata which present with rhizomelia. ... Conradi-Hünermann syndrome is a type of chondrodysplasia punctata. ... Achondroplasia is a type of autosomal dominant genetic disorder that is a common cause of dwarfism. ... Hypochondroplasia is a developmental disorder caused by an autosomal dominant genetic defect in the fibroblast growth factor receptor 3 gene (FGFR3) that results in a disproportionately short stature, micromelia, and a head that appears large when compared with the underdeveloped portions of the body. ... Ellis-van Creveld Syndrome (also called chondroectodermal dysplasia or mesoectodermal dysplasia) is a rare genetic disorder of the skeletal dysplasia type, with numerous anomalies including post-axial polydactyly, congenital heart defects (most commonly an atrial septal defect producing a common atrium, occurring in 60% of affected individuals), pre-natal tooth... Otospondylomegaepiphyseal dysplasia (OSMED) is an inherited disorder of bone growth that results in skeletal abnormalities, severe hearing loss, and distinctive facial features. ... Spondyloepiphyseal dysplasia congenita is a rare disorder of bone growth that results in dwarfism, characteristic skeletal abnormalities, and problems with vision and hearing. ... Osteogenesis imperfecta (OI and sometimes known as Brittle Bone Disease) is a genetic bone disorder. ... McCune-Albright syndrome (polyostotic fibrous dysplasia), described in 1937 by Donovan James McCune and Fuller Albright, is a genetic disorder of bones, skin pigmentation and hormonal problems along with premature puberty. ... Osteopetrosis is an extremely rare inherited disorder whereby the bones harden, becoming denser. ... Metaphyseal dysplasia (also called Pyle disease)is a rare disease in which the outer part of the shafts of long bones is thinner than normal and there is an increased chance of fractures. ... Recessive multiple epiphyseal dysplasia is a disorder of cartilage and bone development. ... Hereditary Multiple Exostoses (HME) is a medical condition whereby multiple exostoses (bony spurs or lumps, also known as osteochondromas) develop on the bones of a child. ... Chondrodystrophy (literally, cartilage bad-nourishment) is a descriptive term no longer in use in the medical literature. ... Atelosteogenesis, type II is a severe disorder of cartilage and bone development. ... Diastrophic dysplasia is a disorder of cartilage and bone development. ... An infant born with an abdominal wall defect has an abnormal opening on the abdomen. ... An omphalocele is a type of abdominal wall defect in which the intestines, liver, and occasionally other organs remain outside of the abdomen in a sac because of a defect in the development of the muscles of the abdominal wall. ... Gastroschisis is a type of abdominal wall defect in which the intestines and sometimes other organs develop outside the fetal abdomen through an opening in the abdominal wall. ... Prune belly syndrome is a rare birth defect affecting about 1 in 40,000 births. ... Ehlers-Danlos syndrome is a group of rare genetic disorders affecting humans and domestic animals caused by a defect in collagen synthesis. ...

  Results from FactBites:
 
eMedicine - Congenital Diaphragmatic Hernia : Article by Robin H Steinhorn (5415 words)
CDH is characterized by a variable degree of pulmonary hypoplasia associated with a decrease in cross-sectional area of the pulmonary vasculature and dysfunction of the surfactant system.
CDH occurs in 1 of every 2000-3000 live births and accounts for 8% of all major congenital anomalies.
While CDH is usually a disorder of the newborn period, as many as 10% of patients may present after the newborn period and even during adulthood.
  More results at FactBites »


 
 

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