Congerital diaphragmatic Kermia | Radiology Reference Amicic| Radiopacdia.org, tatp-//radiopacdia.org/artictes/congemtal_diaphragiatxc_hernia i & Feedback Diao w + Lown * Sup About these ads Search Encyclopedia (browse by topic or sytem) Add Article Patient Cases browse by topic or system) Add Case Quiz Mode (diagnose from case presentations) This site is targeted at medical and radiology professionals, contains user contributed content, and material thet may be confusing toa this site implies acceptance of our Terms of Use audience, Use of Congenital diaphragmatic hernia De Yuranga Weerakkody and Rishi Agrawal eta. ‘Congenital diaphragmatic herniation (CDH) accounts fora smal proportion ofall diaphragmatic here. Its however ene ofthe commonest non eardiae Fetal inteathoraic anomalies, Epidemiology ‘Congenital diaphragmatic hernia are seen in | of every 2000-4000 ive births ‘Glnical presentation “Most congenital diaphragmatic herias are detected cither soon aftr birth or on antenatal ultrasound. Mortality is predominantly du to development of pulmonary hypoplasia, which s thought to be due to mass effect on the developing lung. Sch neonates are hypoxe and have persistent fetal circulation dueCongerital diaphragmatic Kermia | Radiology Reference Amicic| Radiopacdia.org, tatp-//radiopacdia.org/artictes/congemtal_diaphragiatxc_hernia ‘to pulmonary hypoplasia and pulmonary hypertension Pathology Diaphragmatic development is usually complete by ~ the 9" weok of gestation, Congenital diaphragmatic hernias result rom failure of fusion of one of the pleuropertoneal canals at about weeks gestation. They may contain stomach, intestines, liver or spleen Casiation ‘Congenital diaphragmatic herniation can be elasitied ita wo basic types on location 1, Bochdalek hernia © commoner on the left: 75-008 © posterior © lange and associated with poorer outome © presents earlier © mnemonic: BBBBB 2, Mong hes © less common © anterior © presents later ‘While a CDH can oceur as an isolated contin, assovated anomalies are relatively common and include + pulmonary hypoplasia: also a complication * brochopulinonary-sequestaion + ancuplody ean be present in up to 50% rio 13 © trisomy 18 ® trisomy 24 2 Turner syndrome - monosomy X o Paliter-Kilan syndrome -terasomy 12p + Frynssyndome + Comelia de Lange syndrome i PaneCongerital diaphragmatic Kermia | Radiology Reference Amicic| Radiopacdia.org, tatp-//radiopacdia.org/artictes/congemtal_diaphragiatxc_hernia * congenital cardiac anomalies + neural tube defects © anencephals © spina bifida Radiographic features Utewoond Sonographic Findings include «+ cardiomediasinal shift +) abnormal cardiac axis + the stomach being at same transverse level a the heart: this makes left sided hernias comparatively easier to detect on ultrasound (as opposed to hemniaion of echogenic liver on the right sie) «+ portal veinsin thorax (Doppler) + absent bowel lops inthe abdomen * there may be polyhscanios a an ancillary sonographic feature Ps Ceca + indistinct diaphragm with op + seaphoid abdomen + deviation of tines) © endotracheal tube © nasogastic tube © umbilical atrial and venous catheters ification of part of oral the hemithorax (typically left sided) MR Fetal MRI may be helpful in further assessing the hernia and any associated pulmonary hypoplasia. Lung-head rato °: can be assessed on both ultrasound or MRI: 2 axial diameters of lung mukiplied, and divided by head circumference) may be used to _sve an indication of pulmonary hypoplasia, LHR ~ 1-0 is associated with a poor prognosis, nd > 14 a good prognosis, i PaneCongerital diaphragmatic Kermia | Radiology Reference Amicic| Radiopacdia.org, tatp-//radiopacdia.org/artictes/congemtal_diaphragiatxc_hernia pe ‘Complications + development of pulmonary hypoplasia Treatment and prognosis Fetuses with an antenatal diagnosis of CDH should be delivered ina tertiary referral centre with access to neonatal intensive care and paediatric susical ‘cles. Large CDH have a poor prognosis, due to pulmonary hypoplasia and perinatal mortality may be as high as 86, Clearly successful management is dependent ‘on specialist paediatric Facilites, withthe ability to offer surgery, ECMO ete, Sigs suggesting a poor prognosis include large ernial size * early gestational age at diagnosis + intrathoracic iver * smal contralateral ling «+ presence of associated abnor * bilateral CDH, * unfavourable lung head ratio. Some centres perform in utero surgery in selected cases including novel methods such as clamping the fetal tachea to allow expansion ofthe fetal lungs with ‘uid and consequently push the hemiation back into the abdomes Ditfereni ingnosis ‘General imaging differential considerations inchude + congenital cystic adenomataid malformation * hybrid lesion * pulmonary sequestcation (can also be an association) References Synonyms & Alternative SpellingsCongeital diapbragmatx: Rermia | Radiology Reference Amicic| Radiopacdia.org: it Article Share + New Revision History URL of Anite hp:tadapaedie.opiat Article Information: Systems: Musculoskeletal, Paclaties A tatp-//radiopacdia.org/artictes/congemtal_diaphragiatxc_herniaCongcmital diapbragmatx: Kermia | Radiology Reference Amicic| Radiopacdia.org, (Case = right sided Drag hereto reorder ‘Case 2- antenatal ultrasound let sided Drag hereto reorder. + ‘tatp-//radiopacdia org/artictes/congemtal_diaphragiatxc_erniaCongcrital diapbragimatx: Rermia | Radiology Reference Amicic| Radiopacdia.org, Case 3 -on antenatal MRI Draghere to reorder Case 4 Left sided Drag hereto reorder fnip.//radropacdia.orp/aruckes/congenstal_diaphragwatic_hernisCongcmital diapbragmatx: Kermia | Radiology Reference Amicic| Radiopacdia.org, Cases Drag here to reorder Cases Drag here to reorder. i ‘tatp-//radiopacdia org/artictes/congemtal_diaphragiatxc_erniaCongcmital diapbragimatxc Bermia | Radiology Reference Amicic| Radiopacdia.org, Case7 Drag hereto reorder, + G Case 8 Drag hereto reorder ‘tatp//radiopacdia org/artictes/congemtal_diaphragiatxc_herniaCongcmital diapbragimatxc Bermia | Radiology Reference Amicic| Radiopacdia.org, Case 9 Drag hereto reorder CCase 10 on ultrasound Drag hereto reorder. fnip.//radropacdia.orp/aruckes/congenstal_diaphragwatic_hernisCongcmital diapbragimatxc Bermia | Radiology Reference Amicic| Radiopacdia.org, Case 10 -n CT Drag hereto reorder Case 11 -note position of nasogastric tube Drag hereto reorder, ‘tatp//radiopacdia org/artictes/congemtal_diaphragiatxc_herniaCongcrital diapbragimatxc Kermia | Radiology Reference Amicic| Radiopacdia.org: Case 12 Drag hereto reorder, Case 13, Drag hereto reorder oe ‘tatp-//radiopacdia org/artictes/congemtal_diaphragiatxc_herniaCongcmital diapbragmatx: Kermia | Radiology Reference Amicic| Radiopacdia.org, Case 14 Drag here to reorder. Case 15 Drag hereto reorder oe ‘tatp-//radiopacdia org/artictes/congemtal_diaphragiatxc_erniaCongcmital diapbragimatxc Bermia | Radiology Reference Amicic| Radiopacdia.org, Case 16 Drag hereto reorder (Case 17 -n antenatal ultrasound Drag hereto reorder, Imaging Differential Diagnosis peCongcmital diapbragimatxc Bermia | Radiology Reference Amicic| Radiopacdia.org, ‘Congenital cystic adenomataid malformation Drag hereto reorder. eI ‘Congenital cystic adenomataid malformation Drag hereto reorder Encyclopaedia a ‘tatp//radiopacdia org/artictes/congemtal_diaphragiatxc_herniaCongerital diaphragmatic Kermia | Radiology Reference Amicic| Radiopacdia.org, tatp-//radiopacdia.org/artictes/congemtal_diaphragiatxc_hernia By Section: Anatomy * Gams erventonal radiology * Mnemonics * Patholoay = Physis, + Sans + Sasne * Svniomes By System: * Breast = Cardiac += Central Nenous System Chest * Gastrointestinal * Gynaecology *# Haematology Head & Neck + Hepatobiliary ! 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