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Congenital Diaphragmatic Hernia Documented for 300 Years?

Congenital Diaphragmatic Hernia has been recorded for over 300 years, but no one has ever heard of it, unless they encounter it.

Congenital diaphragmatic hernia (CDH) was first described in 1679 by Lazarus Riverius who incidentally noted a CDH on a postmortem examination in a 24 year old.

In 1701, Sir Charles Holt described the classical clinical and postmortem findings of an infant with CDH in philosophical transactions of the Royal Society of London.

And:

In 1848, Victor Alexander Bochdalek, a professor of anatomy in Prague described both right and left posterolateral CDH. To this day, CDH commonly is referred to as a Bochdalek hernia in honor of Victor…

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Featured

  Late-Presenting Congenital Diaphragmatic Hernia

Not all that wheezes is asthma! Add Late Presenting Congenital Diaphragmatic Hernia to the DDX of children with respiratory distress.

 Behind Each Breath, an Underappreciated Muscle

One in every 2,500 babies is born with a hole in its diaphragm. The baby’s liver, intestines and other abdominal organs can push up through this opening against the lungs, stunting their growth and restricting the baby’s breathing. About a third of babies born with congenital diaphragmatic hernias die, and it is likely that still more die of this defect before birth.

Articles of Interest

A Classical case of Congenital Diaphragmatic Hernia

A Single/ Term Male Baby weighing 3.0 kg was delivered via emergency LSCS for fetal distress who was prenatally diagnosed to have congenital diaphragmatic hernia in utero. He was born with Apgar score 4/10, 6/10, was immediately intubated and transferred to NICU. After stabilization, he was kept under the ventilator and planned for surgery by the Pediatric surgery team.

A prompt diagnosis of late-onset congenital diaphragmatic hernia

CDH is diagnosed either during pregnancy or shortly after birth, and it causes life-threatening severe respiratory distress. The clinical diagnosis of late-presenting CDH (5%–25% of cases) is difficult due to its insidious onset.

Congenital Diaphragmatic Hernia

Congenital Diaphragmatic Hernia (CDH) is defined by the presence of an orifice in the diaphragm, more often left and posterolateral that permits the herniation of abdominal contents into the thorax.

Congenital Diaphragmatic Hernia in a Child With Abdominal Pain and Respiratory Distress

We report a case of late presentation of congenital diaphragmatic hernia in a child who presented to the emergency department with abdominal pain and respiratory distress. The usual and unusual presentations of congenital diaphragmatic hernia in older children and their possible complications are discussed.

Congenital Diaphragmatic hernia – a review

The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction.

Congenital Diaphragmatic Hernia: The Surgeon’s Perspective

The role of in utero surgery for CDH remains controversial. However, the recent identification of new and reliable ultrasonographic predictors of postnatal mortality and the evolution of a minimally invasive surgical technique justifies in utero surgery for CDH as a reasonable treatment option for select fetuses who have essentially no chance of survival despite optimal postnatal care.

Congenital diaphragmatic hernias: from genes to mechanisms to therapies

Congenital diaphragmatic hernias (CDHs) and structural anomalies of the diaphragm are a common class of congenital birth defects that are associated with significant morbidity and mortality due to associated pulmonary hypoplasia, pulmonary hypertension and heart failure.

Emergency treatment of late-presenting congenital diaphragmatic hernia...

Congenital diaphragmatic hernia (CDH) is a scarce birth defect. It is called late-presenting CDH when symptoms are found after 1 month of life. The clinical manifestations of late-presenting CDH are diverse...

Face of CDH - Noah's Miracle

CDH is an unpublicized nearly unrecognized devastating birth defect that affects many families every day. Most people do not know anything about CDH,

Genetics of diaphragmatic hernia

The incidence is 1:2000 corresponding to 8% of all major congenital malformations. Morbidity and mortality in affected newborns are very high and at present, there is no precise prenatal or early postnatal prognostication parameter to predict clinical outcome in CDH patients. Most cases occur sporadically, however, genetic causes have long been discussed to explain a proportion of cases.

Surgery in the womb: miracle maker for NHS's tiniest patients

Kypros Nicolaides has spent 40 years caring for babies who have yet to be born. We watched him at work

The History of Congenital Diaphragmatic Hernia from 1850s to the Present

Congenital diaphragmatic hernia (CDH) is believed to result from incomplete fusion of the pleuroperitoneal membrane, and passage of the abdominal contents into the chest. A historical review of the literature on this subject shows a wide divergence of opinion on the etiology of the various types of CDH, and on the recommended treatment.

What to Expect After Bringing a CDH Baby Home - the Basics

The most common issue these babies may have is gastresophageal reflux/foregut dysmotility – it is reported to occur in 45% to 90% of infants with CDH. Reflux can be treated with medications, and many parents also use some sort of angle bed – raised head after and during the feeds. As these babies grow, they can outgrow reflux.

Why I dislike the phrase: "I hate CDH."

I get why parents and family members "hate CDH", our own daughter's death certificate states under "cause of death": "complications of congenital diaphragmatic hernia". I also hated it for a long time. It is a birth defect that sounds so very simple, yet is so complicated.

Resources

CDH International

CDH International was founded to help find the cause, prevention and best treatments of Congenital Diaphragmatic Hernia while raising awareness and supporting patient families.

Alliance of Congenital Diaphragmatic Hernia Organizations

International group of professional charity and research organizations working together to better serve babies and families affected by Congenital Diaphragmatic Hernia.

Breath of Hope

Breath of Hope exists to combat the congenital diaphragmatic hernia birth defect. We are committed to supporting parents, children, family members and friends facing the diagnosis of a congenital diaphragmatic hernia birth defect. We carry out this mission through promoting public awareness, supporting the medical community and encouraging research efforts to one day successfully treat all those diagnosed with a congenital diaphragmatic hernia.

Cherubs

CHERUBS was created to help families of babies born with Congenital Diaphragmatic Hernia by providing support services, promoting research and raising awareness in 1995. In 2017, the organization evolved into CDH International and CHERUBS became the family support division of a new, global entity.

Raising Diaphragmatic Hernia Children

This site offers parents ways to be aware of problems their child may face. There is no two cdh children alike. However many have the same issues.

The Fetal Medicine Foundation

Left (80%), right (15%) and posterolateral or anterior retrosternal (5%). Polyhydramnios at >26 weeks in most cases.

CDC

During pregnancy, there are screening tests to check for some birth defects and other conditions. Doctors can usually diagnose diaphragmatic hernia before the baby is born.

GeneReviews

Congenital diaphragmatic hernias are increasingly diagnosed prenatally; however, they are occasionally diagnosed in symptomatic neonates and even asymptomatic or mildly symptomatic children, teens, and adults.

Nationwide Children's

Babies born with CDH can have small, underdeveloped lungs (pulmonary hypoplasia), as well as reduced blood flow to the lungs caused by high blood pressure in the lungs (pulmonary hypertension). These 2 problems, pulmonary hypoplasia and pulmonary hypertension, cause babies with CDH to have a lot of trouble breathing right after birth.

Physiopedia

Congenital Diaphragmatic Hernia (CDH) two main types, uncommon yet distinct forms, 80% occur on the left side of the diaphragm:

StatPearls

Congenital diaphragmatic hernia (CDH) refers to a developmental defect of the diaphragm that will result in the herniation of abdominal viscera into the chest cavity. Neonates with CDH usually present early in the first few hours of life with respiratory distress. The respiratory distress accompanied by the CDH may be mild.

Teach Me Surgery

In neonates, the mainstay of investigation is with the plain film erect chest radiograph (CXR), which will show bowel loops (or any other abdominal viscera) herniated into the thoracic cavity.