Diseases of Diaphragm Flashcards
Embroyolgic development of diaphragm
- 7-10th week of gestation
- Components:
- Septum transversum
- L/R pleuroperitoneal membranes
- Dorsal mesentary of esophagus
Congenital diaphragmatic hernia (CHD) frequency
Rare, 1 in every 2000-3000 live births
Decreased survival with CHD due to:
- Assocaited congenital abnormalities
- Prematurity
- Low birth weight
MC timing of detection of CHD
In utero (by prenatal ultrasound)
Bochdalek hernia
posterolateral CHD
- MC CHD (90%)
- Males (3:2)
- Left-sided (80-90%)
- Associated defects:
- Cardiac
- Renal
- Neural
- GI
MC CHD
Bochdalek hernia (90% of all CHD)
- Left sided
- Males (3:2)
- Associated defects:
- Cardiac
- Renal
- Neural
- GI
Major co-morbid state assocaited with CHD
Pulmonary hypoplasia and pulmonary HTN
- Due to early herniation and entrapment of abdominal orangs in chest with lung compression (directly on ipsilateral side, indirectly due to compression on contralateral side)
- Respiratory impairment
- May require ECMO
- Feeding problems
- Growth retardation
CXR finding with CHD
Intestinal loops or tip of orogastric tube in chest
Moratality rate associated with Bochdalek hernia
~ 50%
Surgical repair technique for Bochdalek hernia
- Approach from abdomen
- Reduction of hernia contents
- Resection of hernia sac (present in 20% of cases)
- Closure or reconstruction (non-absorbable mesh) of defect
- Resection of extralobar sequestration if present at time of hernia repair if patient stable
Morgagni hernia
Anterior defect
- Between xiphoid and costochondral attachments of diaphragm
- < 2% of all CHD
- Asymptomatic (incidental diagnosis)
- Surgical correction advised
- Risk of incarceration or strangulation of abdominal organs
- Outcomes excellent
- Abominal approach with primary or mesh closure
MCC of acquired diaphragmatic hernias
Trauma
- Diphragm rupture occurs in ~ 3-5% of patients admitted for trauma
- Associated with severe injuries, thus has high mortality
- Mostly due to blunt trauma
TOC for traumatic acquired diphragmatic hernia
Early surgical repair
(avoid compications of strangulation and obsruction)
- Prognosis highly influenced by severity of associated abdominal and overall injury scores
Etilogy of diaphragmatic eventration
Failure of myoblast migration to muscular portion of diaphragm, impairing ability to contract
Associated congenital defects with diaphragmatic eventration
- Spine and skelatal defects
- Hypoplastic lungs
- Pulmonary sequestration
- Transposition of viscera
Etiology of diaphragmatic paresis/paralysis
Acquired condition due to interruption of phrenic nerve conduction to diaphragm.
MCC of diaphragmatic paresis or paralysis
Phrenic nerve injury during surgery (chest or neck)
Phrenic nerve invasion by neoplasm
Less common causes of diaphragmatic paralysis
- Idiopathic:
- central line placement
- spinal cord injury
- bracheal plexus neuropathy (Parsonage-Turner syndrome)
- Neurologic disease:
- myelitis
- encephalitis
- poliomyelitis
- herpes zoster
MC symptoms of diaphragmatic eventration and paralysis
Dyspnea (most patients asymptomatic)
- Due to ventiation impairment and V/Q mismatch in basal portions of lungs
- Orthopnea more typical of paralyssi due to shifting of abdominal organs towards the chest and reduced lung volumes
Diagnostic study to evaluate diaphragmatic paralysis
Sniff test
- Paradoxial upward motion of the paralyzed diaphragm > 2 cm during sniffing under fluoroscopy
- US and MRI alternative modalities
Definitive diagnostic study to evaluate diaphragm paralysis
EMG (w/ phrenic nerve stimuation)
Surgical TOC for diaphragmatic eventration and paralysis
Diaphragm plication
- Goal: reduce paradoxical excursion of diaphragm during inspiration (increasing end-inspiratory and end-expiratory lung volumes)
- Laparotomy or Thoracotomy
- Technique: imbricating stitches in the central tendon of the diaphragm
Acceptable period of observation for suspected diaphrgam paresis
1-2 years (unless symptomatic)
Indications for diaphragmatic pacing
- Rarely used technique
- Indicaitons:
- Central alveolar hypoventilation (Ondine’s curse)
- High cervical cord injury (above C3)
- ALS (to delay need for mechanical ventilation)
Diaphragm pacing technique
Stimulation of phrenic nerve (in neck or chest) by contact with electrode
Tumors of the diaphragm
- Primary tumors very rare
- MC tumors: congenital cysts
- Bronchogenic
- Mesothelial
- Teratoid
- Benign tumors:
- fibromas
- angiomas
- chondroma
- hemangioma
- leiomyoma
- hemartoma
- desmoid
- Malignant tumors: very rare
- rhabdomyosarcoma
- leiomyosarcoma
Neurogenic diaphragm tumors may be associated with _
Hypertrophic pulmonary osteoarthropathy
TOC for diaphragm tumors
Surgical resection with diaphragm repair/reconstruction (prosthetic mesh)
Variety of clinical conditions characteried by passage of fluids, gases, tissues, secretons or intestinal content through diaphragmatic pores from abdomen to chest
Porous diaphragmatic syndrome
Spontaneous pneumothorax occuring in women in relation to menses
Catamenial pneumothorax
- Considered part of porous diaphragmatic syndromes
- diaphragmatic defects associatedin 60% of cases
- pores allow passage of air from abdoment to chest causing pneumothorax
- Visceral pleura endometrial implants and pelvic endometriosis: 20-70% of cases
Surgical treatment for catamenial pneumothorax
- Repair of diaphragmatic defect (if present)
- Resection of endometrial implant
- Pleurodesis and/or pleurectomy
Agenesis of diaphragm
- Extremely rare
- Usually diagnosed early after birth
- High mortality rate
- Familial inheritance with multifactorial genetic etiology
Cantrell’s Pentology
- Anterior diaphragmatic defect
- Supraumbilical abdominal wall defect (omphalocele)
- Diaphragmatic pericardial defect
- Congenital intracardiac defect
- VSD or LV diverticulum
- Lower sternal defect
- Defects cause omphalocele and ectopic cordis
Etiology of Cantrell’s Pentology
Failure of lateral mesoderm during 3rd week of gestation
Treatment for Cantrell’s Pentology
Correction of:
- Hernia defects (ventral and diaphragmatic)
- Sternal defects
- Intracardiac defects (VSD or LV diverticulum)
