Diseases of Diaphragm Flashcards

1
Q

Embroyolgic development of diaphragm

A
  • 7-10th week of gestation
  • Components:
    • Septum transversum
    • L/R pleuroperitoneal membranes
    • Dorsal mesentary of esophagus
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2
Q

Congenital diaphragmatic hernia (CHD) frequency

A

Rare, 1 in every 2000-3000 live births

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3
Q

Decreased survival with CHD due to:

A
  • Assocaited congenital abnormalities
  • Prematurity
  • Low birth weight
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4
Q

MC timing of detection of CHD

A

In utero (by prenatal ultrasound)

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5
Q

Bochdalek hernia

A

posterolateral CHD

  • MC CHD (90%)
  • Males (3:2)
  • Left-sided (80-90%)
  • Associated defects:
    • Cardiac
    • Renal
    • Neural
    • GI
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6
Q

MC CHD

A

Bochdalek hernia (90% of all CHD)

  • Left sided
  • Males (3:2)
  • Associated defects:
    • Cardiac
    • Renal
    • Neural
    • GI
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7
Q

Major co-morbid state assocaited with CHD

A

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
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8
Q

CXR finding with CHD

A

Intestinal loops or tip of orogastric tube in chest

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9
Q

Moratality rate associated with Bochdalek hernia

A

~ 50%

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10
Q

Surgical repair technique for Bochdalek hernia

A
  • 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
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11
Q

Morgagni hernia

A

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
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12
Q

MCC of acquired diaphragmatic hernias

A

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
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13
Q

TOC for traumatic acquired diphragmatic hernia

A

Early surgical repair

(avoid compications of strangulation and obsruction)

  • Prognosis highly influenced by severity of associated abdominal and overall injury scores
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14
Q

Etilogy of diaphragmatic eventration

A

Failure of myoblast migration to muscular portion of diaphragm, impairing ability to contract

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15
Q

Associated congenital defects with diaphragmatic eventration

A
  • Spine and skelatal defects
  • Hypoplastic lungs
  • Pulmonary sequestration
  • Transposition of viscera
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16
Q

Etiology of diaphragmatic paresis/paralysis

A

Acquired condition due to interruption of phrenic nerve conduction to diaphragm.

17
Q

MCC of diaphragmatic paresis or paralysis

A

Phrenic nerve injury during surgery (chest or neck)

Phrenic nerve invasion by neoplasm

18
Q

Less common causes of diaphragmatic paralysis

A
  • Idiopathic:
    • central line placement
    • spinal cord injury
    • bracheal plexus neuropathy (Parsonage-Turner syndrome)
  • Neurologic disease:
    • myelitis
    • encephalitis
    • poliomyelitis
    • herpes zoster
19
Q

MC symptoms of diaphragmatic eventration and paralysis

A

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
20
Q

Diagnostic study to evaluate diaphragmatic paralysis

A

Sniff test

  • Paradoxial upward motion of the paralyzed diaphragm > 2 cm during sniffing under fluoroscopy
  • US and MRI alternative modalities
21
Q

Definitive diagnostic study to evaluate diaphragm paralysis

A

EMG (w/ phrenic nerve stimuation)

22
Q

Surgical TOC for diaphragmatic eventration and paralysis

A

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
23
Q

Acceptable period of observation for suspected diaphrgam paresis

A

1-2 years (unless symptomatic)

24
Q

Indications for diaphragmatic pacing

A
  • Rarely used technique
  • Indicaitons:
    • Central alveolar hypoventilation (Ondine’s curse)
    • High cervical cord injury (above C3)
    • ALS (to delay need for mechanical ventilation)
25
Q

Diaphragm pacing technique

A

Stimulation of phrenic nerve (in neck or chest) by contact with electrode

26
Q

Tumors of the diaphragm

A
  • 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
27
Q

Neurogenic diaphragm tumors may be associated with _

A

Hypertrophic pulmonary osteoarthropathy

28
Q

TOC for diaphragm tumors

A

Surgical resection with diaphragm repair/reconstruction (prosthetic mesh)

29
Q

Variety of clinical conditions characteried by passage of fluids, gases, tissues, secretons or intestinal content through diaphragmatic pores from abdomen to chest

A

Porous diaphragmatic syndrome

30
Q

Spontaneous pneumothorax occuring in women in relation to menses

A

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
31
Q

Surgical treatment for catamenial pneumothorax

A
  • Repair of diaphragmatic defect (if present)
    • Can use mesh to cover the diaphragm
  • Resection of endometrial implant
  • Pleurodesis and/or pleurectomy
  • Hormonal therapy
32
Q

Agenesis of diaphragm

A
  • Extremely rare
  • Usually diagnosed early after birth
  • High mortality rate
  • Familial inheritance with multifactorial genetic etiology
33
Q

Cantrell’s Pentology

A
  • 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
34
Q

Etiology of Cantrell’s Pentology

A

Failure of lateral mesoderm during 3rd week of gestation

35
Q

Treatment for Cantrell’s Pentology

A

Correction of:

  • Hernia defects (ventral and diaphragmatic)
  • Sternal defects
  • Intracardiac defects (VSD or LV diverticulum)