This is the Xray showing both knee joints of a 4 1/2 year old female who was brought to the orthopedic clinic by her mother complaining of a 1 1/2 year history of episodic left knee pain, worsening over the past 6 months, especially when walking.
On examiantion there was Bilateral varus deformity of lower extremities which was more prominent in the left side than the right side.
Identify this Xray ?
- Bilateral depression and deformity of medial aspect of proximal tibial epiphysis and metaphysis
- Beaking of proximal tibial metaphysis bilaterally
1) Mild rickets
2) A physiological bow legs
Blount disease is a growth disorder of the tibia characterized by disordered ossification of the medial aspect of the proximal tibial physis, epiphysis, and metaphysis. This progressive deformity is manifested by varus angulation and internal rotation of the tibia in the proximal metaphyseal region immediately below the knee.
Blount disease most likely is caused by a combination of excessive compressive forces on the proximal medial metaphysis of the tibia and altered endochondral bone formation
Two main types
Infantile Blount disease - tends to be bilateral and occurs in children less than 3 years.The infantile form is generally more common in females and children generally start to walk early, usually when aged 9-10 months.
Juvenile Blount disease - usually unilateral seen in children aged 4-10 years
- The medial epiphyseal line demonstrates irregularity
- Abnormal sloping of the medial tibial epiphysis
- The metaphyseal portion of the plate demonstrates irregular beaking
Treatment: Children can be treated by bracing, epiphyseal stapling, osteotomies, or in extreme cases, by intra-epiphyseal corrective realignment.
It is a congenital malformation of the diaphragm. The most common type of CDH is a Bochdalek hernia; other types include Morgagni's hernia, diaphragm eventration and central tendon defects of the diaphragm. Malformation of the diaphragm allows the abdominal organs to push into the chest thereby impeding proper lung formation.
CDH is a life-threatening pathology in infants, and a major cause of death due to two complications: pulmonary hypoplasia and pulmonary hypertension.
Newborns with CDH often have severe respiratory distress which can be life-threatening unless treated appropriately.
Congenital diaphragmatic hernia occurs 1 in 2200 births.
It occurs more commonly on the left than the right (5:1).
The affected baby, if not diagnosed prenatally, usually presents with respiratory distress between 12 and 24 hours after birth. Other classical signs include:
A scaphoid abdomen
Mediastinal shift to the opposite side
The majority of diaphragmatic hernia are detected antenatally
Postnatally the diagnosis is confirmed by chest radiology, which shows loops of bowel, or other abdominal viscerae, in the thorax.
Delayed X-ray following administration of contrast via the nasogastric tube shows loops of bowel in the hemithorax, confirming the congenital diaphragmatic hernia
DDx - cystic adenomatoid malformation of the lung
Stabilization before surgery- nasogastric aspiration, ventilation and bicarbonate infusion to correct acid-base and blood gas abnormalities
Reduction of abdominal contents and repair of the residual diaphragm with non-absorbable sutures
Postoperative ventilation and early oral feeding are recommended
The overall mortality is 50 to 60%
A good prognostic indicator is the position of the stomach; if it remains in the abdomen the prognosis is good; if it is in the chest the prognosis is poor.
Death is secondary to pulmonary hypoplasia.
X-ray Chest PA view in Kartagener’s syndrome showing dextrocardia and features of bronchiectasis, more on the left side. Left dome of diaphragm is higher and the fundal gas bubble is seen on the right side, indicating situs inversus.
Kartagener Syndrome , also known as immotile ciliary syndrome or Primary ciliary dyskinesia, is a rare, ciliopathic, autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract , sinuses, Eustachian tube, middle ear and fallopian tube.
Kartagener Syndrome = combination of situs inversus, chronic sinusitis, and bronchiectasis
It is thought that the molecular defect is in the dynein component of cilial apparatus.
Chronic recurrent respiratory infections, including sinusitis, bronchitis, pneumonia and bronchiectasis
Chronic suppurative otitis media
Infertility is common, due to defective ciliary action in the fallopian tube in affected females or diminished sperm motility in affected males
In the ECG there are inverted P waves in lead I abd there is reversed R-wave progression.