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Wilson–Mikity syndrome

Wilson–Mikity syndrome, a form of chronic lung disease (CLD) exist only in premature infants and leads to progressive or immediate development of respiratory distress.^[1] This rare neonatal morbidity condition that affects low birth babies and is characterized by rapid development of lung emphysema upon birth, requiring prolonged ventilation and oxygen supplementation. ^[2]

Symptoms and diagnosis

Infants display deteriorating respiratory symptoms along with early chronic lung changes which can be seen on chest radiography.^[2] These changes are diagnosed either directly upon birth or within the first month, as the premature infant requires mechanical ventilation for survival. The onset of respiratory difficulty occurs at the first day of life and continues up to three weeks into the infant's life, at which point treatment is needed for infant survival.^[3] The diagnosis of Wilson-Mikity syndrome can be made through two distinct symptoms: analogous characteristics of respiratory distress syndrome and presence of diffuse and streaky infiltrates with small cystic changes, seen through a chest X-ray. Early screening allows for the identification of a collapsed lung, cystic changes within the lung, and possible start of right-sided heart failure. Upon autopsy, alveolar collapse and alveoli rupture can be seen. This can reduce the number of capillaries within the system and lead to cyanosis. Cyanosis occurs from chronic or intermittent respiratory distress and episodes of dyspnea (or apnea). Symptoms can develop within hours post-birth or be gradual; infants will experience transient respiratory distress, causing a lapse in diagnosis by around 30 to 40 days. Dangerous recurrent apnea (or dyspnea) can occur in the first two to six weeks postpartum. This cessation of breathing can progress to cyanosis and lung collapse.

Around 75% of affected infants survive and are able to receive oxygen therapies and treatments to overcome this disease. In fatal cases, infants do not have noticeable or substantial respiratory recovery and can develop right-sided heart failure, ultimately leading to death. Patients that are not recovering will also continue to show signs of dyspnea, respiratory distress, and continued low body weight, heightening the risk of death. Infants that survive six months or longer have substantially better prognosis.

Mothers who have developed chorioamnionitis during pregnancy put their infant at higher risk for development of Wilson-Mikity syndrome. It is a rare complication that requires prolonged treatment.^[1] Infection, however, is not shown to be an etiological factor, but a correlation to chorioamnionitis is identified as a risk.

The cause is Wilson-Mikity syndrome is unknown.

Treatment and outcome

When caught early enough, continuous, mechanical oxygen therapy can be used to reverse the infant's poor circulation and decreased blood oxygen, a symptom known as cyanosis. Improvement is gradual; however, cases show that after the first year of treatment using oxygen therapy and mechanical ventilators, infants show normal respiratory activity and are free from chest infiltrates with small cystic changes. Absence of fever, and normal white blood cell count correspond to successful reversion and allow for a positive prognosis.

Treatment methods have the possibility to put the infant at risk for rare complications if oxygen supplementation and ventilation is not performed properly. When not enough oxygen is allowed to the infant, the apnea continues and the infant is unable proper recovery, too much oxygen administered can lead to higher risk for retrolental fibroplasia and/or oxygen toxicity within the lungs. Continued dyspnea is a sign Wilson-Mikity syndrome is still affecting the infant and treatment would have to be treated with increased ventilation, those patients in recovery are slowly taken off ventilation and eventually are able to ventilate with minimal to no respiratory distress.

There is a lack in research on the fully long term effect of Wilson-Mikity throughout adulthood.

Related Pages

References

^ ^a ^b "Awareness of Wilson-Mikity Syndrome: A Rare Complication of Chorioamnionitis". Taiwanese Journal of Obstetrics and Gynecology. 44 (3): 278–280. 2005-09-01. doi:10.1016/S1028-4559(09)60155-8. ISSN 1028-4559.
^ ^a ^b Takami, Takeshi; Kumada, Atsushi; Takei, Yukito; Miyajima, Tasuku; Hoshika, Akinori (2003). "A Case of Wilson-Mikity Syndrome With High Serum KL-6 Levels". Journal of Perinatology. 23 (1): 56–58. doi:10.1038/sj.jp.7210830. ISSN 1476-5543.
^ Grossman, Herman; Berdon, Walter E.; Mizrahi, Abraham; Baker, David H.; Grossman, Herman (1965-09-01). "Neonatal Focal Hyperaeration of the Lungs (Wilson-Mikity Syndrome)". Radiology. 85 (3): 409–417. doi:10.1148/85.3.409. ISSN 0033-8419.

Combination of major edits to Wilson-Mikity syndrome and minor edits to Lingual septum and Chorioamnionitis

Lingual septum

The lingual septum is closely associated with the hyoglossus membrane, allowing the binding of the tongue to the hyoid muscles.

Chorioamnionitis

Added grammar corrections and refined throughout every section.

Outcomes

...and Wilson-Mikity syndrome have been associated to the bacterial infection.

Complications

Neonatal complications

Infant respiratory distress

[:0-1] "Awareness of Wilson-Mikity Syndrome: A Rare Complication of Chorioamnionitis". Taiwanese Journal of Obstetrics and Gynecology. 44 (3): 278–280. 2005-09-01. doi:10.1016/S1028-4559(09)60155-8. ISSN 1028-4559.

[:1-2] Takami, Takeshi; Kumada, Atsushi; Takei, Yukito; Miyajima, Tasuku; Hoshika, Akinori (2003). "A Case of Wilson-Mikity Syndrome With High Serum KL-6 Levels". Journal of Perinatology. 23 (1): 56–58. doi:10.1038/sj.jp.7210830. ISSN 1476-5543.

[:2-3] Grossman, Herman; Berdon, Walter E.; Mizrahi, Abraham; Baker, David H.; Grossman, Herman (1965-09-01). "Neonatal Focal Hyperaeration of the Lungs (Wilson-Mikity Syndrome)". Radiology. 85 (3): 409–417. doi:10.1148/85.3.409. ISSN 0033-8419.

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