Tyrosinemia type I is a genetic disorder that disrupts the metabolism of the amino acid tyrosine, resulting in damage primarily to the liver along with the kidneys and peripheral nerves. The inability of cells to process tyrosine can lead to chronic liver damage ending in liver failure, as well as renal disease and rickets. Symptoms such as poor growth and enlarged liver are associated with the clinical presentation of the disease. Clinical manifestation of disease occurs typically within the first two years of life. The severity of the disease is correlated with the timing of onset of symptoms, earlier being more severe.Tyrosinemia type I is an autosomal recessive disorder caused by mutations in both copies of the gene encoding the enzyme fumarylacetoacetate hydrolase (FAH). FAH is a metabolic enzyme that catalyzes the conversion of fumarylacetoacetate to fumarate and acetoacetate. It is expressed primarily in the liver and kidney. Loss of FAH activity results in the accumulation of certain metabolic intermediates in the tyrosine catabolic pathway. These compounds are toxic to cells and lead to differential gene expression and apoptosis in high concentrations. HT1 is diagnosed when elevated levels of succinylacetone (SA), one of the metabolites in this pathway, is detected in blood and urine samples.While there is no cure for tyrosinemia type I, management of the disease is possible utilizing dietary restrictions and medications. A diet low in tyrosine and phenylalanine is utilized indefinitely once a diagnosis is suspected or confirmed.
It is necessary to mark drains in order to identify their location. They should be sutured with loops as well so it will be possible to advance them without re-anaesthetizing the patient while drains are re-sutured to the skin. An absorbent dressing is then applied. A bandnet dressing retainer can be constructed so as to prevent the use of tape. Other things to consider
Nutritional support
Adequate nutrition and hydration support is essential in any patient following surgery, particularly young children. In this case, pain and swelling in the neck region would usually cause difficulties in eating or swallowing, hence reducing patients food and fluid intake. Patients must therefore be well-nourished and hydrated to promote wound healing and to fight off infection. Post-operative care
Extubation, which is the removal of endotracheal tube to liberate the patient from mechanical ventilation, should only be done when the patients airway is proved to be patent, allowing adequate breathing. This is indicated by a decrease in swelling and patients capability of breathing adequately around an uncuffed endotracheal tube with the lumen blocked.During the hospital stay, patients condition will be closely monitored by:

carrying out cultures and sensitivity tests to decide if any changes need to be made to patients antibiotic course
observing for signs of further infection or sepsis including fevers, hypotension, and tachycardia
monitoring patients white blood cell count - a decrease implies effective and sufficient drainage
repeating CT scans to prove patients restored health status or if infection extends, the anatomical areas that are affected.