Question

The Newborn with a Perinatal Injury or Congenital Malformation 1. State the classifications of birth defects. 2. Describe the preoperative nursing care of an infant with hydrocephalus. 3. Explain the difference between spina bifida occulta and spina bifida cystica. 4. What is a cleft palate and how is it treated? 5. How is clubfoot treated? 6. Explain the use of the Pavlik harness and spica cast in the treatment of developmental hip dysplasia.

Answer 1

Birth defects/ Congenital malformation

Any of several medical disorders that are present at birth is called birth defects.

1. classification of birth defects

Birth defects can be classified as Structural and functional congenital disorders.

Structural disorders are

* Heart defects ; A defect in the structure of heart and great vessels present at birth such as patent ductus arteriosus, tetralogy of Fallot.

* Abnormal limbs: Dysmelia is a limb abnormality such as Amelia( absence of limbs), phocomelia( malformations), Polydactyly( extra fingers). Club foot is another common abnormality

* Cleft lip and cleft palate

* Congenital Gastrointestinal malformations such as oesophageal atresia, stenosis

* Neural tube defects: example is  spina bifida

* Kidney and urinary tract congenital disorders

Functional congenital disorders are

* Metabolic disorders ;associated with the chemical reactions in the different system of the body, such as Phenylketonuria

* Sensory problems such as blindness, deafness

* chromosomal defects and brain problems such as Down Syndrome, Fragile X syndrome and disorders associated with development of language, speech etc.

2. Preoperative nursing care

Hydrocephalus : a usual congenital condition in which an abnormal accumulation of fluid in the central ventricles causes enlargement of skull and compression of the brain, destroying much of the neural tissue.

Pre operative nursing care include

* Monitor vital signs of the child every hourly

* Monitor arterial blood gas analysis

* Monitor haemoglobin level and sodium ( in case of vomiting) level

* Assess neurological status , reflexes

* Measure child head circumference

* Maintain the child's head in a neutral position

* provide oxygenation as needed

* Assess the signs of increased ICP( intra cranial pressure)

* head should be elevated to 30 degrees and support head to facilitate drainage of CSF and prevents accumulation

3. Spina bifida oculta and Spina bifida cystica

Spina bifida oculta is also called closed Spina bifida oculta. It is a malformation of one or more vertebrae. In this the birth defect in which a baby's spinal cord fails develop properly.

Spina bifida cystica is the presence of cleft in the vertebral column. It is a bony defect in which the covering of the spinal cord and brain( meninges) protrude through the cleft.

4. Cleft palate

Cleft palate is an opening in the roof of the mouth( palate). a congenital condition in which the two plates of the skull that form the hard palate( roof of the mouth) is not completely joined.

Treatment

* Surgical repair of cleft palate ( palatoplasty, bone grafting) at 9 to 12 months

* Speech therapy

* dental care