Nursing Care of Children with Physical or Developmental Challenges

Fetal Surgery and Pierre Robin Syndrome

• Fetal surgery can be performed in utero between 2-10 weeks to repair nasal mold apparatus to shape better nostrils.
• Pierre Robin syndrome is a triad of micrognathia (small mandible), cleft palate, and glossoptosis (tongue malpositioned downward).

Tracheoesophageal Atresia and Fistula

• Tracheoesophageal atresia is an orifice or passage in the body that is closed or absent.
• Fistula is an abnormal or surgically made passage between a hollow or tubular organ and the body surface, or between two hollow or tubular organs.
• Esophageal atresia is an obstruction of the esophagus.

Esophageal Atresia Classification

• Type D: 1% Proximal and distal fistulas (“Double Fistula”)
• Type E: 4% No atresia of the esophagus

VACTERL Association

• Vertebral anomaly
• Anal atresia
• Cardiac anomaly
• Tracheoesophageal fistula
• Esophageal atresia
• Renal anomaly
• Limb malformation
• About 50% of cases are associated with other mesoderm defects.

Clinical Features

• Coughing spells (saliva overflows from the esophagus into the trachea)
• Rales
• Cyanotic attacks

Omphalocele and Gastroschisis

• Omphalocele: a central defect through the umbilical ring, covered by a thin membrane (can be ruptured), with intestines, liver, and other organs outside the abdomen in a sac.
• Gastroschisis: a lateral defect to the right of the umbilicus, no covering membrane (can have fibrous matter), with intestines outside the abdomen through a hole.

Commonly Affected Systems

• Skeletal system
• Gastrointestinal system
• Nervous system
• Physical and developmental challenges

Ankyloglossia (Tongue Tied)

• Abnormal restriction of tongue by an abnormally tight frenulum
• The only management is surgery.

Cleft Palate and Lip

• Failure of the maxillary and median nasal processes to fuse
• Can range from a small notch in the upper lip to total separation of lip and facial structure
• Can be unilateral or bilateral
• More common in boys
• The palate process closes at 9th-12th week of intrauterine life
• Usually occurs in conjunction with cleft lip.