skeletal and digestive system

• Nursing Care of the Child Born with a Physical or Developmental Challenge
• Module 4: PHYSICAL & DEVELOPMENTAL DISORDERS OF THE SKELETAL SYSTEM
• Causes of Skeletal Disorders in the Newborn

  • Genetic
  • Environmental
  • Maternal drug ingestion
  • Virus invasion
  • Amniotic band formation in utero
  • Unknown
• Polydactyly


  Presence of one or more additional fingers or toes
• Syndactyly


  Two fingers or toes are fused, the fusion is usually caused by a simple webbing
• Pectus excavatum or "funnel chest"


  Indentation of the lower portion of the sternum, most common congenital deformity of the anterior chest, occurs in about 1 out of 500 live births, affects boys 4 times more often than girls
• Pectus carinatum


  The sternum is displaced anteriorly, increasing the anteroposterior diameter of the chest, can be surgically corrected
• Torticollis (Wry Neck)


  Occurs as a congenital anomaly when the sternocleidomastoid muscle is injured and bleeds during birth, occurs in newborns with wide shoulders when pressure is exerted on the head to deliver the shoulder either with a vaginal or cesarean birth
• Craniosynostosis


  Premature closure of the sutures of the skull, occurs in utero or early in infancy because of rickets or irregularities of calcium or phosphate metabolism, also occurs as a dominantly inherited trait, more often in boys than in girls
• Craniosynostosis


  1. Sagittal suture line closes prematurely - head tends to grow anteriorly and posteriorly

  2. Coronal suture line fuses early - orbits of the eyes become misshapen and increased intracranial pressure may lead to eye disorders, diagnosed by X-ray or ultrasound

  3. Sagittal - treatment may involve only careful observation

  4. Coronal - needs to be surgically opened to prevent brain compression and abnormally shaped head by 9 to 12 months
• Achondroplasia (Chondrodystrophia)


  Failure of bone growth inherited as a dominant trait, causes a disorder in cartilage production in utero, head continues to grow normally causing children's heads to appear unusually large in contrast to their extremities, forehead is particularly prominent and the bridge of the nose becomes flattened, trunks are of near-normal size, but a thoracic kyphosis and lumbar lordosis of the spine may develop
• Talipes Disorders


  Ankle-foot disorders, popularly called clubfoot, bone deformity and malposition of foot with soft tissue contracture, foot is twisted out of alignment, maybe misshapen, 1 in every 1000 children is affected, common in boys, probably inherited as a polygenic pattern, usually occurs as a unilateral problem

  
• Types of Talipes Disorders

  • Talipes equinovarus - plantar flexion (an equinus or "horse foot" position) with the forefoot lower than the heel
  • Dorsiflexion - the heel is held lower than the forefoot or the anterior foot is flexed toward the anterior leg
  • Varus deviation - the foot turns in
  • Valgus deviation - the foot turns out

  
• Assessment of Talipes Disorders

  1. Straightening all newborn feet to the midline as part of the initial assessment to detect this disorder

  2. Refer to the pediatric physician and orthopedist specialist to begin the process of evaluating the infant properly
• Therapeutic Management of Talipes Disorders

  1. For correction, a series of casts or braces are applied to gradually mold the foot into good alignment (a Ponseti method)

  2. The cast or brace extends above the knee to ensure a firm correction

  3. Change diapers frequently to prevent a wet diaper from touching the cast and causing it to become soaked with urine or meconium

  4. Check the infant's toes for coldness or blueness and how to blanch a toenail bed and watch it turn pink to assess for good circulation
• Developmental Dysplasia of the Hip (DDH)

  Improper formation and function of the hip joint, considered a spectrum of abnormalities affecting the hip joint, common musculoskeletal condition found in newborns, cause is unknown, may be from a polygenic inheritance pattern, uterine position that causes less-than-usual pressure of the femur head on the acetabulum, occur with breech birth, a female infant, and a mother's first pregnancy, additional risk factors: family history of DDH, oligohydramnios, large birth weight for gestational age, metatarsus adductus, and torticollis

  
• Assessment of Developmental Dysplasia of the Hip (DDH)

  1. All infants should be screened for DDH from birth and up until 3 months of age by performing the Ortolani & Barlow maneuver

  2. Detecting developmental dysplasia of the hip in the newborn is important because the longer the condition goes undetected, the more difficult it is to correct

  3. On inspection, the affected leg may appear slightly shorter than the other because the femur head rides so high in the socket

  
• Ortolani and Barlow Signs

  • Ortolani sign - a clicking or clunking sound is heard when a displaced femoral head reenters the acetabulum
  • Barlow sign - a feeling of the femur head slipping out of the socket posterolaterally, indicative of hip instability associated with the developmental dysplasia of the hip

  
• Procedure for Assessing Ortolani and Barlow Signs


  1. Lay the infant supine and flex the knees to 90 degrees at the hips

  2. Place your middle fingers over the greater trochanter of the femur and your thumb on the internal side of the thigh

  3. Abduct the hips while applying upward pressure over the greater trochanter, and listen for a clicking sound

  4. With your fingers in the same position and holding the hips and knees at 90-degree flexion, apply a backward pressure (down and laterally) and adduct the hips, note any feeling of the femoral head slipping
• Therapeutic Management of Developmental Dysplasia of the Hip (DDH)

  1. Close monitoring in mild DDH cases, as 60% to 80% of clinically identified abnormalities and 90% of ultrasonographic abnormalities spontaneously resolve without treatment in early infancy

  2. Infants less than 6 months of age - treated by using flexion-abduction splinting devices

  3. Correction of subluxated and dislocated hips involves positioning the hip into a flexed, abducted (externally rotated) position to press the femur head against the acetabulum and cause the acetabulum to deepen its contour from the pressure

  4. Brace and splints, such as the von Rosen, Pavlik, Craig, or Frejka, may be utilized for treatment of an unstable hip, and patient–family preferences should have a substantial influence on which type is chosen

  5. The Pavlik harness is shown to have a high success rate in treating subluxation and reducible DDH

  6. There will be a small number of children who do not achieve correction by noninvasive methods will have corrective and therapeutic hip surgery, which may involve having a pin inserted to stabilize the hip

  
• Therapeutic Devices for Developmental Dysplasia of the Hip (DDH)

  • Pavlik harness
  • Hip abduction cast
  • Spica cast

  
• Ankyloglossia (Tongue-tie)


  Congenital anomaly in which a tight or shortened lingual frenulum causes restricted tongue mobility and impaired tongue function, normally the frenulum appears short and is positioned near the tip of the tongue, may cause difficulty with breastfeeding due to difficulty with tongue mobility and the ability to latch to create a strong suction at the breast
• Treatment of Ankyloglossia (Tongue-tie)


  Frenotomy release can be performed in the newborn period
• Thyroglossal Cysts


  Irregular neck mass or a lump which develops from cells and tissues left over after the formation of the thyroid gland during developmental stages, arises from an embryogenic fault that leaves a cyst formed at the base of the tongue, which then drains through a fistula to the anterior surface of the neck, are congenital defects located in or around the midline of the neck, extending to the base of the tongue, may occur as an autosomal dominant trait
• Thyroglossal Cysts


  The cyst may involve the hyoid bone and may contain aberrant thyroid gland tissue, if infected, the cyst often appears swollen and reddened, with drainage of mucus or pus from the anterior neck and requires antibiotic therapy
• Cleft Lip


  The fusion fails to occur in varying degrees, causing this disorder to range from a small notch in the upper lip to total separation of the lip and facial structures up into the floor of the nose, with even the upper teeth and gingiva absent, cleft lip is the most common orofacial cleft, the deviation may be unilateral or bilateral, the infant's nose generally appears flattened because the incomplete fusion of the upper lip has allowed it to expand in a horizontal dimension
• Causes of Cleft Lip

  Appears to be caused by the transmission of multiple genes aided by teratogenic factors present during weeks 5 to 8 of intrauterine life, such as a viral infection, certain seizure medicines such as phenytoin, maternal smoking or binge drinking, hyperthermia, stress, and maternal obesity, folic acid deficiency may also be associated with incomplete anterior midline closures
• Cleft Palate


  An opening of the palate that occurs when the palatal process does not close as usual at approximately weeks 9 to 12 of intrauterine life, the incomplete closure is usually on the midline and may involve the anterior hard palate, the posterior soft palate, or both, may occur as a separate anomaly or in conjunction with a cleft lip, occur more frequently in girls, incidence rate: 1 out of every 1,000 births
• Assessment of Orofacial Clefts: Cleft Lip & Cleft Palate


  Cleft lip may be detected by a sonogram while an infant is in utero, apparent on inspection of the mouth at birth, when assessing newborns, be sure you have good lighting so you can visualize the palate clearly, because cleft palate is a component of many syndromes, assess the child for other congenital anomalies as well
• Therapeutic Management of Orofacial Clefts: Cleft Lip & Cleft Palate


  If discovered at birth, a cleft lip can be repaired surgically shortly thereafter, often at the time of the initial hospital stay or between 2 and 12 weeks of age, because the deviation of the lip interferes with sucking, infants may be a better surgical risk as newborns than they are after a month or more of poor nourishment, because facial contours change as a child grows, a revision of the original repair or a nasal rhinoplasty to straighten a deviated nasal septum may be necessary when the child reaches 4 to 6 years of age
• Pierre Robin Sequence


  Rare triad of micrognathia (small mandible), cleft palate, and glossoptosis (posterior displacement of the tongue)
• Cleft lip


  Detected by sonogram while infant is in utero
• Cleft lip


  Apparent on inspection of the mouth at birth
• When assessing newborns, be sure you have good lighting so you can visualize the palate clearly
• Because cleft palate is a component of many syndromes, assess the child for other congenital anomalies as well
• OROFACIAL CLEFTS: CLEFT LIP & CLEFT PALATE
• Therapeutic Management of cleft lip

  1. If discovered at birth - a cleft lip can be repaired surgically shortly thereafter, often at the time of the initial hospital stay or between 2 and 12 weeks of age

  2. Because the deviation of the lip interferes with sucking, infants may be a better surgical risk as newborns than they are after a month or more of poor nourishment

  3. Because facial contours change as a child grows, a revision of the original repair or a nasal rhinoplasty to straighten a deviated nasal septum may be necessary when the child reaches 4 to 6 years of age
• Pierre Robin Sequence (Syndrome)

  Rare triad of micrognathia (small mandible), cleft palate, and glossoptosis (a tongue malpositioned downward)
• This triad of conditions can result in severe upper airway obstruction, which then may cause secondary respiratory distress due to upper airway obstruction
• Estimates of occurrence range from 1 in every 8,500 births to 1 in every 20,000 births