CHAPTER 27 Flashcards by Jonamarie Pabon

Commonly Affected Systems in Children Born with Physical or Developmental Challenges

• Skeletal system
• Gastrointestinal System
• Nervous System

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“tongue-tied”
- abnormal restriction of tongue by abnormally tight frenulum

ANKYLOGLOSSIA

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It is a failure of the maxillary & median nasal processes to fuse that range from small notch in the upper lip to total separation of lip & facial structure up into floor of the nose, with even upper teeth and gingiva absent.

CLEFT LIP

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It is a palate opening that usually on the midline & involves anterior hard palate, posterior soft palate, or both.
- palate process closes at 9th-12th WOOL

CLEFT PALATE

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a triad of micrognathia (small mandible, cleft palate, and glossoptosis (tongue malpositioned downward)

PIERRE ROBIN SYNDROME

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an example of cleft palate occuring as only one part of a syndrome

PIERRE ROBIN SYNDROME

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orifice/passage in the body that is closed or absent

ATRESIA

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-abnormally/surgically made passage between hollow or tubular organ and body surface, between 2 hollow or tubular organs

FISTULA

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obstruction of esophagus

ESOPHAGEAL ATRESIA

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Management of FISTULA, ATRESIA, ESOPHAGEAL ATRESIA

•emergency surgery
•antibiotics
•gastrostomy
•close monitoring until suture dissolves

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Abdominal contents protruding through wall of abdomen at the junction of umbilical cord and abdomen.
Intestines are usually herniated but sometimes it included STOMACH and LIVER

OMPHALOCELE

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usually converted and contained by THIN AMNION and CHORION LAYER with umbilical cord protruding from EXPOSED SAC

OMPHALOCELE

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defect < 4cm

HERNIA OF UMBILICAL CORD

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> 10cm

TRUE OMPHALOCELE EXIST

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covered by thin membrane (but can be ruptured)
central through umbilical ring

OMPHALOCELE

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No covering membrane (but there could be fibrous matter)
lateral to umbilicus (often to the right)

GASTROSCHISIS

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Management of OMPHALOCELE

surgery within 24hrs after birth
IF SMALL (one stage repair may be possible)
IF LARGE (surgical approach with the use of prosthetic patch
Silastic pouch / “silo”
TPN

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pt had hydramnios throughout pregnancy
findings: newborn is < 30mL gastric contents can be aspirated, distended abdomen and tender to palpate
- when infant vomit; NOT SOUR SMELLING, appearance may be GREEN or BLACK

STENOSIS NARROWING

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pain can be manifested by hard, forceful distressful crying, and legs pulling up against abdomen
Tachypnea as diaphragm is pushed up
abdominal flat-plate radiograph pr sonogram reveals ABSENCE OF AIR AT THE LEVEL OF OBSTRUCTION

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Confirmation of STENOSIS NARROWING because this aids greater visualization of point of obstruction

BARIUM ENEMA/SWALLOW

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other term is “anal stenosis

IMPERFORATE ANUS

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Assessment of IMPERFORATE ANUS INCLUDE THESE 3

• wink reflex is absent
• no stools will be passed
• distended abdomen

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is narrowing the end of tube that takes stool out of the body

ANAL STRICTURE

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• absence of stool
• passing of stools in other openings
• swollen belly
• absence of anal opening

Clinical Manifestations of Newborns with IMPERFORATE ANUS (first physical examination)

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Management of IMPERFORATE ANUS

• NPO until surgery done • temporary colostomy until repair, if possible • OGT/NGT insertion • IV for fluid and electrolyte replacement • Surgery

- protrusion of an abdominal organ (usually the stomach or intestine) through a defect - usually occurs on the left side causing CARDIAC DISPLACEMENT to the right side of chest and COLLAPSE of lungs

DIAPHRAGMATIC HERNIA

Assessment of DIAPHRAGMATIC HERNIA

• bowel removal can be don me via FETOSCOPY • dyspnea occurs due to lung compressed by an abdominal organ

• abdomen looks generally sunken • affected side has absent breath sounds • infant looks cyanotic • retraction in the intercoastal or subcoastal retractions • shunting of right-to-left by foramen ovale and may cause patency of ductus arteriosus

DIAPHRAGMATIC HERNIA assessment

Management of DIAPHRAGMATIC HERNIA

• surgical emergency repair: LAPAROSCOPY • semi fowlers position • NPO • NGT/gastrostomy insertion

protrusion.of a portion of the intestine through the umbilical ring, muscle and fascia surrounding umbilical cord

UMBILICAL HERNIA

creates bulging protrusion under the skin at the umbilicus rarely noticeable at birth while cord is still present but becomes increasingly noticeable at health care visits during first yr

UMBILICAL HERNIA

an excess CSF (cerebrospinal fluid) in the ventricles or the subarachnoid space

HYDROCEPHALUS

can result from fluid production by choroid plexus, the passage of fluid is blocked, or there is something that interferes CSF absorption

Excess CSF

to identify abnormalities in the body HYDROCEPHALUS

Transillumination Test

Management of HYDROCEPHALUS

• ACETAZOLAMIDE (diuretic) • tumor removal • laser surgery • VP shunt

build up of cerebrospinal fluid (CSF) in the brain

HYDROCEPHALUS

latin word which means "divided spine"

SPINA BIFIDA

- happens when the fusion of posterior laminae of vertebrae fails - poor perfusion is noticeable when dimpling, abnormal tufts of hair or any discoloration of skin

SPINA BIFIDA

benign disorder that does not need immediate intervention

SPINA BIFIDA OCCULTA

when meninges herniate through unformed vertebrae

MENINGOCELE

protruding mass appears and usually have the same size of an orange at the center back

MENINGOCELE

layer of skin or just clear dura mater covers the protrusion

MENINGOCELE

the spinal cord and meninges protruding the vertebrae same with meningocele

MYELOMENINGOCELE

- child can feel flaccidity, inadequate sensation of LE, uncontrolled bladder and bowel - absent of motor and sensory function at the end of spinal cords -> lower motor neuron damage

MYELOMENINGOCELE

this can differentiate the diagnosis of meningocele to myelomeningocele

CT scan Ultrasound MRI

a cranial meningocele or myelomeningocele

ENCEPHALOCELE

- disorder occurs most often in the occipital area of skull but may occur as a nasal or nasopharyngeal disorder - covered fully by skin, but they may be open or covered only by the dura

ENCEPHALOCELE

Management of ENCEPHALOCELE

• Prenatal ultrasound, fetoscopy • Fetoscopic sugery • CS birth • lower extremities observation • immediate surgery • voiding and nature of micturition and defecating assessment

- absence of cerebral hemispheres - mother may experience difficult labor, most of this condition present at breech

ANENCEPHALY

slow growth of brain that falls more than 3x below normal on growth charts

MICROCEPHALY

can be caused by infection, severe malnutrition, or anoxia in early infancy

MICROCEPHALY

- hip underdevelopment or imperfect hip can affect the head, acetabulum or both - occurs 7x more in females than males

DEVELOPMENTAL HIP DYSPLASIA

mildest form; femoral head remains in acetabulum

ACETABULAR DYSPLASIA

most common form; femoral head partially displaced

SUBLUXATION

femoral head not in contact with acetabulum; displaced posteriorly and superiorly

DISLOCATION

hip started dislocated - test will reduce the hip

ORTOLANI TEST

hip started reduced - test will dislocate the hip

BARLOW TEST

unequal height suggest developmental dysplasia of the hip or short femur

GALEAZZI TEST

spica cast from the waist below the knees; brace for DEVELOPMENTAL HIP DYSPLASIA pt

PAVLIK HARNESS

can result from ingestion of maternal drug, viral invasion, and amniotic band formation

ABSENT OR MALFORMED EXTREMITIES

prostheses of lower extremity are fitted as early as 6 months while upper extremities are fitted this early so infant can handle and explore objects readily

ABSENT OR MALFORMED EXTREMITIES

early introduction of this prevents child from adjusting to missing extremity

PROSTHESIS

a presence of one or additional fingers or toes usually amputated off early

POLYDACTYLY

when two fingers or toes are fuse

SYNDACTYLY

- indention of lower portion of sternum - there can be decreased lung volume, heart is displaced to the left

PECTUS EXCAVATUM

when the sternum is displaced anteriorly, increasing AP diameter of the chest

PECTUS CARINATUM

wry neck term derived from tortus (twisted) and collum (neck) congenital anomaly when the sternocleidomastoid muscle is injured and bleeds during birth

TORTICOLLIS

- suture of the skull is closing prematurely and may occur in utero or early infancy - this conditions needs to be detected early because sealed skull will compromise brain growth

CRANIOSYNOSTOSIS

can be cause by inherited trait, rickets,, irregularities of calcium, or phosphate of metabolism

CRANIOSYNOSTOSIS

- failure of bone growth inherited as a dominant trait - causes disorder in cartilage production in utero - the epiphyseal plate of long bones cannot produce adequate cartilage for longitudinal bone growth, which results in both arms and legs becoming stunted

ACHONDROPLASIA

from latin word talus (ankle) and pes (foot) - ankle foot disorder, clubfoot

TALIPES