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HAIZEL RABOY
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 Hirschsprung’s
Disease
It also called congenital aganglionic megacolon) occurs when some of your
baby’s intestinal nerve cells (ganglion cells) don’t develop properly, delaying
the progression of stool through the intestines. The intestine becomes blocked Shrunken
with stool, and your baby or child will be constipated (unable to have normal Absence of
Rectum
bowel movements). Hirschsprung’s disease occurs in one out of 5,000 births. Ganglion Cells

Etiology S&S Pathophysiology

● Genetic Common symptoms in infants NEW BORN & ADULT
● Associated Factors: Down include:
Syndrome, Trisomy 21, other ● Failure to have a bowel movement Male > Diet, Activity,
associations include Waardenburg in the first 48 hours of life Female Race
syndrome, congenital deafness, ● Abdominal distension (stomach Absence of Ganglion Cells
malrotation, gastric diverticulum, bloating)
and intestinal atresia, Congenital ● Gradual onset of vomiting (-) ganglionic innervation = (-) nerve cell
Heart Disease. Fever
● Constipation or failure to pass
Risk Factors regular bowel movements
(-) Peristalsis

● Having a sibling who has Chronic constipation Red, Dark Blood

Hirschsprung’s Disease In older children includes: Stool
● Being male ● Constipation that becomes worse
Intestinal Obstruction/ Impaction
● Having other inherited conditions with time
Discomfort Anemia
● Loss of appetite
Abdominal pain
● Delayed growth
Complications ● Passing small, watery stools Abdominal Fullness / Shock
● Abdominal distension Distension
Enterocolitis – it is life-threatening
situation DEATH
 Hirschsprung’s Treatment & Management
Disease ● Leveling Colostomy
● Single-stage Pull-through Procedure
● Ostomy Surgery
Diagnostics For child w/ milder defect:
● Management: dietary modification, stool softeners, isotonic
● Physical Examination irrigations to prevent impaction.
● Patient & Family History ● Injections of botulinum toxin (botox) into the anal sphincter to relax
● Abdominal Radiography this muscle
● Unprepared Singe-contrast Barium ● Appendicostomy
Edema
● Rectal Biopsy Singe-contast
● Rectal Manometry Barium Edema Ganglion Normal Colon
innervating colon

Nursing Diagnoses
Aganglionic
● Constipation related to decreased bowel motility. Colon
● Imbalanced nutrition: less than body requirements related to anorexia. Resected
● Fear (in the older child) related to impending surgery.
● Risk for impaired skin integrity related to irritation from the colostomy. Pull-through Surgery Preserved Anal
Acute pain related to the surgical procedure. Colon
● Deficient fluid volume related to postoperative condition.
● Impaired oral and nasal mucous membranes related to NPO status and
irritation from NG tube. Nursing Interventions
● Deficient knowledge of caregivers related to understanding of
postoperative care of the colostomy. ● Observance for the passage of meconium
● Thorough history of weight gain, nutritional
intake & bowel habits
● Fluid & electrolyte balance
● Teach parents how to ensure regular bowel
movements
● Daily rectal irrigations w/ normal saline
● Prevent skin breakdown in the rectal area
● Protective ointment at each diaper change
 Neural Tube Defects Therapeutic Management
● A neurosurgical
● Genetic counselling or screening
● Open NTD: Should be kept warm and the defect covered with a
sterile wet saline dressing, with consideration for prophylactic
Any of a group of congenital malformations involving defects in the skull and
antibiotics
spinal column that are caused primarily by the failure of the neural tube to ● Positioned in the prone position to prevent pressure on the defect
close during embryonic development. Also known as spinal dysraphisms, are
● Myelomeningocele: Prompt closure within the first 72 hours after
a category of neurological disorders related to birth defects of the brain, spine,
birth
or spinal cord. The exact causes of NTDs are not known.  ● Closed NTD associated with cord tethering: Surgery involves
removal of structures that are anchoring the cord
Etiology Risk Factors ● Severe hydrocephalus: Ventriculoperitoneal shunt placement should
be considered at the time of myelomeningocele closure
Related factors: ● Obesity Diagnostics
● Genetic ● Have poorly controlled ● Maternal Serum Alpha Fetoprotein (MSAFP), a screening test that
● Nutrition diabetes is performed on a pregnant woman's blood at approximately 16-18
● Environment ● Take certain antiseizure weeks of pregnancy
medicines ● High Resolution Ultrasound, which may detect an NTD visually
after approximately 18 weeks of pregnancy. Some severe forms of
Assessment NTDs such as anencephaly, may be detected earlier than 16 weeks
● Fetoscopy ● Prenatal UTZ ● Amniocentesis, a test that samples the amniotic fluid (fluid that
● Amniocentesis surrounds the baby) after 15 weeks of pregnancy.
● Observe and record whether an infant born with NTD has spontaneous
movement of the lower extremities
● Assess the nature & pattern of voiding & defecation
Nursing Diagnoses
● Risk for infection r/t rupture or bacterial invasion of the neural tube
sac
Prevention ● Risk for imbalanced nutrition, less than body requirements, r/t
● Folic Acid Supplement (400 mcg each day) difficulty assuming normal feeding position.
● Vitamin C, and Riboflavin supplementation ● Risk for ineffective cerebral tissue perfusion r/t increased
● Foods w/ folic such as: leafy green vegetables, fruits, dried beans, peas, & intracranial pressure
nuts, cereals, grain products ● Impaired physical mobility r/t neural tube disorder
● Risk for impaired elimination r/t NTD
 Types of NTD
1 Anencephaly
It is a congenital absence of major portions
of the brain and malformation of the brain
stem.
Occurs when the head end of the neural tube
fails to close during the 23rd & 26th days of
pregnancy. They are usually blind, deaf and
unconscious They will never regain
consciousness Infants are either stillborn or
usually die within a few hours after birth.
2 Microcephaly
a disorder in which brain growth is so slow
that it falls more than three standard
deviations below normal on growth charts.
2 Encephalocele
Characterized by protrusions of the brain
through the skull that are sac like covered
with membrane. They can be grove down
the middle of the upper part of the skull,
between the forehead & nose or the back of
the skull Often obvious & diagnosed
immediately.
 Types of NTD
1 Spina Bifida Occulta
Spina bifida occulta occurs when the
posterior laminae of the vertebrae fail to
fuse. This occurs most commonly at the fifth
lumbar or first sacral level but may occur at
any point along the spinal canal. It is
identified externally by a skin depression or
dimple, dark tuft hair, or soft subcutaneous
limpomas at the site.
1 Meningocele
If the meninges covering the spinal cord
herniate through unformed vertebrae, a
meningocele occurs. The anomaly appears
as a protruding mass, usually approximately
the size of an orange, at the center of the
back.
1 Myelomeningocele
the spinal cord and the meninges protrude
through the vertebrae the same as with a
meningocele. The difference is that the
spinal cord ends at the point, so motor and
sensory function is absent beyond this point.
Hydrocephalus CFS in Ventricles
CFS
CFS build-up in
Ventricles

Hydrocephalus is a condition in which excess cerebrospinal fluid (CSF) builds

up within the fluid-containing cavities or ventricles of the brain. This excess
fluid causes enlargement of the skull.
● Extraventricular Hydrocephalus (Communicating) – if there is a block to Blocked CFS Flow
such passage of fluid
● Intraventricular Hydrocephalus (Noncommunicating) – if fluid can reach
the spinal cord

S &S
Etiology
Early Signs:
● Congenital structural defect in infancy ● Dandy-Walker syndrome ● Rapidly increasing head
● Chiari II Malformation ● Intraventricular hemorrhage circumference
● Sylvius/ aqueductal stenosis ● Bossing Wide, bulging
The amount of CSF can increase when: fontanelle
●“Cracked-pot” sign
● Obstruction. a blockage develops that prevents CSF from flowing normally ● Prominent, distended scalp Prominent scalp veins
● Poor Absorption. there is a decrease in the ability of blood vessels to absorb veins, translucent scalp skin
it ● Sun setting eyes
● Overproduction. your brain produces an excess amount of it. Too much of ● Increased tone Increased head
this fluid puts your brain under too much pressure. This pressure can cause circumference
● Irritability or lethargy and
brain swelling, which can damage your brain tissue. poor feeding Sunset eyes
● Decline in level of
Diagnostics consciousness
● UTZ ● MRI ● Difficulty holding head up
● CT – scan ● Skull x-ray Late Signs:
● Transillumination ● Apnea
● Daily measurement of head ● Vomiting
circumference ● High pitch cry
● Difficulty swallowing or
Hydrocephalus
Risk Factors Nursing Interventions
Newborns ● Preventing injury
● Abnormal development of the central nervous system that can obstruct the ● Promoting skin integrity
flow of cerebrospinal fluid ● Preventing infection
● Bleeding within the ventricles, a possible complication of premature birth ● Promoting growth and
● Infection in the uterus during a pregnancy, such as rubella or syphilis, that development
can cause ● inflammation in fetal brain tissues ● Reducing family anxiety
Other contributing factors
● Lesions or tumors of the brain or spinal cord
● Central nervous system infections, such as bacterial meningitis or mumps Treatment & Management
● Bleeding in the brain from a stroke or head injury
● Other traumatic injury to the brain ● Surgery
● Ventriculoperitoneal (VP) Shunt surgery
● Ventriculoarterial (VA) Shunt
Nursing Diagnoses ● Lumboperitoneal Shunt
● Shunts are replaced: over time as a child grows and needs a
● Risk for ineffective cerebral tissue perfusion r/t increased intracranial bigger shunt. if they get infected or stop working
pressure ● Endoscopic third ventriculostomy (ETV) surgery
● Risk for imbalanced nutrition, requirements, pressure less than body r/t  
increased intracranial
● Risk for impaired skin integrity r/t extra weight and immobility of head
● Deficient Knowledge r/t the family’s understanding of the child’s condition Pharmacologic Management
and home care
● Risk for Injury r/t increased ICP ● Diuretics
● Risk for Infection r/t the presence of a shunt. ● Anticonvulsants
● Risk for Delayed Growth and Development r/t impaired ability to achieve ● Antibiotics
developmental tasks
● Anxiety r/t the family caregiver’s fear of the surgical outcome.
Online References
Belleza, R. M. N. (2021c, February 11). Hirschsprung disease (aganglionic megacolon). Nurseslabs.
https://nurseslabs.com/hirschsprung-disease-aganglionic-megacolon/

Belleza, R. M. N. (2021c, February 11). Hydrocephalus. Nurseslabs. https://nurseslabs.com/hydrocephalus/

Hirschsprung’s disease | boston children’s hospital. (n.d.). Boston Children’s Hospital. Retrieved May 13, 2021, from https://
www.childrenshospital.org/conditions-and-treatments/conditions/h/hirschsprungs-disease

Hydrocephalus - symptoms and causes. (2019, July 26). Mayo Clinic.

https://www.mayoclinic.org/diseases-conditions/hydrocephalus/symptoms-causes/syc-20373604#:%7E:text=Hydrocephalus
%20is%20the%20buildup%20of,the%20brain%20and%20spinal%20column
.

Hydrocephalus – causes, symptom and surgical treatments. (n.d.). AANS. Retrieved May 13, 2021, from https://
www.aans.org/en/Patients/Neurosurgical-Conditions-and-Treatments/Hydrocephalus

Neural tube defects (NTDs) | duke molecular physiology institute. (n.d.). Duke Molecular Physiology Institute. Retrieved
May 14, 2021, from
https://dmpi.duke.edu/neural-tube-defects-ntds#:%7E:text=Neural%20tube%20defects%20are%20considered,certain%20anti
convulsant%20(antiseizure)%20medications
.

Neural tube defects. (n.d.). MedlinePlus. Retrieved May 14, 2021, from https://
medlineplus.gov/neuraltubedefects.html#cat_93

Shimony, N. (2021, April 22). Neural tube defects treatment & management: Medical care, surgical care, consultations.
Medscape. https://emedicine.medscape.com/article/1177162-treatment#d6