- It is the absence of a fetal limb or fetal part at birth.
- This condition may be the result of the constriction of fibrous bands within the membrane that surrounds the developing fetus (amniotic band syndrome) or the exposure to substances known to cause birth defects (teratogenic agents).
Etiology
- Unknown
- There have been reports associating congenital amputation with maternal trauma, oophorectomy during pregnancy, intrauterine contraceptive device and amniocentesis.
- There are case reports in families with connective tissue disorderes (Ehler-Danlos syndrome).
- A teratogenic insult might be implicated as causing the disorder.
Pathophysiology
- Harmful influences in the environment of the developing fetus possibly account majority of congenital amputation.
- Factors shown to have an adverse effect on the human fetus, heavy irradiation, rubella, toxoplasmosis, and teratogenic agents.
- Harmful factors produce damage, differentiation of tissues and structures in taking place at the first trimester of pregnancy.
- Anomalies of mesodermal development.
- Rupture of amnion.
- In limbs, constricting rings ordilarily do not extend below deep fascia. In some instances they reach deeply to attach to the periosteum.
Signs and Symptoms
Limbs
- Constriction rings of limbs or digits
- Lymphedema
- Pseudosyndactyly (involves only the distal portion of the digits).
- Abnormal dermal ridge patterns
- Simian crease
- Club feet
Cranium
- Multiple and asymmetric cephaloceles
- Anencephaly
- Acrania
Face
- Cleft lip, palate, and face
- Nasal deformities
- Asymmetric microphthalmos
- Incomplete or absent calcifications of the skull
Thorax
- Rib clefting
- Scoliosis
Abdominal wall
- Gastroschisis
- Omphalocele
- Bladder exstrophy
- Perineum
- Ambiguous genitalia
- Imperforate anus
Assessment
- History of Prenatal Care, drugs taken at first trimester of pregnancy.
- Familial history
- Physical examination with emphasis on musculoskeletal system.
- Prenatal test
- Chorionic villi sampling
- Amniocentesis
- Triple screen of alphafetoprotein
- Screens for teratogenic communicable disease.
- Radiographic visualization of absence of affected limb.
Nursing Diagnoses
- Ineffective breastfeeding
- Ineffective breathing pattern
- Altered tissue perfusion
- Impaired ohysical mobility
- Activity intolerance
- Risk for aspiration
- Self-care deficit
- Knowledge deficit
- Ineffective family coping
- Disturb body image
Nursing Interventions
- Demonstrate how to support and position the baby for effective breastfeeding.
- Encourage adequate fluid intake.
- Always assess patient’s breathing pattern.
- Encourage position of comfort.
- Assess amount of consistency or respiratory secretions and strength of gag reflex.
- Teach the patient proper toilet training.
- Assist the patient to develop self-independence as he is capable of assuming.
- Allow the patient to have prosthesis to have a normal development and less wasting of muscles of the limbs.
- Consult the patient to a surgeon for possible muscle transplant and bone drafting.
- Refer the patient to occupational and physical therapist.
Complications
- Myelomeningocele
- Myelodysplasia
- Paralysis
- Sensory loss
- Tropic ulcers
- Osteomyelitis
- Contractures
- Bowel and bladder incontinence
