Spina Bifida – medical problems

This series is covering all you need to know about spina bifida. In the previous posts we looked at issues around diagnosis and antenatal counselling. This week we look at the problems associated with spina bifida.

All body systems are involved in spina bifida.   Issues may be:-

Neurological

• Hydrocephalus – shunt dysfunction; disconnection; infection; extravasation
• Meningitis
• Seizures
• Brain stem problems – central apnoea, vocal cord paralysis, swallowing dysfunction
• Eye problems – nystagmus, 6th nerve palsy, optic atrophy, amblyopia, impaired vision
• Arnold-Chiari malformation
• Syringomyelia
• Tethered cord syndrome
• Diastamatomyelia

In addition to the central nervous system problems, spina bifida also affects the peripheral nervous system.

Patients can have lower motor neurone dysfunction, which presents as flaccid paralysis of the muscles. This is a fixed deficit i.e. there are no improvement in nerve function, and no deterioration (unless there are complications). About 10% have some spasticity in lower limbs. Sensory deficit limits feedback about muscle function, position of limb, and learning to walk.

Orthopaedic

• Joint deformity
• Dislocation
• Subluxation
• Fractures: osteoporosis; osteopaenia; joint deformity; instability
• Osteomyelitis
• Osteoarthritis

Urological

Patients have inefficient emptying of bladder leading to:

•   Recurrent urinary tract infections
•   High pressure bladder
•   Ureteric reflux
•   Hydronephrosis
•   Renal damage – renal failure
•   Calculi – bladder, kidneys
•   Hypertension
•   Incontinence

Management of urological problems include:

• Intermittent catheterisation which may lead to ureteric stricture
• Appendicovesicostomy (where the appendix is used as a conduit between the skin and bladder) which may result in stenosis, granulation
• Dialysis with multiple subsequent issues
• Renal transplant with multiple subsequent issues

Gastrointestinal

• Constipation – haemorrhoids, anal fissure
• Diarrhoea
• Faecal incontinence
• Peritoneal shunt
• Inguinal hernia
• Rectal/vaginal prolapse
• Gastro-oesophageal reflux

Skin

Sensory loss – reduced pain, position sense resulting in:

• Pressure areas with subsequent cellulitis
• Ulcers
• Sinus tracts/fistulas
• Poor circulation/discoloration/cold limbs
• Excoriation (due to incontinence)
• Burns

Also:

• Lymphoedema
• Capillary haemangioma in myelomeningocele repair area
• Hairy patch in myelomeningocele repair area
• Excoriation/skin breakdown in myelomeningocele area secondary to scoliosis/lordosis

Back

Back pain is common – there are multiple causes

• Scoliosis
• Kyphosis
• Lordosis
• Pelvic obliquity
• Postural and seating issues
• Disc degeneration
• Occult fractures secondary to osteopaenia

Respiratory

• Obstructive apnoea
• Sleep apnoea
• Restrictive lung disease due to scoliosis/kyphosis
• Pneumonia
• Use of CPAP/BIPAP

Endocrine

• Short stature
• Precocious puberty
• Delayed puberty
• Male infertility
• Undescended testes
• Breast development (disproportionate to short spine)
• Obesity

Other problems include:

• Adjustment disorders – family, patient
• Anxiety
• Depression – parent, patient
• Cognitive impairment especially executive functioning
• Learning issues at school
• Time commitments to caring
• Hospitalisations – planned, urgent
• Sibling concerns
• Financial impacts
• Future children

You can check out the rest of the series here:-

• What is spina bifida?
• Antenatal counselling for spina bifida
• Medical problems in spina bifida
• Bladder and bowel issues in spina bifida
• Fertility issues in spina bifida
• Prognosis in spina bifida