Hydrocephalus ailment in children, deadly but curable

Aside malaria and other endemic diseases that pose a serious threat to children in Sub-Saharan Africa, hydrocephalus, in its unusual manner and manifestations, is also an alarming health risk.

As defined by several medical journals, “hydrocephalus is excessive accumulation of cerebrospinal fluid (CSF) within the cranial vault due, to excessive production or inadequate absorption, which makes the head to grow bigger and larger, if not detected early and given required medical treatment.”

The attitude of government, civil societies, parents and general public to hydrocephalus in the country is a breach of children’s survival, development, protection and participation rights, as enshrined in the Child Rights Act.

The ailment is prevalent in developing countries and hindered by significant economic constraints and delays in treatment, whereby most patients don’t present for treatment at least seven months after onset of clinical symptoms.

Unlike Africa, the prevalence and incidence of hydrocephalus in developed nations is estimated at 0.9 to 1.2/1000 and 0.2-0.6/1000. And though there is no reliable estimate in Africa, but its incidence is likely higher, because of untreated or poorly treated neonatal meningitis and nutritional deficiencies.

According to a consultant obstetrician and gynaecologist, Dr. Sunday Adeboye, CSF is produced predominantly by choroid plexus of the four cerebral ventricles, at a rate of 20 mL/hour.

He said: “Hydrocephalus has been categorised as communicating or non-communicating. The former is due to failure of CSF absorption by the arachnoid villi, whereas the latter involves obstruction of CSF flow into the subarachnoid space. A small minority of cases exhibits excessive production of CSF, most commonly secondary to a choroid plexus papilloma.”

Adeboye explained that despite the prevalence of malaria in Africa and reports of associated hydrocephalus, there is no clear relationship between its cerebral form and subsequent hydrocephalus. The clinical presentation is characterised by noticeable signs and symptoms.

He listed the symptoms to include: fatigue, drowsiness, vomiting, headache, failing vision, deteriorating mental function and enlarged head circumference; with such signs as wide tense fontanel, papilloedema, reduced visual acuity, failure of upward gaze, general clumsiness, dyspraxic gait and increasing head circumference.

Adeboye, who was the Chief Medical Director (CMD) of State Specialist Hospital, Akure, however, said older children would not present with increased head circumference. Rather, they would often complain of the classic triad such as, headache, vomiting and lethargy.

He explained that hydrocephalus is a common childhood disorder and a known cause of neurological morbidity among infants, particularly in developing countries, and its management is quite challenging.

A 2015 report by Sahel Medical Journal, an official publication of the Usmanu Danfodiyo University Teaching Hospital, Sokoto, underscored that it has more prevalence in northern Nigeria. A study conducted by the Radiology department of the institution over a period of two years through transfontanelle US scans in 64 children, revealed that 39 boys and 25 had the ailment.

The results showed that “Fifty-two patients (81.3 per cent) had hydrocephalus of congenital origin. Eleven cases (17.2 per cent) had postmeningitic hydrocephalus, while only one case (1.6 per cent) was posthemorrhagic. Twenty-five patients (48.0 per cent) of the congenital cases were due to cerebral aqueduct stenosis. Eleven (21.2 per cent) of the congenital cases were from obstruction at the exit foramina of Luschka and Magendie resulting in the communicating type of hydrocephalus.”

Similarly, the Nigerian Journal of Clinical Practice, which undertook same medical exercise on 34 patients for a period of two years by the Department of Surgery, Neurosurgery Unit, Lagos University Teaching Hospital, Lagos, said: “Globally, the burden of hydrocephalus in paediatric population is high, with prevalence rate of 1.2/1000 children.

“The impact of hydrocephalus on the patient, caregivers and the healthcare system is enormous. Untreated hydrocephalus can result in delayed developmental milestones, blindness, learning difficulties and eventual death. The economic impact of hydrocephalus runs into millions of US dollars in Sub-Saharan Africa.”

Recently raising concern over society and people’s attitude towards the malaise, Founder of Liron Hydrocephalus Foundation in Nigeria (LHF), Mr. Martins Ohiani, decried the poor awareness and priority given to the menace by government and other vital agencies, describing people’s superstitious inclinations to it as disastrous.

Ohiani, who recounted the daunting experience he passed through, when trying to register the foundation, due to low-level awareness among relevant government agencies, said the high level of ignorance, even among parents of hydrocephalous patients, scales up morbidity rate.

He said: “It would interest you to note that this is probably the only legally registered foundation in Nigeria, with an interest to ameliorate the suffering posed by this condition. Children with this condition are dying daily, as society here views the condition as something spiritual that needs spiritual treatment in the church or mosque, or better still, in the hand of the herbalists, voodoo specialists and idol worshippers.

“This explains why cases are not reported early to hospitals. By the time families discover and are convinced that it is actually a medical condition, it is always too late, thus giving the children little chance of survival, as early diagnosis and treatment is the only guarantee for good life.”

Aside the superstitious beliefs and customs, he identified poor standard of living in the country as one of the obstacles against treatment of the disease, as many people with such health challenges resort to ineffective and more dangerous means for solution.

Sharing his experience, Ohiani said: “I have a 22-month-old hydro boy. He was diagnosed and treated with shunt placement, when he was four months old and revision at age eight months. He is doing very well presently and it is amazing how early detection and treatment can guarantee normal life for hydro children.”

The chairman of LHF and wife of the founder, Erelu Modupe Martins, said the burdens of the disease on children leaving with such disabilities is minute in comparison to the discrimination and stigmatisation the society put on them.

She explained that over 87 percent of babies born with spina bifida, another birth defect called NTD, which occurs when bones of the spine don’t form properly around the baby’s spinal cord, would also develop hydrocephalus.

She said: “Each year, 70 per cent of babies with hydrocephalus are born in developing world, with limited or no access to life saving neurological care. Many of these children will die. Hydrocephalus can occur at any point in life.

“A further form of hydrocephalus occurs only in later life known as normal pressure hydrocephalus (NPH), which can be easily compared with what might be regarded as normal symptoms of old age and dementia.”

The LHF boss explained that the advocacy to stem its rising incidence, led to the commemoration of October 25 annually as World Spina Bifida and Hydrocephalus Day.

“World Spina Bifida and Hydrocephalus Day is led by International Federation for Spina Bifida and Hydrocephalus and its member associations,” he said. “It is an opportunity to raise awareness and understanding about these health conditions. It is also a way to advocate and promote the rights of persons with these impairments.”

She said LHF aims to begin reducing the incidence of hydrocephalus in the country, by raising public awareness and through medical intervention for affected children, whose case are congenital and present at birth or acquired and develop during or at any point after birth.

“The world hydrocephalus day is very significant, as it reminds the public and policy makers that hydrocephalus is a reality and that it is imperative to increase awareness about it and improve the lives of people living with this impairment,” she explained.