It’s one thing to see a mother cradling her child, tears of joy, relief and gratitude rolling down her cheeks, but quite another when you hear how a Tanzanian family, the *Juma’s managed to secure life-changing cleft-palate surgery for their two-year-old daughter, *Amani.

They’d been to a public hospital in their hometown of Vanderbijlpark where the front desk said they could not admit him because they did not do cleft palate surgery. They suggested the family try Chris Hani Baragwanath Hospital in Soweto, but the parents thought they’d be turned away. 

At a cost of R198 000, the private route was not an option. As they were about to give up, a friend told them that he’d seen an Operation Smile poster promoting a five-day programme at the private Nelson Mandela Children’s Hospital in Johannesburg. It seemed too good to be true, especially when the father googled the director of Operation Smile South Africa, Professor Anil Madaree to discover he’s one of the country’s leading plastic surgeons.

*Amani was among 61 children afflicted with variations of the same condition who were screened at the hospital. A total of 41 of them were found to be surgery-appropriate, all aged between nine months and 12 years.

The need for cleft lip and palate care in South Africa is huge, but late presentation and low awareness are major treatment barriers for disfiguring craniofacial conditions. 

“People don’t seem to know that this op is available free through Operation Smile. But more importantly, we have stunning infrastructure and appropriate surgeons available in Gauteng. The system is failing them. You’d think the numbers of patients coming through would be growing, but those we do see often present late, many are already teenagers when it’s more difficult to get optimal results – and even people all the way up to seventy-year-olds often need help,” says Nelson Mandela Children’s Hospital anaesthetist Clover Lee, one of the Operation Smile volunteer team.  

The most common among these birth defects are cleft lip which is a visible split in the upper lip; cleft palate is when there’s a split in the roof of the mouth and is typically only visible when a baby’s mouth is open. The conditions cause debilitating emotional and physical trauma, including speech impediments, social stigma, educational handicaps and very often, major discomfort.

The causes of cleft lip and palate are genetic, dietary, and nutritional, with some of those afflicted dying, “simply because they’re not fed enough”. 

Late presentation  

According to Dr Saleigh Adams, a professor of plastic surgery at the University of Cape Town, cleft palate occurrence is highly variable geographically, but stands at one in five hundred Asians, one in a thousand Caucasians, and one in 2000 Africans. Cleft palate and lip and palate incidence was highest in South-East Asia. In the United States between 2010 and 2014, cases with cleft lip, with or without cleft palate was one in 1000 births.
With both it stood at one in 1563 births.

In South Africa, the incidence is 0.2 to 0.3 per 1000 of the population, but surgeons speak of weekly seeing adult patients for whom treatment at an earlier stage would have avoided a world of pain and stigma.  Surgical repair was best done at three to four months of age, enabling better postoperative physical development and adaptation.

“Ideally, you want to do a child at three months and before a year of age – before they talk and make sounds, otherwise their sounds and language get ingrained, and they learn bad speech habits,” explains Madaree, head of the cleft and craniofacial department of plastic and reconstructive surgery at Durban’s Albert Luthuli Tertiary Hospital. 

He says that when palate repair happened late, speech was not as good, but nevertheless improved by 60% or more. 

“The closer the op is between one and four years old, the better the result, better than say, after 35 years old. With the cleft lip one can get very good results no matter the age,” Madaree said.

Limited skills and resources 

With just ten maxillofacial surgeons and twenty plastic surgeons doing cleft palate surgery and ten appropriately tailored multi-disciplinary treatment clinics in South Africa, the need for transformative surgery and treatment remains “huge”, the country’s top experts say.

Skills transfer is essential, leading to the launch of the Operation Smile Skills Academy this year. Twenty-four newly qualified maxillofacial, plastic surgery specialists and younger registrars plus an equal number of colleagues on-line, hungry to learn from the thinly available expertise, attended a day long clinical teaching session at the hospital. Over the following two days they scrubbed in for a weekend of surgery led by four top surgeons.

Unlike some of the others, *Amani did not require intensive post-operative care, and the parents were assured that within days or a week, she would begin to speak and eat properly, gain weight, and thrive. 

Causes and Prevention 

Adams says that in embryology, the genesis of cleft palates and/or lips was the failure of fusion of the paired palatal shelves from the maxillary process. This usually happened between weeks five and 12 weeks in utero. In his opinion, the causes are approximately fifty percent genetic/hereditary, and fifty percent environmental. He said the University of the Western Cape was conducting advanced gene studies on the subject.

Adams breaks the environmental causes down as smoking (a twofold increased risk), alcohol (equivocal linkage), foliate deficiencies, steroids (a three-fold increased risk), anticonvulsants (a tenfold risk), and advises women that Vitamin A should be avoided if they are planning a pregnancy.

Existing evidence is both questionable and scarce because the capacity and ability to do research varies widely between countries.    

“There are too many extraneous and/or confounding factors, so it’s also very hard to research in order to institute preventative measures,” explains Madaree. 

Madaree says not all the genes that predispose a person to cleft lips or palates have been discovered, but risk factors included a four percent next generation risk if one child or either parent had a cleft lip or palate. If two children in the same family had clefts, the next generation risk increased to 25%.

“There are some other clue-giving syndromes. For example, Van der Wouder’s Syndrome shows up as pits on the lips or includes an almost invisible cleft in a sibling or parent. Then you know the next child has a chance of having it,” he says. Within the 34 countries currently served by Operation Smile International, there are an estimated 1,655,448 children and adolescents (under 15) with orofacial cleft in need of care, whether surgical or nonsurgical. All healthcare professions spoken to emphasised that the majority of the children they treated for cleft palate and/or lip were mentally normal. –  Health-e News