Fusion

I had stopped by the operating theatre to try and schedule a case. When I was there, the anesthetist asked me to see a patient he had in the waiting area.

The patient was a 9 month old baby girl, the daughter of a clinical officer at TDH. Both parents were there looking concerned. The problem, they reported, was that the baby's genitalia were abnormal.

They removed the baby's clothes so that I could look, and immediately I saw the problem. There was no vaginal opening. I could see the urethra (where the urine comes out) and the rectum was normal, but the labia were sealed shut, almost as if she had had a female circumcision.

It was unlikely. There is only 1 tribe in Uganda that does female circumcision, and although they are not far from Tororo, they would not do it in a child this young. I ask the parents, but they are not from that tribe, and they did not have any circumcision done to the child. They don't know how long the labia have been that way.

I am perplexed. I was expecting something more like ambiguous genitalia. But this is clearly a girl - there is no penis. Maybe it isn't a girl? Should I do an ultrasound and confirm the presence of a uterus and ovaries? Would I even be able to see them at this age? I can't think of anything else to do.

It could also be an imperforate hymen or a vaginal septum. But this appears to be the labia. An imperforate hymen would be inside the labia, and normal labia would still be visible. A vaginal septum would be even more internal.

Well, I don't know what it is, but would I need to do anything about it? The girl isn't going to menstruate for another 10-13 years or so, and operating on a small infant is more difficult than operating on a child or adolescent. The urethra appeared normal, so she can urinate without a problem.

The parents do report that the girl cries every time she urinates for the last 2 months or so. It's possible that this closure is causing some partial blockage of the uretral meatus, preventing the urine from exiting efficiently and allowing infection to build. If that is the case, she might then need surgical correction.

I decide to do some research and have them come back. I tell them to get a urinalysis to look for infection, and to return to me next Wednesday when I normally hold my clinic. They are grateful, and very worried that their little girl might need surgery - or worse, might have a permanent abnormality.

In the meantime, I call a trusted obstetrician in Kampala, Dr. O. I have enormous respect for this man. He is well-known in the field of global Obstetrics & Gynecology, and has made great efforts to reduce maternal mortality in Uganda. He is one of the investigators on my research projects. He is also an extraordinary clinician, a firm but patient teacher and unfailingly professional and polite, even when juggling multiple acute responsibilities. I have called him with questions many times, and he never fails to help me. When he comes to visit the study site, he is able to briefly tour the faciluty and then present a list of ideas for improvement and progress that are always perfectly on point.

When I reach him, I tell him about the problem.

"Oh yes," he says, "I have seen this before. The parents are not washing the baby properly. They are failing to wash the labia, and the baby develops a mild infection and inflamation of the skin, and the labia fuse together."

I am stunned. "Really? That's it? You have seen this before?"

"I have seen this many times. I have even had babies referred to me by urologists."

"What should I do?" I ask.

"It is very easy to manage. You just take a piece of gauze, and you pry open the labia manually. They will come open easily."

I am skeptical. "The labia looked really fused together. Do you really think it will come open, just like that? I am worried about hurting the child."

"You will be surprised, he says. "You will not hurt her. She will cry. You will have to have someone hold her down, and maybe have the parents wait outside the room because they will become upset. But you will not be hurting her very much, and she will stop crying when you are finished. I have done this many times, it is always fine."

I trust this man, but I am pretty nervous about this. "Ok, I will try it. Just in case, if I can't open it, can I take a photo and send it to you?"

"You can send me a photo if you need," he says. He sounds like he knows I won't need to.

The parents return and find me in the clinic. S, another clinic doctor who shares my office, is present. The little girl is as cute as ever. The parents report that the urine result was negative, and the symptoms have gone away.

I tell them what Dr. O has told me. I explain what I am going to do. I ask if they want to be in the room when I do it to hold her, or if they want to wait outside.

The husband turns to his wife. "Do you want to hold her?"

She looks unperturbed. "I can hold her."

"Ok," I say, "women are strong. They can push, and they can hold the crying baby. Men, they cry and they faint." We all laugh. Everyone here likes African-women-are-strong jokes.

I ask S to help me with this, just in case I need a hand. We take the baby into the other room. The mother undresses her. She leaves on the string of beads tied around the baby's waist. Many girls and women wear these beads. They have some kind of cultural significance that I'm not sure about, but they are very pretty. I almost want some.

We lie the baby on the bed and she is already crying. The mother bravely holds her legs. We look at the perineum. The labia look totally fused. There is a line of fusion, but it really doesn't look like it is going to come apart. I am nervous.

Gently, I take a piece of cotton and use light pressure to try to spread the labia. The baby is crying but the mother is holding her well. As I apply pressure, a small depression appears along the fusion line. S is surprised, as am I. He helps me to continue applying gentle pressure. Magically, the labia appear. There is a normal vaginal introitus. The labia have a tiny bit of blood on either side, but otherwise are fine. I can't believe it.

It takes me a few seconds of staring to realize that it actually worked. We instruct the mother on proper cleaning of the labia so that it does not happen again.

The baby is crying, but as soon as the mother picks her up, she stops. She even looks content. Amazing.

The mother is very grateful. I reinforce the cleaning procedure, and she leaves.

S and I are still quite surprised that it actually worked. Now I am even more in awe of Dr. O. Maybe this is something that pediatricians see all the time - I don't know - but I have never seen or even heard of it. S remarks to me that he knows of a 13-year-old girl who has to go for surgery soon to have the same thing fixed, but he didn't realize that a simple hygiene lapse was the cause of it. If the labia had stayed fused for years, this baby would later have required surgery because the fusion would have been too severe for manual separation.

Ten minutes later, my phone rings. Her husband is calling.

"Doctor, I am very grateful! I had to go back to work, so I have not seen her, but my wife has passed the message that she is fine and you have cured her. Thank you so much! I am very relieved and very grateful."