A Wig in a Child’s Stomach

Little  Iresha  is  admitted  to  my  ward  with  abdominal  pain

On 8^th June 2008 a 14-year old girl, Iresha, from a remote District in Sri Lanka came with her parents and was admitted to my ward at Sri Jayewardenapura General Hospital, Nugegoda (SJGH).  Little did everyone realise that “little” Iresha would attract much publicity sooner or later. Two weeks later Iresha’s story was published in many English and Sinhala Newspapers in Sri Lanka without her identity being divulged.

Suffered for three months

Iresha had been suffering for three months prior to admission and had been treated by many doctors and at various hospitals in her locality. She was complaining of pain in the upper part of her belly together with vomiting. She had also lost her appetite and could not retain any food. Naturally, she was losing weight. She had not complained of anything else and the parents too had not noticed something very important which would have clinched the diagnosis. That is the “TOP SECRET”.

Diagnosed to have “Gastric”

In Sri Lanka patients with such abdominal symptoms are usually diagnosed to have a condition called “gastric” which is not reported in text books of Medicine or Surgery. But this is a common “diagnosis”. The poor and ignorant patients are given some form of medication, usually antacids for such symptoms.  The patients take the medicines with much hope, but without much improvement as the diagnosis for the abdominal pain has not been made. Thus the patients go from one doctor to another in search of someone who can cure them. This is a syndrome referred to as “Doctor Shopping”.

Iresha too was diagnosed to have “gastric” and her parents went “doctor shopping” in search of a Doctor who could diagnose their little daughter’s disease, give her appropriate treatment and cure her. They had gone  first to the General Practitioner, then to the District Hospital and finally to the General Hospital (GH) in that Province. An Ultrasound Scan and a Computer Tomography (CT) of her abdomen had also been carried out at the GH.  However the disease could not be identified. It is only at this stage that they were advised to seek treatment in my ward at SJGH by a young doctor working in that area who had his training in my unit.

Admitted to SJGH

Once again Iresha’s symptoms were

• Abdominal pain
• Vomiting
• Loss of appetite
• Loss of weight

On examination we found that her body was wasted. She was pale and appeared ill. The examination of her abdomen did not reveal anything unusual. We too did not look for that all important “sign” since we had not seen such a patient before and did not expect such a rare diagnosis.

Her dehydration and anaemia was corrected first. As I suspected that she had a problem in her stomach I carried out an Endoscopic examination of her stomach. I could not pass the endoscope into the stomach – there was a “block”, but I also noticed something like strands of hair in the stomach. The next step was to perform surgery and that was to open her abdomen (laparotomy).

Diagnosis clinched at operation

We were all aware that we were in for a surprise but did not know what it was going to be. My junior doctors were ready with their cameras. As I opened the abdomen, I found that the stomach was enlarged, full of something unusual and there was no doubt the stomach was obstructed and was the cause of all her symptoms. Next I opened the stomach with a scalpel and a Diathermy needle (gastrostomy). To my surprise I found the stomach full of hair entangled into a ball taking the shape of the stomach and also extending into the next part of the gastrointestinal tract, the duodenum and Jejunum in the form of a tail. I removed the entire specimen in one piece while my Assistant Dr Sanduni Sirimanne was taking photographs. Everyone was excited and others in the operation theatre complex too rushed in to see this rare operation. I could not be too excited as I had to complete the operation. After posing for a few photographs carrying the specimen, I closed the opening in the stomach and then the cut in the abdominal wall. That was the end of the operation. She recovered dramatically except for a minor infection of her wound. 

Photographs taken at Operation

THE  EXCITEMENT WAS ALL CREATED BY A BALL OF  HAIR  IN  THE  STOMACH AND THAT  WAS  CAUSING  ALL  THE PROBLEMS  FOR  THE  14  YEAR OLD IRESHA

A RARE CASE INDEED

It was a rare case indeed and now we had to find out more about this problem. I had never heard about patients having hair balls in the stomach and I did not expect my assistants to know about such a condition either.

Research – Literature Survey

Now we had to learn about “hair balls” in the stomach. Going through the literature on this subject I came across a whole lot of new terms and syndromes that had been identified since ancient times regarding this condition. I will introduce those as bullet points.

• Bezoar : a small hard collection which may form in the stomach

• Trichobezoar : a ball formed by hair in the stomach. Described by Baudamant in 1779

• Trichophagia : a desire to eat one’s own hair

• Trichotillomania: is also referred to as “hair pulling disorder”. It is classified under Obsessive- Compulsive disorders and involves recurrent, irresistible urge to pull hair from the scalp, eye brow, eye lid and other areas.  It was described byHallopeau in 1889.       

• Rapunzel Syndrome : a condition characterised by a large hair ball in the stomach extending to the small intestine or more rarely into the large bowel in the form of a tail. This was described by Vaughn in 1968. It is named after a charming fairy tale written in 1812 by Brothers Grimm about a 12 year old Princess named Rapunzel who was shut in a tower with neither stairs nor doors. The young Princess with long tresses lowered her hair to the ground from high in the prison tower to permit her young Prince to climb up to her window and rescue her.

The Top Secret

Now I can reveal what the top secret in diagnosing this rare condition. That was to ask the patient whether she was eating her own hair. We could not ask this question from the patient straight away, but asked her parents. They denied any knowledge of this. On recovery we asked the question from Iresha and she confirmed it. When we examined her scalp carefully, there were patches where the hair was very scanty.

More about Rapunzel Syndrome

The symptoms are abdominal pain, vomiting, loss of appetite and weight loss. When the symptoms develop, the hair ball is usually quite large. The patients are anaemic. The syndrome is commonly seen in young girls 12-14 years of age (90%). Fatal complications could also occur and these include bleeding intestinal obstruction, ulceration and perforation of the bowel. 

How is the Hair Ball formed?

When the patient keeps eating hair it accumulates in the stomach. The hair is not digested and its slippery nature prevents the hair from being propelled out of the stomach by its contractions. Thus the hair mixes with the food particles and is churned into a ball taking the shape of the stomach and once it is full the excess protrudes through the outlet into the next part of the bowel as a tail. The fermentation of fats gives the Bezoar a putrid smell. The acid contents of the stomach denature the protein and give the Bezoar its black colour.

Dimensions of the Hair ball

The hair ball removed from the stomach of Iresha weighed 750gm and its length was 75 cm.

Rare Syndrome

A review of the literature on Rappunzel Syndrome revealed that only 27 cases had been reported in the world up to 2008. Thus this is the 28^th case and the first case of Rapunzel Syndrome in Sri Lanka

What else in the care of the patient

Since this syndrome is associated with Obsessive-Compulsive Disorder, that aspect too needed attention. Iresha was referred to a Psychiatrist on recovery and the parents were asked to keep a close watch on her. With the advise of the Psychiatrist she had no further problems of eating hair and recovered fully. Now she is 26 years of age, married with one child and has put on a tremendous amount of weight. Iresha now leads a normal life just as anyone else.

Much publicity

The word went round and Iresha attracted much public attention with many journalists visiting the ward to get more information of the first case of Rapunzel Syndrome in Sri Lanka. However it was our duty to maintain the privacy of this young girl and we gave the necessary information for publication in the news print.

HISTORY  CREATED  WITH  THE  REPORTING OF THE  FIRST  CASE  OF  RAPUNZEL  SYNDROME  IN  SRI LANKA

Dear Reader, Another story of a midnight crash and a huge gash on his neck Silva never expected to live another day: ‘SILVA BACK AT HIS “WHEEL”’