HAIR SCARE

HAIR SCARE

A Wig in a Child’s Stomach

Little  Iresha  is  admitted  to  my  ward  with  abdominal  pain

On 8th 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.

BUT  THIS  IS  A  QUESTION  I  HAVE  NEVER  ASKED  FROM A  PATIENT  ADMITTED  WITH  ABDOMINAL PAIN.   AFTER THIS OPERATION TOO THIS QUESTION HAS NEVER BEEN ASKED BY ME WHEN A PATIENT PRESENTS TO THE WARD WITH ABDOMINAL PAIN. IF  A  DOCTOR  ASKS   THIS  QUESTION  FROM  A  PATIENT  WITH  ABDOMINAL  PAIN  THE  PATIENT  MIGHT   THINK  THAT  THE   DOCTOR  IS  SILLY!  THIS IS A ONE OFF CASE OR ONCE IN A LIFE TIME FINDING AND IT WAS SO FOR ME AS WELL.

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 28th 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

Twelve years later at Iresha’s home in the Uva Province, Sri Lanka
                           29th September 2020


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”’


20 thoughts on “HAIR SCARE

  1. Doctor Gamini,
    I can remember this story as I even saw the image of it. Poor girl what a mess it would have been for her to carry such an amount of hair in her stomach like this… She must be lucky, that she somehow was directed to you in a crucial time, than risking her life, when it had been impossible to save her. God bless!

  2. This definitely is the rarest case ever ! Scary to think of such kind of diseases are there in humans… She is blessed to have got the right surgeon… Hope she didn’t go through this problem again in her life.

  3. Sir, I can remember it as it was happen today. Bcos I was the assistant nurse biside u. Nice to remind thosedays.
    Good luck.

  4. Sir I read this article curiously till I to know the responsible secret for this girl’s symptoms.While on reading this article though my mind suggested different possibilities I didn’t think these symptoms due to a space occupying mass in stomach as she had under gone to previously USS and CT abdomen without positive findings.Sir your invaluable decision for Endoscopic examination lead to reveled hidden secret behind this girl’s symptoms and management.Sir my immense thanks to you for sharing your invaluable past experiences to enhance my knowledge.

    1. Dear Ramyalal
      Thank you for reading the story titled – Hair Scare. I appreciate your comment as well. The lesson is that we must decide on our investigations depending on the symptoms. This is what I used to tell students and H.O.’s – Relevant investigations based on History & Examination. My stories are to Educate and Inspire and I am making use of my experiences to do this for the benefit of the younger generation. All the other channels to educate the juniors are not available to me! Please share my stories with all those who are interested
      Best wishes
      Gamini Goonetilleke

  5. Dear Sir,
    What a wonderful diagnosis. It is an achievement from a eminent Surgeon of your caliber.
    Your retirement from SJGH is a great loss to the hospital and patients who are yearning for a dedicated consultant surgeon of your stature.

  6. Many thanks, Gamini. Even though I am miles away in my own discipline, I never miss reading your tales – it is so inspiring. God Bless. Godwin

  7. Iresha is specially blessed to have had a surgeon of your calibre who is so much dedicated to the job.

  8. Dear Sir,
    I have started sharing your dedicated surgical history with many. it is my pleasure to do so as you present many rare and interesting cases. I am yet to find the contacts of Dr. Doctor and Dr. Nilesh Doctor (J&J India.)

  9. Thank you for highlighting this extremely rare case. The surgeon should be commended for the diagnosis and treatment.

  10. Now this is something I read very slowly and studied very carefully. As soon asI saw this title I was curious to know how this happened and why and how did the surgeon detect this. By the time I finished reading not only did I get answers for these questions but also I started wondering what kind of doctor this is?
    Already many had examined her but failed to diagnose it and it was never reported in the country before. Even with all this thrilling experience the surgeon has done a great study on the matter and stated some useful remedies and precautions. Truly this is skill, enthusiasm and charity at its best. Hats off to you Dr. Gamini.
    AMS

  11. Dear Avishka
    Thank you for your comment. Glad that you had studied the case carefully while reading. These are once in a life time cases and must not only be documented but others must be made aware of these problems.
    Surgery is an exciting job. Healing is not a job but a vocation. I am happy that I was able to heal this poor girl from a remote area in Sri Lanka. I am in touch with her many years after the operation. She is 26 years old now, happily married with one kid and not eating hair anymore!
    Keep on reading. Many more exciting cases on the way and many more stories to educate and inspire.
    Best wishes
    Gamini Goonetilleke

  12. Impressed!
    When I visited your ward you showed me some bundle in a bottle and asked me to guess what it was . I just couldn’t. Then you said it was hair. Today I have seen the photo in your Life experiences sharing. Thanks for all the sharing. God bless.

  13. Yes Sir,
    I remember this girl when she was in the ward but, can’t even recognize by seeing her picture.
    Every day we had new experiences with your surgeries.
    But I wonder whether she still taking meds for her psychological issues…
    Anyway, she is alive bcz she was on good surgeon’s hands.

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