Authors: Gianluca Nicolò Piras; Luca Tomassini; Edoardo Bottoni; Cira di Gioia; Costantino Ciallella · Research

Can Hair-Eating Lead to Death in Children?

A rare case of fatal Rapunzel syndrome in a young child highlights the dangers of hair-eating behavior and challenges in diagnosis.

Source: Piras, G. N., Tomassini, L., Bottoni, E., di Gioia, C., & Ciallella, C. (2023). An atypical death from Rapunzel syndrome: a case report. Forensic Science, Medicine and Pathology, 19, 207-214. https://doi.org/10.1007/s12024-023-00588-4

What you need to know

  • Rapunzel syndrome is a rare and potentially fatal condition caused by eating hair, which can form large masses in the stomach and intestines
  • The condition can be difficult to diagnose, as symptoms are often vague or intermittent
  • Even large hair masses may not cause obvious symptoms until they lead to sudden, severe complications
  • Parents and doctors should be aware of hair-eating behavior in children and take it seriously, as early intervention is key

A tragic case reveals hidden dangers

In a troubling case report, forensic experts describe the sudden death of a 4-year-old girl due to an extremely rare condition called Rapunzel syndrome. This syndrome occurs when a person repeatedly eats their own hair, leading to the formation of large hair masses called trichobezoars in the stomach and intestines.

The case highlights how this condition can go undetected, even as it becomes life-threatening. It also underscores the need for greater awareness among parents and medical professionals about the risks of hair-eating behavior in children.

What is Rapunzel syndrome?

Rapunzel syndrome is named after the fairy tale character with extremely long hair. It develops as a complication of two psychiatric disorders:

  1. Trichotillomania - A compulsive urge to pull out one’s own hair
  2. Trichophagia - The habit of eating the pulled-out hair

Over time, the ingested hair can accumulate in the stomach, forming a large, matted mass called a trichobezoar. In Rapunzel syndrome, this mass grows so large that it extends into the small intestine, like Rapunzel’s long braid.

While uncommon overall, Rapunzel syndrome occurs most often in young women and girls under age 20. Risk factors may include other mental health conditions like anxiety or depression, as well as developmental delays or difficult family circumstances.

A puzzling medical mystery

In the case described, a 4-year-old girl was brought to the hospital with vomiting and abdominal pain that had lasted for two days. Her mother reported the child was having trouble eating and drinking.

Doctors examined the girl but found no obvious cause for her symptoms. Blood tests showed an elevated white blood cell count, suggesting inflammation or infection. After 5 days of treatment for dehydration and metabolic imbalances, her condition seemed to improve and she was sent home.

A psychologist who evaluated the child during her hospital stay noted an unusually close relationship between mother and daughter. The mother appeared very anxious, and the child seemed whiny when her mother was present but more responsive when speaking to the psychologist alone. Further psychological assessment was recommended but not completed.

Over the next two months, the girl’s eating and drinking troubles decreased, though she still experienced occasional nausea. Her parents did not seek further medical care, as the symptoms seemed mild.

Then, tragedy struck. Two months after her hospital stay, the child ate some pasta, vomited, and suddenly lost consciousness. Despite emergency medical efforts, she died within minutes.

Startling autopsy findings

When forensic experts performed an autopsy, they were shocked to discover the true cause of the child’s death: massive hair masses blocking her digestive tract.

The largest mass, weighing over 300 grams (about 2/3 of a pound), was found in the stomach. It measured 15 x 11 x 5 centimeters - about the size of a large potato. Two smaller but still sizable masses were found in the small intestine.

These masses, called trichobezoars, were made up of tightly packed hair mixed with partially digested food. The one in the stomach had a core of undigested vegetable matter surrounded by a layer of hair.

How did this happen?

The forensic team concluded that the child had been eating her own hair for some time, allowing these large masses to form. The parents had noticed their daughter sometimes ate her hair and responded by cutting it shorter more often. However, they did not realize the seriousness of this behavior or seek help for it.

It appears the trichobezoars had been present for weeks or months, slowly growing larger. They likely caused the girl’s earlier symptoms of nausea, vomiting, and trouble eating. However, because these symptoms were intermittent, their true cause went undetected.

On the day she died, the mass in her stomach seems to have shifted, suddenly blocking her digestive tract completely. This led to rapid, severe complications including:

  • Obstruction of her stomach and intestines
  • Build-up of pressure in her abdomen
  • Impaired blood flow return to her heart
  • Severe electrolyte and fluid imbalances
  • Aspiration of vomit into her lungs

This cascade of issues quickly became fatal, before the underlying problem could be identified or treated.

Challenges in diagnosis

This case illustrates how difficult Rapunzel syndrome can be to diagnose, especially in young children. Some key challenges include:

Vague, inconsistent symptoms: The blockages caused by trichobezoars can lead to a wide range of digestive symptoms. These may come and go, making it hard to pinpoint the cause.

Lack of specific signs: Unlike many digestive conditions, Rapunzel syndrome doesn’t typically cause obvious physical signs a doctor would notice during an exam.

Limited communication from young patients: Small children may have trouble describing their symptoms clearly. They also may not tell adults about hair-eating behavior.

Lack of awareness: Many doctors are not familiar with this rare condition, making them less likely to consider it as a possibility.

Need for imaging: Diagnosing Rapunzel syndrome usually requires CT scans or other imaging tests to visualize the hair masses. These may not be done if symptoms seem mild.

In this case, no imaging tests were performed during the child’s hospital stay. If they had been, the trichobezoars likely would have been discovered and removed before they became life-threatening.

Recognizing the risk

While Rapunzel syndrome is quite rare, this case shows how dangerous it can potentially be. Parents, caregivers, and doctors should be aware of warning signs that may indicate a child is eating their hair:

  • Noticeable hair loss or thinning patches
  • Frequently playing with, pulling, or chewing on hair
  • Visible hair in the child’s mouth or stool
  • Chronic bad breath or unusual taste complaints
  • Recurrent, unexplained digestive symptoms

Hair-eating behavior in children always warrants medical and psychological evaluation. Early intervention can prevent the formation of large, dangerous trichobezoars.

Treatment and prevention

When caught early, Rapunzel syndrome can often be treated by removing the hair masses, either surgically or sometimes using specialized endoscopic techniques. However, addressing the underlying behavior is crucial to prevent recurrence.

Treatment typically involves:

  • Cognitive behavioral therapy to address hair-pulling and eating behaviors
  • Family therapy to identify and address any contributing family dynamics
  • Evaluation for other mental health conditions that may be involved
  • In some cases, medication to help control urges or treat anxiety/depression

Parents can help by:

  • Watching for signs of hair-pulling or eating
  • Seeking prompt medical care for unexplained digestive symptoms
  • Following through with recommended psychological care
  • Providing positive support for the child’s mental health

Conclusions

  • Rapunzel syndrome, while rare, can have severe and even fatal consequences if left untreated
  • The condition may not cause obvious symptoms until it becomes an emergency
  • Any hair-eating behavior in children should be taken seriously and evaluated medically
  • Imaging tests are often needed to diagnose trichobezoars in the digestive tract
  • Early intervention and psychological treatment are key to addressing the underlying causes
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