Shall We Score? The SHALL-WE Score for Optimizing Sclerotherapy in Superficial Lymphatic Malformations
This study introduces the SHALL-WE score, a clinical tool based on disease severity, histology, and location, to determine the optimal number of sclerotherapy sessions for superficial lymphatic malformations and guide timely treatment escalation when success rates decline.
Original paper licensed under CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). This is an AI-generated explanation of the paper below. It is not written or endorsed by the authors. For technical accuracy, refer to the original paper. Read full disclaimer
Imagine a child born with a soft, spongy lump under the skin, a harmless but stubborn collection of fluid-filled sacs that never quite go away on their own. These are lymphatic malformations, a type of birthmark where the body's drainage system fails to form correctly. While they are not usually life-threatening, they can grow, cause pain, or disfigure a child's face or body. For decades, doctors have had a few ways to handle them: they can cut them out with a scalpel, use powerful drugs to shrink them, or inject a special chemical to irritate the sacs and make them collapse. The chemical injection, known as sclerotherapy, is often the first choice because it is gentle and avoids the risks of surgery. However, a difficult question has long haunted parents and physicians alike: how many times should you try the injection before admitting it is not working? If a doctor keeps injecting a stubborn lump that refuses to shrink, they might be wasting precious time, or worse, causing scarring that makes future surgery much harder. There has been no clear rulebook to say when to stop and switch strategies.
A team of researchers from across Japan set out to solve this uncertainty by looking at the real-world experiences of hundreds of children. They gathered data from 709 patients with these superficial lumps, analyzing every detail of their cases, from the size and shape of the malformation to the number of times it was treated. Their goal was to find a pattern that could tell a doctor exactly when to stop the injections and move on to a different approach. They discovered that the answer depends entirely on three specific features of the lump: where it is located on the body, what it looks like under a microscope, and how severe the condition was when it first appeared. By combining these three factors, they created a simple scoring system called the SHALL-WE score. This tool assigns points based on whether the lump is on the head or neck, whether it is made of solid tissue rather than clear fluid-filled cysts, and whether the disease was already severe at the start.
The researchers found that these three factors act as a reliable warning system. For a child with a low score—meaning the lump is on the body rather than the face, is fluid-filled, and is mild—the injections work well, and doctors can safely try the treatment up to two times. However, as the score rises, the chances of success drop sharply. If a child has a score of one, the injections are likely to stop working after just one attempt. For those with the highest scores, where the lump is on the face, made of solid tissue, and is severe, the injections rarely succeed at all, regardless of how many times they are tried. In fact, for these difficult cases, the success rate of the injections stays low no matter how many sessions are performed, suggesting that continuing with this method is futile.
The study confirms that sticking to these limits improves the outcome for patients. Children whose doctors followed the new scoring system and stopped the injections at the right time had a success rate of nearly 72 percent. In contrast, those who continued with injections beyond the recommended limit saw their success rate fall to just over 51 percent. This difference is significant because it means that by knowing when to stop, doctors can avoid the frustration of failed treatments and the physical toll of unnecessary procedures. The researchers noted that for the most difficult cases, where the score is high, the best path forward is to escalate treatment early, perhaps moving to surgery or medication sooner rather than later. This approach prevents the tissue from becoming scarred and inflamed from repeated injections, which can make the eventual surgery much more difficult and risky.
While this new scoring system offers a clear guide, the authors are careful to note that it is a tool to support a doctor's judgment, not a replacement for it. The data came from a survey conducted before a new drug called sirolimus became widely available for these conditions in Japan, so the current landscape of treatment options is even broader than what the study captured. The researchers also emphasize that their findings are based on a specific group of patients with superficial lumps and may not apply to deeper, more complex cases. Nevertheless, the SHALL-WE score provides a much-needed compass for navigating a complex medical decision. It transforms a guesswork-heavy process into a structured plan, ensuring that children receive the right treatment at the right time, and that families do not have to wait years to find a solution that actually works.
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