CASE REPORT

Management of gingival hyperplasia of genetic cause (Ullrich muscular dystrophy) using diode laser in pediatric patients: a case report

 Managementul hiperplaziei gingivale de cauză genetică (distrofia musculară Ullrich) folosind laserul diodă la pacientul pediatric: studiu de caz

First published: 03 iunie 2024

Editorial Group: MEDICHUB MEDIA

DOI: 10.26416/Pedi.73.1.2024.9654

Abstract

These days, laser systems and their use in oral surgery and dentistry are developing quickly. Diode lasers operating in continuous or pulsed modes with wavelengths between 810 and 980 nm are considered as a potential soft tissue surgery modality in the oral cavity. An 8-month-old baby girl with Ullrich muscular dystrophy was referred to the pe­dia­tric surgery service of our hospital for therapeutic ma­nage­ment. The child had a global developmental dis­order and growth failure, and the clinical examination of the oral cavity revealed marked, bimaxillary, bilateral gin­gi­val hyperplasia at the canine-molar level. In or­der to improve the weight curve by facilitating age-ap­pro­pri­ate nutrition, we considered the surgical excision of the hyperplastic gingival tissue necessary. Since the he­ma­tolo­­gi­cal parameters were at the lower limit of normal, we decided that the minimally traumatic method was the laser intervention. Ten days after the laser intervention, the gum had a normal appearance both in terms of volume and co­lo­ra­tion, the canines and first temporary molars were fully ex­posed and functional, allowing the feeding of semi-solid and, with caution, even solid foods. Lasers are now more ap­pea­ling in pedodontic applications (in­clu­ding pediatric sur­gery) due to recent advancements in technology, as they can be utilized as an addition to or substitute for con­ven­tio­nal methods. 

Keywords
diode laser, soft tissue, oral surgery, minimally invasive, congenital muscular dystrophy (CMD)

Rezumat

În zilele noastre, sistemele laser cunosc o dezvoltare im­por­tan­tă, iar utilizarea lor în stomatologie în general şi în chirurgia orală în mod particular a câştigat din ce în ce mai mult teren. Laserele cu diodă care funcţionează în module continue sau pul­sa­tile, cu lungimi de undă între 810 şi 980 nm, sunt luate în con­si­de­ra­re ca o modalitate potenţială de intervenţie chi­rur­gi­cală asupra ţesuturilor moi din cavitatea orală. O fetiţă de 8 luni, cu distrofie musculară Ullrich, s-a prezentat în serviciul de chi­rurgie pediatrică al spitalului nostru pentru management te­ra­peutic. Copilul prezenta tulburare globală de dezvoltare şi falimentul creşterii, iar la examenul clinic al cavităţii orale s-a evidenţiat hiperplazie gingivală marcantă, bimaxilară, bi­la­te­rală, la nivel canin-molar. În scopul ameliorării curbei pon­de­ra­le, prin facilitarea alimentaţiei corespunzătoare vâr­stei, am considerat necesară intervenţia chirurgicală de ex­ci­zie a ţesutului gingival hiperplazic. Întrucât parametrii he­ma­to­lo­gici erau la limita inferioară a normalului, am decis că me­to­da mi­nim traumatică este intervenţia cu laser. După 10 zile de la in­ter­ven­ţia chirurgicală cu laser, gingia avea aspect normal, atât din punctul de vedere al volumului, cât şi al coloraţiei, iar caninii şi molarii primi temporari erau complet expuşi şi func­ţio­nali, per­mi­ţând alimentaţia cu alimente de consistenţă se­mi­so­lidă şi, cu pre­cau­ţii, chiar solidă. În concluzie, laserele sunt, la momentul ac­tual, o alternativă „prietenoasă” în prac­ti­ca pedodontică (in­clu­siv în chirurgia pediatrică). Datorită pro­gre­se­lor recente, teh­no­lo­gia laser poate fi utilizată ca adjuvant sau în­lo­cui­tor pentru metodele convenţionale.
 

Introduction

A defined monochromatic beam with a preset wavelength is produced using an active medium in light amplification by stimulated emission of radiation, or LASER(1). This extremely powerful laser beam is a part of the infrared and ultraviolet radiation spectra(1). In the field of dentistry, laser therapy has taken center stage as a reasonable and well-liked substitute for a number of traditional approaches, either alone or in conjunction with these surgical techniques(2). Lasers have been used in therapeutic applications in the medical and allied health professions since 1970. The new era of using carbon dioxide lasers in dentistry to treat oral diseases was frightening in the beginning of the 1980s. Developed in 1987 specifically for dental surgical treatments, the first specialized dental laser was the neodymium yttrium-aluminum-garnet (Nd:YAG) laser(3).

Diode lasers are very effective in surgically managing the related tissues because of the high concentration of chromophores in gingival tissues. A preset requirement for the treatment method, this extremely tissue-specific diode laser is engaged in contact mode to provide enough tactile sensation(4). Due to the diode laser’s characteristics, substantially less anesthesia is needed, and the tissue responds better to the therapy afterward.

Providing pediatric patients with the needed dental care in a serene and kid-friendly setting is the main goal of pediatric dentistry(5). In pediatric dentistry, lasers are used for soft tissue surgery, endodontic therapies, and the diagnosis and prevention of dental caries(5). The most dependable treatment option for the pediatric population is now laser application and modalities, which is a significant alternative to the current standard surgical techniques due to the recent and widely accepted breakthroughs in these fields. Pre-procedurally, the diode laser can also be used to clean the area. It works well in cases of periodontal disease and in laser-assisted periodontal therapy because of its antibacterial impact. It can be used prior to ultrasonic descaling operations because of its antibacterial properties. Since the gingiva is a blood-irrigated tissue, using a diode laser is the best choice when it comes to gingival remodeling.

These treatments are necessary in a variety of circumstances, such as frenectomy, patients with periodontal disease, or the need to remove gums to create way for a molar that is erupting. These are only a few of the many treatments it can be used for; the most crucial thing to keep in mind is that the diode laser promotes speedier healing and greatly lowers bleeding and postoperative pain.

Case presentation

An 18-month-old baby girl with Ullrich muscular dystrophy, genetically confirmed, was referred to the Department of Pedodontics, following the presentation in the pediatric surgery service of our hospital for therapeutic management. The child had a global developmental disorder and growth failure, water and electrolyte disorders, gastrostomy, protein-energy malnutrition, intestinal malabsorption, feeding difficulties, and the clinical examination of the oral cavity revealed marked, bimaxillary, bilateral gingival hyperplasia at the canine-molar level.

According to the mother, in the last 2-3 months, the gingival mucosa at the level of both jaws, in the posterior region, began to swell and, sometimes, even to the touch or with minimal oral hygiene, to bleed as well. The girl became agitated near meals, refused food, which led the mother to conclude that the hypertrophic areas were painful to the touch.

Upon intraoral examination (Figure 1), bimaxillary, bilaterally, in the temporary canine-first molar region, rectangular, symmetrical gingival hyperplasia was evident, with a length of approximately 2.5-3 cm; the gum was pink-reddish in color, firm to the touch, with small whitish dots corresponding to the projection of the cusps of the underlying teeth.

Figure 1. The hyperplastic gingival areas prior to removal
Figure 1. The hyperplastic gingival areas prior to removal

Considering the basic condition of the child, the associated neurological development deficiency explained the insufficient neurological impulse for tooth eruption, which led to the appearance of hypertrophic gums (such an operculum) at the level of subgingivally retained teeth, beyond the physiological age of eruption.

In order to discover the retained teeth and, thus, facilitate the age-appropriate nutrition, contributing to the improvement of the weight curve and, at the same time, obtaining an improved endooral status, we considered the surgical excision of the hyperplastic gingival tissue necessary.

Treatment options

We were able to choose between two different methods of treatment: laser surgery or surgical excision of the lesion using a knife and sutures to halt the bleeding. The decision was made to proceed with laser-assisted treatment due to its many benefits over scalpel-based procedures.

First of all, the laser surgery has a notable ability to disinfect the tissue being treated as well as the surrounding tissue; this lowers the likelihood of postoperative inflammation and, consequently, the requirement for antibiotics. Secondly, faster healing can be achieved by increasing metabolic activity with a laser treatment. Thirdly, there is the benefit of quickly halting postoperative bleeding, which eliminates the need for sutures. Last but not least, a laser therapy helps prevent postoperative pain.

We decided that the minimally traumatic method to expose the child’s retained teeth, taking into account the parents’ concerns and the child’s illness, since the hematological parameters were at the lower limit of normal, was laser surgery, using a diode laser. Written informed agreement from the parents was obtained after the condition’s available treatment options were explained, and a diode laser was used to arrange the teeth’s exposure.

We chose to use the Wiser 3 Diode Laser from Doctor Smile, with 980 nm wavelength in continuous mode, at a power setting of 16 Watt, and the procedure was carried out under general anesthesia (Figure 2). The diode laser was selected, additionally, because it had advantages over other wavelengths for soft-tissue surgery. The energy does not damage or disperse into surrounding tissue, making the procedure quick, easy and minimally invasive.

Figure 2. The Wiser 3 Diode Laser from Doctor Smile
Figure 2. The Wiser 3 Diode Laser from Doctor Smile

The surgery stages

The hyperplastic gum was removed (Figure 3), and then it was sent for histological analysis. The laser’s settings were adjusted to subablative energies of 20 mJ, 0.2 W, and 10 Hz (gentle treatment mode), and a longer, 1.3-mm diameter tip was employed to disperse heat superficially and halt the bleeding, so that coagulation could occur in the affected area. The movements in this respect were slow and repetitive, and the bleeding was checked with a gauze pad to see if it had stopped.

Figure 3. The macroscopic appearance of the excised hyperplastic gum
Figure 3. The macroscopic appearance of the excised hyperplastic gum

The bleeding had stopped within 60 seconds; thus, no coagulating agent was required. In order to reduce the risk of scarring, minimize food residue buildup around the suture, minimize eating obstruction, and lessen subsequent stress to the patient’s mother and the tissue, we made the decision not to suture the area (Figure 4).

Figure 4. Intraoperative view and the appearance of the mucosa and exposed teeth after the excision of hyperplastic gingiva
Figure 4. Intraoperative view and the appearance of the mucosa and exposed teeth after the excision of hyperplastic gingiva

The girl’s mother was instructed to sanitize the wounds with sterile compresses and physiological serum and to keep semi-solid food, avoiding acidic or hot foods for the surgery day, then a follow-up after a week.

Fibrin had covered the lesion region 48 hours after the surgery, and the lesion edges seemed to have shrunk (Figure 5).

Figure 5. The fibrin covering the wounds 48 hours after surgery
Figure 5. The fibrin covering the wounds 48 hours after surgery


A week later, no scarring was visible (Figure 6). The tissue fully recovered. The follow-up was positive, with the teeth erupted in their precise places, with soft, normally colored gums, and the periodontal mucosa had a normal appearance.

Figure 6. The wounds were healed, and the gums were supple and normally colored, one week after surgery
Figure 6. The wounds were healed, and the gums were supple and normally colored, one week after surgery

Discussion

Congenital muscular dystrophy (CMD) is the name given to a diverse range of conditions marked by hypotonia and muscle weakness from birth, with a muscular pathology akin to muscle atrophy. The severity, comorbid symptoms, and prognosis of this complex illness vary(6). Certain genes – such as fukitin, integrin receptor a7, and the extracellular matrix protein merosin (laminin a2)(7) – have already been linked to CMD. There are minimum thirty distinct forms of congenital muscular dystrophy. Each kind differs in its range of symptoms and genetic etiology, but they all share the trait of being present at birth or shortly thereafter, and affecting mostly the muscles involved in movement.

A mutation in one of the collagen VI genes (COL6A1, COL6A2, and COL6A3) results in Ullrich CMD. The body typically produces very little or no collagen VI protein as a result. The primary protein in the body’s connective (supporting) tissue, collagen, keeps muscle cells stable. Babies with Ullrich CMD may move less, and they frequently exhibit hypotonia, or low muscular tone or floppiness. Other typical symptoms include contractures in the elbows, knees and hips, as well as a stiff neck (torticollis). Although the precise mechanisms by which these genetic alterations cause the disease are not fully understood, it is believed that, in addition to weakening the structure that supports the muscle cells, the changes may also affect the parts of the mitochondria that supply energy and make the muscle cells more susceptible to cell death(8).

Sometimes, the first symptoms appear after a few months, when babies exhibit poor head control or a delayed learning curve for new tasks like sitting on their own(8). The muscles involved in breathing are impacted during night, which is why children frequently experience breathing difficulties even while their motor performance is mostly steady or only gradually improving. Chest infections may also increase in frequency, as the respiratory muscles deteriorate. Ullrich CMD patients may have “contractures” from birth, which are characterized by tightened muscular tendons that limit the range of motion in the limbs and joints. The majority of kids with Ullrich congenital muscular dystrophy also experience scoliosis, or a curvature of the spine(9).

After the first few years, weight loss (also known as failure to thrive) is another common issue that calls for nutritional supplements. Since children’s skin naturally contains collagen VI, scars may heal more slowly in them or may become thicker and more raised (keloid development).

Against this background of the general hypodevelopment specific to Ullrich syndrome, the neurological deficiency outlines the premises of some secondary clinical pictures, among which the dental eruption disorders can also be found (chronologically, sequentially or of the added inflammatory phenomena).

Gingival hyperplasia is one of the complications that can disturb the process of tooth eruption, being found predominantly in the temporary dentition in children with damage or delay in neurological development (as in the case of those with Ullrich muscular dystrophy), in which the deficient nerve transmission leads to insufficient impulses of tooth eruption, teeth remaining intraosseous or subgingivally retained, with reactive inflammation of the overlying gum. On this ground, both nutrition and oral hygiene are disturbed, creating a vicious circle through which gingival inflammation is self-perpetuating, with the secondary appearance of bleeding and, frequently, an overabundance of bacterial flora causing more or less “noisy” infectious phenomena.

In conditions where there is already a developmental deficiency, with secondary malnutrition (as is the case presented), the clinical picture becomes even more complicated, creating difficulties in the therapeutic management. Therefore, it is obvious the need to approach the lesions in the oral cavity as quickly as possible, efficiently and with minimal trauma.

If until not long ago, these desired goals were difficult to achieve, fortunately, at the present time, advancing technology allows us to approach these cases (and not only) from a modern perspective, more precisely through the use of laser therapy; in our specific case, it was the laser surgery that allowed us to successfully manage the case, leading to the overall improvement of the patient’s status.

The diagnosis and prevention of caries, restorations, pit and fissure sealants, soft-tissue laser ablation, primary tooth endodontics, traumatology, disinfection and decontamination, and the exposing of unerupted teeth are just a few of the numerous uses of laser technology in pediatric dentistry(10). By acting on cell receptors, low-level laser therapy promotes healing and the subsequent restoration of tissues. It also lessens postoperative pain and inflammation, which minimizes the requirement for any local anesthetic during the treatment(11).

The recommended wavelength range of 810-1064 nm is safe for oral surgical procedures, since hard teeth tissue absorbs it poorly(12). Diode lasers are utilized in pulsed mode for periodontal, endodontic and frenectomy operations, and in continuous wave mode for surgical procedures. Tissue injury results from continuous wave mode ablation of the tissue surface; moving the laser beam quickly reduces the heat buildup that results(12). The dental laser’s fiber helps focus the laser beam on the intended area.

When a tooth emerges into the oral cavity at a time that significantly differs from the typical pattern determined by the person’s sex and history, tooth eruption is said to be a delayed process(13). In their research, Roberts-Harry et al. verified that employing lasers to expose unerupted teeth improved patient and dentist acceptance(14).

In order to achieve sufficient hemostasis and surface carbonization of the lesion, the laser fiber tip is swept across the surgical surface. As a result, sutures are not advised after laser surgeries; instead, the surgical site is allowed to heal naturally. Numerous additional sources of vitamin E, sufficient lipids, minerals, cyanoacrylate etc. speed up the healing process of wounds. High in antioxidants, vitamin E improves enzyme activity, boosts brain function, and promotes the faster healing of surgical areas(15).

Conclusions

The diode is the most common type of laser, with the widest range of uses. The diode laser was developed in the early 1960s by Robert N. Hall, obtaining in 1966 a US patent for the stimulated emission semiconductor device.

Great progress has been made in the dental field, the dimensions of diode lasers being smaller and smaller, and the interface much friendlier. These features help in performing soft tissue procedures where good hemostasis is required.

Diode lasers have many characteristics, being clinically versatile. The modalities of therapy include soft tissue surgery, bleaching, and pain therapy.

Different dental conditions, such as gingival (gingivitis, periodontitis) or caries, can be treated with the help of the laser. Medical procedures are much easier to be accepted by the patients, who are delighted with the immediate results and the increased comfort they enjoy during pain-free treatments.

There are multiple benefits of diode laser, such as:

it does not cause pain

it reduces gum swelling and bleeding

it does not cause any vibration or noise that creates discomfort

it leads to quick recovery, and the fear of going to the dentist disappears

the accuracy of the procedure is very high, the interventions being minimally invasive

the risk of infection is reduced to a minimum, because the laser sterilizes the place it acts on during the intervention.

These allow us to conclude that, especially for the pediatric patients, the use of a high-power diode laser can be a good substitute for traditional surgical methods, offering improved postoperative outcomes.   

 

Corresponding author: Diana-Monica Preda E-mail: diana_monica_preda@yahoo.com

Conflict of interest: none declared.

Financial support: none declared.

This work is permanently accessible online free of charge and published under the CC-BY licence.

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