Management of patient with acrometageria for routine dental treatment: A case report

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The population of special needs patients in dental offices is growing. Therefore, the demand for well-trained, educated practitioners must increase to fit the need. Conditions such as intellectual developmental disorder, Down syn- drome, and autism spectrum disorder are more readily encountered in dental settings. However, it is equally appropri- ate to identify management techniques for patients with less common condi- tions. A case is reported in which a 38-year-old Caucasian male with a his- tory significant for acrometageria and associated signs of Mallampati Class IV, micrognathia, decreased mouth opening, decreased thyromental distance, and decreased cervical range of motion pre- sented for routine dental treatment under intravenous sedation. Providers should recognize appropriate manage- ment techniques to safely and effectively care for a wide patient demographic.

A B S T R A C T Management of patient with

acrometageria for routine dental treatment: A case report

Andrew S. Young, DDS;1* Matthew R. Cooke, DDS, MD, MPH;2 Lynne M. Taiclet, DMD3

1Resident in Dental Anesthesiology, School of Dental Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania; 2Assistant Professor, Department of Dental Anesthesiology and Pediatric Dentistry, School of Dental Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania; 3Director and Assistant Professor, Center for Patients with Special Needs, School of Dental Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania.

*Corresponding author e-mail: any29@pitt.edu

Spec Care Dentist 36(1): 32-38, 2016

Acrometageria was first described by Greally et al. in 1992 as a manifestation of two premature aging syndromes—acr- ogeria and metageria.3 While acrogeria was described initially by Gottron (1940) who reported on siblings with skin changes characteristic of aging on their hands and feet, metageria was described by Gilkes et al. (1974) as a phenotype differing sufficiently to warrant a sepa- rate classification.4,5 In 1985, Kaufman et al. explored the occurrence of acrogeria and metageria in the same family leading authors to suggest that the two condi- tions exist as variants of one another.6 These observations have led to further suggestions proposing the existence of a spectrum of disorders including both acrogeria and metageria referred to by an encompassing term—acrometageria.3 Due to their rarity, the aforementioned premature aging syndromes are largely defined by their clinical manifestations.

A comparison of acrogeria, metageria,

and acrometageria are outlined in Table 1.

In dentistry for special needs patients, practitioners encounter various conditions. Understanding how to manage patients with unique conditions is important for providing successful comprehensive dental care. By recogniz- ing management options, both behavioral and pharmacological, dental providers may address barriers to treatment.

Case repor t

Medical history

A 38-year-old Caucasian male (162 cm, 51 kg) presented at the University of Pittsburgh School of Dental Medicine for comprehensive dental treatment in the Center for Patients with Special Needs.

The patient had a medical history signifi- cant for acrometageria, scoliosis, cataracts, Raynaud’s syndrome, and intellectual

Introduction

Acrometageria is a progeroid (premature aging) syndrome characterized by a short, thin stature, beaked nose, atrophy on extremities and little subcutaneous fat.1–3 Mode of inheritance is yet to be determined, although both acrogeria and metageria have been identified as autosomal recessive.

KEY WORDS: special needs management, acrometageria, behavior guidance, sedation, dental treatment, general anesthesia

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disability with associated signs of hyper- flexible joints, thin stature, beaked nose, atrophy on extremities, and little subcuta- neous fat (Table 2). Further physical examination and airway assessment revealed Mallampati Class IV, microg- nathia, decreased maximum mouth opening, decreased thyromental distance, and decreased cervical range of motion;

these predictors of difficult airway poten- tially complicate sedation and/or general anesthetic management (Figure 1).

The patient’s father mentioned previ- ous surgical history for hyperflexible joints of the patient’s hands in 1993. A traumatic motor vehicle accident (MVA) resulted in a compound fracture of his right humerus and fracture of cervical

vertebrae (C3/C4) which was corrected by spondylodesis in 2004 (Figure 2). The remainder of the patient’s information including patient demographic, past medical history, allergies, medications, social history, and family history are out- lined in Table 2.

Dental history

The patient exhibited shy and moder- ately anxious demeanor, but was largely cooperative for the dental examination with verbal and physical (handholding) encouragement from the patient’s father.

Frequent breaks were required due to the patient’s limited opening necessitating shorter appointments for subsequent visits.

The initial oral examination revealed existing restorations on #30 and #31 with recurrent caries, which were treat- ment planned for amalgam restorations.

Subsequent dental appointments revealed secondary caries on #14MO and #31MO;

primary caries on #30MO, #15O, #21D,

#28D, and #31B; and incipient caries on

#5D, #19M, and #21O. Tooth #14 was treatment planned for amalgam, #31 for MOB amalgam, and remaining caries for resin-based composite restorations (Table 3). At a subsequent restorative appointment, tooth #14 restoration was Table 1. Clinical manifestations of premature aging syndromes.

Acrogeria Metageria Acrometageria/This Patient

Stature Normal Tall and thin Short and thin

Face Micrognathia & atrophy of skin on tip of nose Beaked nose Micrognathia & beaked nose

Skin Atrophy on extremities Atrophy on limbs Atrophy on extremities

Scalp hair Normal Fine and thin Fine and thin

Eyes Normal “Prominent” Normal

Nails Dystrophic and thickened Normal Normal

Limbs No leg ulcers Generalized loss of subcutaneous fat Little subcutaneous fat

Cardiovascular Normal Early atherosclerosis Normal

Skeletal Normal Normal Scoliosis

Metabolic Normal Early onset diabetes mellitus Normal

Inheritance Autosomal recessive Autosomal recessive Unknown

Sex incidence Mostly female Both equally Male

Onset Birth Birth Birth

Prognosis Normal lifespan Dependent on severity of DM and

atherosclerosis

Unknown

Table 2. Patient information.

Patient demographic 38-year-old Caucasian male 162 cm, 51 kg

ASA II Medical history Acrometageria

Scoliosis Cataracts

Raynaud’s syndrome Intellectual disability

Allergies Penicillin

Medications Denies

Past surgical history Orthopedic Hand Surgery for Joint Hypermobility 1993 Spondylodesis C3/C4 following Motor Vehicle Accident 2004

Social history Denies

Family history: Mother: Factor V Leiden Thrombophilia Father: None

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determined to be unrestorable and treat- ment planned for extraction. The patient was successfully managed for initial

examination, radiographs and subse- quent restorative appointments with behavioral, psychological, and other

nonpharmacological techniques. Tooth

#14 was planned for extraction under intravenous sedation.

The patient’s father accompanied him to the treatment area and provided encouragement for intravenous periph- eral catheter placement. A 20-gauge catheter was secured in the left forearm, through which the patient received mida- zolam 2 mg and sufentanil 2.5 mcg.

Propofol infusion was initiated at a rate of 40 mcg/kg/min and supplemented with incremental dexmedetomidine in 4 mcg doses over the course of treatment for a total of 24 mcg. The patient’s father remained in the treatment area until the patient was sedated. The patient received 2% lidocaine (72 mg) with epinephrine 1:100:000 (0.036 mg) for maxillary infil- tration and tooth #14 was extracted with minimal bleeding. Following extraction, the propofol infusion was discontinued;

sedation was maintained with dexme- detomidine and a second dose of sufentanil 2.5 mcg. The resin restoration of tooth #21 was tolerated with minimal- to-moderate sedation. Based on

limitations associated with providing dental care in a learning institution and the patient’s disabilities, the remaining treatment was scheduled to be completed during subsequent appointments.

Discussion

Progeroid syndromes are conditions characterized by premature aging and shortened life expectancies.2 Frequently, patients exhibit signs of accelerated aging such as sclerotic skin, heart and bone abnormalities, and growth attenuation.

Affected individuals display respiratory and cardiovascular capacities comparable to the elderly.7 In many cases, craniofa- cial features are evident including

“bird-like” facies, alopecia, craniofacial disproportion, hypodontia, delayed erup- tion, and dental crowding. While there are over a dozen syndromes that tend to be associated with premature aging, the approach to dental management of patients is relatively similar.2

In patients with progeroid syn- dromes, attention to developmental status as it relates to chronological age is Figure 1. Physical examination and airway assessment as predictors of difficult airway. (A) Frontal

view. (B) Sagittal view. (C) and (E) Frontal view of patient exercising maximum opening. The decreased mouth opening may be appreciated. (D) Sagittal view of patient depicting decreased maximum opening and cervical range of motion. (F) Patient Mallampati Class IV. Decreased open- ing and limited visualization of soft palate, uvula, and tonsillar pillars. (G) Patient thyromental distance approximately two fingerbreadths.

Figure 2. (A) and (B) demonstrate the wrinkled skin on the hands and feet; hypermobile joints. (C) Incision site for spondylodesis of cervical vertebrae C3/C4 following traumatic MVA.

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important for successful treatment. A thorough medical history and clinical examination are useful in establishing

parameters for care. Scheduled appoint- ment times, length of appointments, stress reduction protocols, and proper

positioning are other necessary consider- ations.8 As with older adults and geriatric patients, those with premature aging may have limitations which create a need for specialized dental care.

The American Dental Association (ADA) defines patients with special needs as “those who due to physical, medical, developmental, or cognitive conditions require special consideration when receiving dental treatment.6” A variety of management techniques have been developed to aid patients with special needs to receive dental treatment services (Figure 3). Clinical guidelines have been drafted by the American Academy of Pediatric Dentistry (AAPD) and supported by the Special Care Dentistry Association (SCDA).9 Furthermore, in 2009, the SCDA pub- lished a consensus statement on sedation, anesthesia, and alternative techniques for people with special needs.18 These techniques and considera- tions were applied in treatment of the patient presented in this case report.

Information gathered from the comprehensive oral evaluation was utilized for proper patient assessment—

considering medical history, physical examination, and dental treatment plan.9 In determining a treatment modality for this patient, consideration was given to each of the patient’s conditions and asso- ciated limitations to treatment (Table 4).

For the initial comprehensive oral examination, diagnostic radiographs, adult prophylaxis, and restorative treatment, the patient was successfully Table 3. Patient’s dental treatment plan.

Tooth # Existing restorations Existing defects Treatment plan

5 None D incipient caries No treatment

14 MO amalgam MO secondary caries MO amalgam

15 None O primary caries O resin-based composite

19 None M incipient caries MO amalgam

21 None O incipient caries O resin-based composite

28 None D primary caries D resin-based composite

30 DO amalgam M Caries, O secondary caries MOD resin-based composite

31 MO amalgam B primary caries. MO secondary caries MOB amalgam

Note: Tooth #14 was eventually retreatment planned for extraction.

Table 4. Patient’s conditions and associated limitations.

Patient’s condition

Patient’s associated signs and symp- toms

Considerations for dental treatment

Dental anxiety Shy, restrained demeanor Duration of procedure Moderate fear of dental treatment Doctor–patient communication Acrometageria Hyperflexible joints Patient positioning

Mallampati Class IV Duration of procedure

Micrognathia Inability to sustain mouth opening

Decreased mouth opening Decreased cervical range of motion

Scoliosis Spinal deformity/curvature Patient positioning Duration of procedure Intellectual

disability

Limitations in adaptive functioning

Chronological versus developmental age

Doctor–patient communication Trauma/surgical

history

C3/C4 spinal fracture and spondylodesis

Patient positioning Duration of procedure Figure 3. Patient management options.

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managed with behavioral, psychological, and other nonpharmacological support techniques. Behavioral support techniques utilized in patient treatment included voice control, nonverbal communication, tell-show-do, positive reinforcement, con- tingent escape, distraction, and parent presence.12 The patient exhibited moder- ate anxiety, but appeared to benefit from the presence of his father at chairside for the duration of the procedure. The father assisted by providing periodic verbal and physical encouragement in the form of reassurance and handholding, respectively.

The patient’s limited opening and microg- nathia necessitated frequent rest periods during procedures. Scoliosis, limited

range of motion, previous history of trauma, and intellectual disability affected the patient’s ability to tolerate long appointments requiring shorter sessions.

Table 5 details the behavioral support techniques used with this patient.

Psychological support was also uti- lized in the treatment of this patient.

Current literature shows that patients with special needs are sometimes more comorbidly dental phobic than others.13 The patient presented in this case report appeared to experience moderate dental anxiety, which was addressed with psy- chological support techniques including relaxation, breathing, and effective use of developmentally appropriate operatory

language. These methods created a calm work setting for both doctor and patient.

Social support was additionally employed to provide continuity of treatment through patient and family education.

This approach benefited the patient with knowledge for home care and overall oral health maintenance.11

Nonpharmacological techniques proved successful for examination, radio- graphs, and operative dentistry; however, deep sedation was provided for a poten- tially difficult extraction of tooth #14.

Patients with special needs require a vari- ety of modalities for treatment, ranging from good rapport to general anesthesia.

While the ADA and AAPD provide a number of guidelines in literature related to providing sedation and general anes- thesia for dentistry, few specifically discuss indications for use of sedation or anesthesia. The focus of guidelines is often on education for methods of deliv- ery.9,10,14–16 The Special Care Dentistry Association lists seven rationales for use of sedation and general anesthesia, which were similar rationales for treatment of the presented patient.17,18 The factors include: healthy history, medical status, time and effort, cost, and risks among others listed in Table 6.

The patient presented fulfilled a number of rationales for sedation and general anesthesia. He showed decreased developmental status versus chronologi- cal age, required a reduction in pain and anxiety, and was not able to cooperate in a manner that would allow surgery to be completed in an optimally safe fashion.

The patient also requested the use of sedation or general anesthesia for extrac- tion of #14, recognizing the utility of decreasing pain and anxiety.

In the decision-making process for determining treatment planning sedation/

general anesthesia, a number of factors are considered (Table 7).16 The presented patient’s health history and physical status played a major role in forming a definitive plan for combination deep and moderate sedation. Manifestations of patient’s acro- metageria revealed Mallampati Class IV, micrognathia, decreased mouth opening, decreased thyromental distance, and decreased cervical range of motion; all are Table 5. Behavioral support techniques.

Behavioral support techniques Description

Voice control Alteration of vocal volume, pace, and intonation to gain patient’s attention and influence behavioral direction Nonverbal communication Recognition of nonverbal cues: sensitivity to touch, body

language, and facial expressions

Tell-show-do Approach of adding sensory demonstration cues (visual, audi- tory, touch, proprioception, etc.) to a simple verbal description of procedure prior to the performance of procedure

Positive reinforcement Process of rewarding acceptable/desired behavior with verbal praise, expression, touch, or tokens

Contingent escape Offer of momentary cessation of treatment or other positive reinforcers, conditional upon periods of acceptable target behavior

Distraction Method of diverting a patient’s mental focus to positive thoughts, favorable environmental stimuli, or other stimulating sensory images in an effort to override unpleasant procedures

Parental presence Utilization of a parent to increase the patient’s psychological comfort and reduce patient anxiety

Source: Lyons (2009)

Table 6. Keys to patient assessment.

Evaluation factors

1. Patient’s health history and current medical status 2. Likelihood of procedures being completed successfully

3. Time and effort required from the patient and health care provider 4. Cost of contemplated procedures

5. Risk of side effects of any aspect of treatment 6. Social environment and support available 7. Availability of various treatment modalities 8. Urgency of care for that individual Source: Glassman et al. (2009)

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indications of a poor airway. Furthermore, intubated general anesthesia may have proven difficult given the patient’s history of trauma, fusion of C3 & C4, scoliosis, hyperflexible joints, predisposition to frac- tures, and concerns with patient head and

neck positioning for endotracheal tube placement.

After considering the risks and benefits of providing sedation and gen- eral anesthesia (Table 8), combination therapy of deep sedation with transition

to minimal/moderate sedation was selected. The patient was properly posi- tioned with a shoulder roll and mild chin support to maintain airway patency for extraction. The dental history demon- strated cooperation at previous visits without sedation for routine restorative dentistry; a reduction in sedation would likely be tolerable for the remainder of the patient’s appointment. Behavioral and psychological supports were used in con- junction with minimal-to-moderate sedation for completion of the appoint- ment without incident. In fact, literature shows that good communication has the potential to facilitate the acceptance of successful pharmacosedation.19

The remaining restorative treatment was planned for completion with non- pharmacological support techniques. In the event nonpharmacological techniques prove unsuccessful, sedation may be reconsidered. Combination support proved to be the most effective approach for subsequent appointments. However, in addition to understanding support techniques, recognizing barriers to care is also important for comprehensive treatment of dental patients with special needs.20 Access to proper health care is hindered by insufficient numbers of trained personnel, difficulty in obtaining informed consent for treatment deci- sions, lack of accessibility to facilities, and inadequate financial coverage.

Successful oral health care support for patients with disabilities requires identifi- cation of patient management techniques within the larger scheme of addressing general barriers to treatment.

Conclusion

The population of special needs patients is growing with advances in medical care, deinstitutionalization, and changing soci- etal values.11 Many of these individuals require additional attention and support for dental treatment. While a number of treatment modalities exist, universal guidelines for their implementation are not always clear. However, with proper patient assessment, provider education, and treatment planning consideration, a combination of appropriate support Table 7. Rationales for use of sedation and general anesthesia.

1. Individuals with cognitive impairment or emotional conditions who have difficulty understanding what is expected in a dental treatment situation

2. Patient whose fear about receiving dental treatment prevents them from receiving the needed treatment

3. Patients who are unable to sit in a dental chair or remain still enough to have dental procedures performed

4. Patients who have extensive dental needs that would require extended dental treatment over a prolonged period of time

5. Patients who require dental procedures that cannot easily be performed with local anesthesia because of an inability to achieve adequate local anesthesia for that procedure 6. Individuals with complex medical problems who require intra- and perioperative monitoring 7. Individuals with complex medical problems (e.g., severe hypertension and cardiac or respiratory disease) whose physiologic state will be more safely controlled in a sedated or anesthetized state

Source: Glassman et al. (2009)

Table 8. Advantages and disadvantages of general anesthesia.

Advantages Disadvantages

1. Success rate is 100% 1. The patient is unconscious during treatment 2. Patient cooperation is not

absolutely necessary

2. The patient’s protective reflexes are depressed

3. The patient is unconscious during treatment

3. Vital signs are depressed

4. The patient does not respond to pain

4. Advanced training is required for the person administering GA

5. Amnesia is present after procedure

5. A team of professionals (rather than an individual dentist) is required to provide treatment

6. General anesthesia may be the only technique that will prove successful for certain patients

6. Special anesthesia equipment is required

7. The onset of action of general anesthesia is usually quite rapid

7. A recovery area must be available for postoperative monitoring (a private setting or the dental operatory itself may serve as a recovery room)

8. Titration is possible with the patient receiving smallest volume of drug required

8. Intraoperative and postoperative complications are more common during GA than sedation

9. It is recommended that patients receiving GA should refrain from drinking clear liquids for at least 2 hours preoperatively and solids and nonhuman milk for at least 6 hours preoperatively

10. Patients receiving GA may require extensive preoperative evaluation and testing

Source: Malamed (2010)

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modalities may be utilized in successfully caring for patients with special needs.

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