Wednesday, November 30, 2016

Oral manifestations in patients with aplastic anemia

Department of Pediatric Dentistry
Lutheran Medical Center
           
Resident’s Name: Nicholas Paquin                                                               Date: 11/30/2016
Faculty Mentor: Dr. Hencler
Article Title: Oral manifestations in patients with aplastic anemia
Author(s): Michael T. Brennan, DDS et al
Journal: Oral Surg Oral Med Oral Pathol Oral Radio Endod
Date: 2001
Major Topic: Oral manifestations and clinical considerations of patient with aplastic anemia
Type of Article:
Main Purpose: To know clinical guidelines of patients with aplastic anemia and possible oral manifestations and treatment goals.
Key Points: (2 lines Max): 1. Patient with aplastic anemia produce insufficient numbers of hematopoietic stem cells, leading to deficient production of erythrocytes, granulocytes, and platelets. 2. Patients with AA presented more frequently with oral petechiae, gingival hyperplasia, spontaneous gingival bleeding and herpetic lesions. 3. Grossly infected teeth in patients with AA put them at risk for systemic infections from oral sources. Patient with AA should have routine dental exams and treatment.  
-        Patient with aplastic anemia produce insufficient numbers of hematopoietic stem cells, leading to deficient production of erythrocytes, granulocytes, and platelets.
-        The disease is uncommon, 2cases/million in western countries.
-        50% unknown cause. There are associations with certain medications, benzene exposure, insecticides, viruses and hepatitits.
-        Severe AA neutrophil counts <500/ul and platelet counts <20,000/ul
-        Complications from prolonged pancytopenia include high risk of infections. Bacterial sepsis and fungal infections represent the most frequent cause of death in patients with AA.
-        Patients with AA presented more frequently with oral petechiae, gingival hyperplasia (16% - it should be noted patients with cyclosporine treatment were at 22 fold increased risk), spontaneous gingival bleeding (no risk factors were indicated, could be due to heavy plaque accumulation and calculus) and herpetic lesions (16%).

-        Dental management issues: predental treatment prophylaxis is often indicated (platelet transfusions or antibiotics or both). Hemoglobin levels before and after extractions, development of fever or infection after treatment, presence of bacteremia.

-        It is recommended that extractions of more invasive dental treatment for AA patients with pancytopenia be provided in a hospital setting to ensure appropriate follow-up of acute complications that may arise. In this study 30% of patients undergoing extractions required at least one more infusion of platelets and/or packed RBCs within 4 days of the procedure.

-        Grossly infected teeth in patients with AA put them at risk for systemic infections from oral sources. Patient with AA should have routine dental exams and treatment. 
Remarks:
1)     Good summary of this article, all major points presented is this review.
2)     As aplastic anemia is rare, I have not treated a patient with aplastic anemia.
3)     Important for pediatric dentists to be familiar with AA as some reports suggest peak incidence may occur in children aged 3-5 years old or 10 to 25 years old.
4)     Acquired form of AA occurs as reaction to certain meds (anti-inflammatory, anti-epileptical, anti-diabetic, anti-malarial), and chemical agents.  Complications associated with hepatitis have also been implicated in AA.  In half of patients with AA no causative agent can be identified.
5)     Prolonged bleeding in AA patients with platelet counts lower than 50,000mm3 must be considered with invasive dental procedures.  Avoid posterior superior alveolar nerve blocks, avoid inferior alveolar nerve blocks.  For invasive dental procedures, patient with AA should be seen in hospital setting under GA with appropriate hematologic and antibiotic coverage.
6)     Further reading to consider – Conservative Management for a Patient with Aplastic Anemia Alton G. McWhorter, DDS, MS and Sharon D. Hill, DDS, MS Pediatric Dentistry July/August 1991 vol13, number 4
Assessment of Article:  Good article presenting oral findings in patients with AA.
Level of Evidence/Comments: In this study many of the risk factors associated with AA were not found to be statistically predictive of observed oral findings. 


Tuesday, November 29, 2016

The use of bisphosphonates in children: review of the literature and guidelines for dental management.

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name: Amir Yavari (Mentor: Dr. Mindy)                                               Date: 11/30/2016

Article Title:  
The use of bisphosphonates in children: review of the literature and guidelines for dental management.
Author(s):  RN Bhatt, SA Hibbert, CF Munns
Journal: Australian Dental Journal 
Date: 2014
Major Topic: Clinical use of bisphosphonate in children
Type of Article: Literature Review
Main Purpose:
This paper aims to outline pediatric uses and adverse effects of bisphosphonates and present recommendations on the dental management of children receiving bisphosphonates.

Summary:
Bisphosphonates are inhibitors of osteoclastic bone resorption with therapeutic benefit in a variety of bone disorders. There is uncertainty regarding the dental management of children treated with bisphosphonates, particularly when invasive dental procedures, such as extractions and oral surgical procedures, are required. There are limited data with which to make recommendations about the dental management of patients treated with bisphosphonates, and there are no published recommendations that specifically address pediatric patients.

Successful uses of bisphosphonates in children are more diverse than in adults and include:
1.     Primary structural defects in type I collagen and other structural bone proteins (e.g. OI)
2.     Fibrous dysplasia of bone (e.g. McCune-Albright syndromes)
3.   Bone abnormalities resulting from systemic disease or the effects of systemic treatment (e.g. steroid treatment of chronic disease of immobilization)
4.     Bone matrix abnormalities (e.g. osteoporosis pseudoglioma syndrome)
5.     Conditions with a primary defect in bone mineralization (e.g. idiopathic juvenile osteoporosis)
6.     Malignancy associated hypercalcaemia
7.     Focal orthopedic disorders (e.g. traumatic avascular necrosis of the femoral head)

Dental considerations for Bisphosphonates in children
- Bisphosphonate-related osteonecrosis of the jaw (BRONJ): No cases have ever been reported in children
- Tooth eruption and exfoliation: Baseline study at the children hospital at Westmead, Australia, infra-occlusion of primary teeth (delayed exfoliation and /or ankylosis) identified in 27% of patients.  Delayed eruption of permanent teeth in 12.5% of subject in whom this could be assessed. 
- Orthodontic implications: There is not clear understanding of difficulties. Patients and parents through an informed consent should be aware of the potential for BRONJ (due to extractions), the potential for increased treatment time, and possible compromises in the treatment plan and outcome.

General principles for prevention of BRONJ in children:
- Comprehensive assessment + elimination of dental disease or infection, prior to use of bisphosphonates.
- Patient education for maintenance of optimal dental health.
- Postponement of bisphosphonate therapy until dental health is optimized.

Recommendations for oral surgical procedures in children on bisphosphonates:
- Whether bisphosphonate treatment has already commenced
- Whether bisphosphonate treatment will be short-term or long-term therapy?
- Whether dental treatment is immediately required, or can be deferred?




Protocol for invasive dental procedures in children:
(1) The timing of the procedure should be as long as possible from the last infusion, leaving a healing period of three weeks prior to the next infusion to allow for closure of surgical wounds.
(2) Use 0.12% chlorhexidine mouth-rinse twice a day for 5 days before the procedure to minimize inflammation and reduce the microbial burden at the surgical site; followed by twice daily rinse for 5 days after the procedure or until primary healing has occurred.
(3) Conservative surgical technique
(4) Suturing of extraction sockets to approximate the gingival tissues as much as possible. Primary closure is not considered necessary if it would necessitate raising a mucoperiosteal flap.
(5) Periodic review of surgical sites up to 12 months postoperatively.
(6) Where an elective drug holiday has been taken, bisphosphonate therapy should not be recommenced until the surgical sites have healed (14–21 days).
(7) Prophylactic antibiotics should only be considered for those children deemed to be at highest risk for BRONJ


Remarks:
-       A pediatric dentist should know and be aware the most pediatric patients receiving bisphosphonates are not receiving a daily dose rather they may be getting IV or per os dose once every 1-3 months. Those patients might overlook the fact that they are taking medications when reviewing med hx. 
-       Past history of taking these medications are extremely important because their effects might linger in the bone for up to a decade after cessation of medication.
-       These 2 points highlight the importance of inquiring about past hx and past medications that have been prescribed. 

Assessment of Article:  Level of Evidence/Comments:  Level I

Monday, November 28, 2016


Department of Pediatric Dentistry

Lutheran Medical Center
           
Resident’s Name: John Diune   Mentor’s Name: Dr. Shabtai Sapir   Date: 11/30/2016

Article Title:  Branchial Arch Syndromes
Author(s): David Alfi DDS MD, Din Lam DMD MD, Jaime Gateno DDS MD
Journal: Atlas of the Oral & Maxillofacial Surgery Clinics of North America
Date: 2014
Major Topic: Growth and Development – Branchial arch syndromes
Type of Article: Perspective article / Review of topic
Main Purpose: Presentation of Branchial Arch Syndromes, features, treatment and treatment concerns
Key Points: (2 lines Max): Branchial arch syndromes present with variable expressivity
 
 
 
 
ARCH
GROOVE
POUCH
FIRST
1.        Mandible and Maxilla
2.        Meckle’s cartilage
a.        Incus and malleus of innter ear
b.        Sphenomalleolar ligament
c.        Sphenomandibular ligament
External auditory meatus
1.        Tympanic membrane
2.        Tympanic cavity
3.        Mastoid antrum
4.        Eustachian tube
SECOND
1.        Reichert’s cartilage:
a.        Styloid process of termporal bone
b.        Styloid ligament
c.        Lesser horns of hyoid bone
d.        Upper part of the body of the hyoid bone
Obliterated by down growth of the 2nd arch
1.        Largely obliterated
2.        Contributes to tonsil
THIRD
1.        Lower part of the body of the hyoid bone
2.        Greater horns of the hyoid bone
 
1.        Inferior parathyroid gland
2.        Thymus
FOURTH
Cartilage of the larynx
 
1.        Superior parathyroid gland
2.        Ultimobranchial body
FIFTH & SIXTH
Transient
Transient
Transient
 
 

 
1)      Treacher Collins syndrome
a.       Aberrant production of Ribosomal RNA leading to BILATERAL SYMMETRIC abnormal development of structures arising from 1st and 2nd branchial arches
                                                               i.      1st branchial arch – mandible and zygomaticomaxillary complex
                                                             ii.      2nd branchial arch (hyoid arch) – middle ear
b.      Genetics
                                                               i.      1:50,000 live births
                                                             ii.      60% cases new or sporadic mutations
                                                            iii.      Autosomal dominant inheritance pattern (small percentage autosomal recessive)
                                                           iv.      3 recognized gene mutations
1.       Autosomal dominant  – TCOF1 and POLR1D
2.       Autosomal recessive – POLRIC 
c.       Normal intelligence
d.      Treatment concerns
                                                               i.      Airway concerns: Life threatening choanal atresia or stenosis
                                                             ii.      Feeding: lip seal or cleft lip or palate concerns
                                                            iii.      Hearing: early testing
                                                           iv.      Vision: possible extraocular muscle dysfunction or visual acuity deficits;  unprotected corneas due to deficits in lateral and inferior orbital support
                                                             v.      Speech: standard cleft treatment
e.      Surgical treatment
                                                               i.      Secondary treatment at end of skeletal maturity – orthognathic surgery and zygomatic-orbital reconstruction
                                                             ii.      Maxillomandibular reconstruction at early skeletal maturity (13-15 yo)
f.        Differential Diagnoses:
                                                               i.      Craniofacial microsomia
                                                             ii.      Pierre Robin sequence
                                                            iii.      Stickler syndrome – eye, ear, joint deformities plus Pierre Robin sequence
                                                           iv.      Nager syndrome – malformed upper limbs, cleft palate, severe palatal hypoplasia
 
2)      Goldenhar syndrome (term used interchangeably with Hemifacial microsomia [HFM], Oculoauriculovertebral dysplasia)
***note: Goldenhar syndrome used usually when syndrome includes epibulbar dermoids and cervical spine anomalies
a.       Theorized to be due to vascular disruption to developing 1st and 2nd branchial arches (stapedial artery implicated) that is UNILATERAL (or always ASYMETRIC bilateral)
                                                               i.      Microtia
                                                             ii.      Hemifacial microsomia
                                                            iii.      Epibulbar dermoids
                                                           iv.      Preauricular skin tags
b.      Genetics
                                                               i.      Usually acquired sporadically (but also autosomal recessive and dominant transmission)
                                                             ii.      1:3500-26,000 live births
c.       5 major manifestations of HFM – OMENS
                                                               i.      Orbit – abnormal size and/or position
                                                             ii.      Mandible – abnormal ramus, condyle, TMJ
                                                            iii.      Ear – abnormal ear canal, lobule, middle structures
                                                           iv.      Facial nerve – weakness upper and/or lower facial nerves
                                                             v.      Soft tissue – subcutaneous and muscular hypoplasia
d.      Treatment considerations
                                                               i.      Timing of jaw surgery determined individually (controversy over whether mandibular asymmetry is progressive or not with growth)
                                                             ii.      Facial reconstruction should be delayed until skeletal maturity (except in cases of compromised airway, psychosocial problems, or early treatment will aid in subsequent surgeries)
                                                            iii.      Maxillomandibular reconstruction at time of early skeletal maturity (13-15 yo)
                                                           iv.      Computer-aided surgical simulation improves orthognathic surgery outcomes
e.      Differential Diagnoses
                                                               i.      VACTERL – vertebral anomalies, anal atresia, cardiac malformations, tracheoesophageal fistula, renal anomalies, limb anomalies
                                                             ii.      Nager syndrome – downward palpebral fissures, midface hypoplasia, micrognathia, upper limb deformities (usually bilateral findings)
                                                            iii.      TCS
 
3)      Möbius syndrome
a.       Congenital, nonprogressive, bilateral facial (CN VII) and abducens (CN VI) nerve palsy along with other cranial nerve palsies and limb deformities
                                                               i.      “Mask-like” faces – expressionless faces with inability to abduct eye
b.      Genetics
                                                               i.      Sporadic in nature (some cases of autosomal dominant)
                                                             ii.      Theorized to be vascular insufficiency or obstruction of basilar and vertebral arteries causing necrosis of brains stem nuclei with resultant hypoplasia of affected cranial nerves
c.       Treatment considerations
                                                               i.      Social development severely handicapped by inability to express feelings
                                                             ii.      Microsurgical facial reanimation to manage facial expression
                                                            iii.      Dental concerns are increased risk for dental disease (lack of facial muscle movement and masticatory function – poor self-cleansing, trapped food, poor salivary function)
d.      Differential Diagnoses
                                                               i.      Hereditary congenital facial paralysis – shares similar findings but palsy of facial nerve only
 
 
Remarks:
Nothing much to say. Branchial arches and the different components that arise from them are good to know. However, some malformations are not from branchial arches alone, but also from the pouch and grooves. It is important to know the structures that arise from the arches, pouch, and grooves as well and WHY and HOW certain malformations occur.
Example…why are Otic malformations associated with Branchial arches 1 and 2?
Example…why are the Malleus and Stapes associated with Branchial arch defects, which can lead to hearing loss (conductive), and why are esthetic problems associated?
Assessment of Article:  Level of Evidence/Comments: