Gorham-Stout Disease of the Skull Base
Description
●Patient Information
•Gender and Age: 27-year-old female
•First Presentation: May 2017
•Previous State of Health: Previously in good health
•Signs and symptoms
➣First:
•gradual loss of teeth over several months
•Swelling of the left mandible (5-day history)
•Trismus (limited movement of the jaw)
•Reduced oral intake with minor injuries
➣Later Signs and Symptoms:
•Pain in the area of the left jaw (January 2018)
•gradual loss of teeth in the mandibular area
•New pimple (or "knot") in the left cheek (February 2020)
➣Results of the Examination
•Over time, the mandible and base of the skull experience progressive osteolysis and bone loss.
•Initial post-operative areas were stable, however later additional lesions were observed.
● Research
➣Picture:
•May 2017 CT Maxillofacial: Osteolysis, hypoplastic mandible, nondisplaced fracture
•CT (January 2018): Right mandibular progression
•CT (February 2020): New osteolysis in the lateral orbital wall, squamosal temporal bone, zygomatic arch, and sphenoid
•Follow-up CT (Post-RT): No new osteolysis, disease stability
•The study of histopathology
•Prolonged inflammation
•Fibrosis of the marrow
•Vascular deformity that is compatible with GSD
➣Diagnosis
•Vanishing Bone Disease, or Gorham-Stout Disease
•An uncommon idiopathic osteolysis condition
•The diagnosis is based on:
•Pattern of radiologic osteolysis
•Histopathology (lack of cancer, vascular proliferation)
•Clinically ruling out neoplasms or infections
• Timeline for the Treatment Process
● Management by Surgery
➣June 2017:
•Mandibulectomy on the left
•Reconstruction using the mandibular plate and free-flap fibular fascio-osseous
➣August 2018:
•Complete mandibulectomy as a result of advancement
•Mandibular prosthesis and right radial forearm free-flap reconstruction
•Overview of the Gorham-Stout Disease
•Definition: A rare progressive osteolysis disease in which vascular or lymphatic tissue replaces bone.
•Cause: Unknown; aberrant vascular proliferation is hypothesized.
•Commonly impacted areas include the mandible, base of the skull, ribs, pelvis, spine, and shoulder girdle.
➣Clinical Course: Unpredictable → may quickly advance or stable on its own
Protocol
●Medical Treatment
•Zoledronic acid (5 mg IV, single dosage) prevents bone resorption caused by osteoclasts.
•Supplemental calcium (1200 mg daily)
•50,000 IU of vitamin D each week
•RT, or radiation therapy
➣ Indication: Progressive illness that is resistant to medication and surgery
➣ Planning Specifics:
• 42 Gy in 21 fractions (2 Gy/fraction) using IMRT
• Clinical Target Volume (CTV): every bone region that is impacted
• CTV + 3 mm margin is the planning target volume (PTV).
• Risky organs include the eyes, brain, spinal cord, optic nerves, and salivary glands --> dosages within tolerance ranges
➣ Delivery:
• Cone beam CT every day for setup confirmation
• No pauses in therapy
•Adverse Reactions to RT
➣ Acute Impact (controllable):
•Analgesics are used to treat grade 2 oral mucositis.
•Grade 1 dysphagia and xerostomia: symptomatic treatment
•Topical treatments for erythema in grade 1 skin
•Lubricating eye drops for grade 1 dry eyes
➣Aftereffects:
• mild grade 2 facial lymphedema brought on by RT and surgery
•Mucositis (Mouth Sores) Issue: Patients may experience excruciating inflammation and ulcers in their throat and mouth, which can make it difficult for them to eat, increase their risk of infection, and result in inadequate nutrition.
➣ Management and Nursing Interventions:
• To reduce the risk of infection and worsening mucositis, promote and teach good oral hygiene.
• Steer clear of irritating products like mouthwashes with alcohol and use simple rinses like saline or sodium bicarbonate.
• For pain management, apply topical analgesics (such as doxepin rinses), antacid-diphenhydramine blends, or mucoadhesive gels.
• Make dietary adjustments and give advice on soluble, non-irritating, high-protein, high-calorie foods.
• Consult nutrition services and make arrangements for enteral tube feeding in extreme circumstances.
• The issue with salivary gland dysfunction and xerostomia (dry mouth) is: Dry mouth, trouble eating, an increased risk of dental cavities, oral infections, and osteonecrosis are all consequences of decreased saliva.
➣ Nursing and Management Interventions:
• Encourage the use of sugar-free gum or lozenges, saliva replacements, and regular sips of water.
• Encourage routine dental care, including fluoride treatments, gentle toothbrushes, and early problem-solving.
oral health education and the use of chlorhexidine rinses.
• Skin Reactions (Dermatitis) Issue: Radiation exposure results in pain, peeling, erythema, and an increased risk of infection at the treatment locations.
➣ Nursing and Management Interventions:
• Teach them how to cleanse their skin gently and to stay away from irritants like tight garments and soaps with fragrances.
• Apply topical steroids or moisturizers without fragrance to the afflicted regions.
• Keep an eye out for infection symptoms, such as pus-filled redness or an unpleasant odor, and accelerate treatment.
• Keep an eye out for infection symptoms, such as pus-filled redness or an unpleasant odor, and accelerate treatment if necessary.
• Give advice on how to shield the skin from the sun and other harm.
• Fatigue Issue: Severe exhaustion that is not alleviated by rest is a prevalent and complex issue in cancer treatment.
➣ Nursing and Management Interventions:
• Determine the contributory causes (anemia, depression, sleep disturbance, diet) and take appropriate action.
• Promote energy-saving techniques, moderate exercise, and enough downtime.
• If you suspect a mood disorder, see mental health or supportive treatment.
• Side effects related to the digestive system (nausea, vomiting, diarrhea)
• The issue encountered: Dehydration, malnutrition, and electrolyte imbalances are common side effects of radiation exposure to the abdomen and pelvis.
➣ Nursing and Management Interventions:
• Report any dehydration or uncontrollable symptoms, and keep an eye on your fluid and electrolyte balance.
• As directed, administer antidiarrheal medications and antiemetics for nausea.
• Encourage the consumption of short, frequent, low-fat meals, steer clear of oily or spicy foods, and follow a diet that is easy to digest.
• Inform people about the symptoms of dehydration, such as dry mouth and decreased urine production, and when to get treatment.
• The issue with nutritional deficiencies is poor intake brought on by fatigue, anorexia, nausea, mucositis, and changed taste.
➣ Nursing and Management Interventions:
• Set up a nutritionist or dietician consultation for an evaluation and customized strategy.
• Provide additional nourishment, either orally or intravenously if required.
• Keep an eye on laboratory results and weight.
•The issue with lymphedema (swelling) is: Particularly following surgery or lymph node radiotherapy, it shows up as excruciating swelling and limited mobility.
➣ Nursing and Management Interventions:
• Teach and support the recommended exercises and limb elevation.
• Wear compression clothing as directed.
• For manual lymphatic drainage, if necessary, consult physiotherapy.
• Repercussions and Psychosocial Requirements
➣ Issue: It may take months or years for fibrosis, strictures, secondary malignancies, persistent pain, depression, and anxiety to manifest.
➣ Nursing and Management Interventions:
• Maintain ongoing surveillance for side effects and recurrent cancers.
• Assess for and treat psychosocial problems; if required, assist in making referrals for therapy or psychiatric assistance.
• Encourage adherence to survivorship care plans and routine follow-up visits.
Notes
For more details visit :https://pmc.ncbi.nlm.nih.gov/articles/PMC8543380/?utm_source=chatgpt.com
