Introduction
Osteopetrosis is a rare genetic bone disease caused by a functional abnormality of the osteoclasts. Until now there is no codified management for the complications of this pathology and few cases cited in the literature.
Presentation of case
a 19-year-old adult followed in our maxillofacial surgery department in the IBN ROCHD University Hospital for chronic osteomyelitis complicating mandibular osteopetrosis with skin fistulas. Patient operated several times. The persistence of osteomyelitis prevents the installation of a dental prosthesis and the appearance of new fistulas with continuous flow of pus alters the patient's quality of life.
Discussion
Osteopetrosis is a group of rare genetic diseases characterized by osteoclastic insufficiency, poor bone remodeling and increased bone density. the benign form of osteopetrosis called Albers-Schönberg disease. It is a genetically inherited autosomal dominant disease.
The large number of surgical interventions and the use of antibiotics for long periods of time (risk of development of resistance) significantly reduces the quality of life of patients. We must seek other measures to improve the prognosis and codify management.
Conclusion
In osteopetrosis, the maxillofacial surgeons should be aware about the early diagnosis and the appropriate management of the signs and prevent complications.
Syringocystadenoma papilliferum is a rare benign adnexal tumour of the sweat glands. It is considered an infantile tumour since it preferentially affects the newborn in 50% of cases and the child before puberty in 15–30% of cases. And its preferential location is the head and neck, but rare in the face. And the first line treatment remains surgery. We report here a case of Syringocystadenoma papilliferum in a nasal location in a 70 year old subject with a history of pemphigus vulgaris, treated by surgical excision, whose postoperative course was aggravated by Koebner phenomenon.
Introduction
Maxillomandibular sygnathia is a rare and severe craniofacial deformity defined by gingival mucosal fusion (synechia) or bony fusion (synostosis).
Case report
We will present a case of complete closure of the mouth since birth from eastern Morocco treated in our department of maxillofacial surgery at the University Hospital of Casablanca. The patient was referred after two months by the pediatrician of the provincial hospital and following the placement of a nasogastric tube. On admission, she presented with facial dysmorphosis, signs of malnutrition with closure of the mouth and fusion of both gums with pro-alveoli and retromandibulia. CT scan with 3D reconstruction confirmed maxillomandibular synostosis. 5 days later, she underwent a fixed mucosal incision with osteotomy at the maxillomandibular joints with early and prolonged active physical therapy. The evolution was marked by the recurrence of mouth closure, the little girl was operated a second time and then she died by a mucous plug at the level of her tracheostomy cannula during resuscitation.
Discussion
Maxillomandibular sygnathia is a very rare pathology whose origin remains unknown. Very few cases published in the literature.
Conclusion
The therapeutic difficulties encountered outside the ideal age of the operation and the management of recurrences were also linked to socio-economic factors making it difficult to ensure adequate postoperative follow-up.
Introduction
Ameloblastoma is a rare tumor, benign but rapidly extensive and prone to recurrence. Its management remains difficult and its treatment relies mainly on surgery. For giant ameloblastoma or in people with an advanced stage the gesture remains very mutilating.
Presentation of case
This is a 22 year old patient, the onset of the symptomatology dates back to 2 years ago with the appearance of a mandibular swelling that increased in size with dental mobility. this motivated the patient to consult a CT scan and a panoramic radiograph as well as a biopsy that objectified an ameloblastoma. He benefited from a surgical excision with reconstruction.
Discussion
It is a rare tumor that mainly affects young people and especially males. It can be discovered by chance or generally in front of a mandibular swelling. Radiological examination is essential as well as biopsy to confirm the diagnosis. The treatment is surgical, which consists of an exeresis with safety margins. Reconstruction should be discussed especially for young healthy subjects.
Conclusion
Although ameloblastoma remains a benign tumor, it is a tumor that evolves rapidly and recurs a lot, which is why it is necessary to take care of it quickly with a radical treatment and a regular follow-up with the patients.
Introduction
cutaneous adenoid cystic carcinoma is a rare epithelial tumor with slow development, it is most often described in the scalp; palpebral involvement is very rare. And is accompanied by local aggressiveness and frequent recurrence. The aim of this work is to discuss the management and the interest of a close surveillance of adenoid cystic carcinoma.
Case presentation
The authors report a case of palpebral cystic adenoid carcinoma in a 65-year-old patient who underwent surgical excision followed by reconstruction of the loss of substance.
Discussion
Cutaneous cystic adenoid carcinoma of the eyelids remains a very rare entity that must be evoked in front of a nodular cutaneous palpebral tumor that progresses slowly. An early diagnosis and a complete excision guarantee a better prognosis.
Conclusion
Adenoid cystic carcinoma, because of its local aggressiveness and its capacity to recur despite satisfactory surgical removal, requires long-term surveillance. Its surgery may be responsible for functional and aesthetic sequelae.
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