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ABSTRACT: Craniofacial clefts are certainly among the most challenging congenital malformations with respect to functional, aesthetic and psychosocial consequences. The aetiology is still under discussion, recent molecular genetic findings suggest defects in the ciliary function of neural crest cells during facial development. The severity of craniofacial clefting is known to be extremely variable. Different classifications have been proposed however nomenclature is not uniform. If vertical, median craniofacial clefting of fronto-naso-maxillary structures is accompanied by auriculo-mandibular malformations the term oculo-auriculo-fronto-nasal syndrome (OAFNS) has been proposed. Extreme craniofacial abnormalities have to be expected in this rare disorder. Adequate correction is a surgical challenge and interventions have to be adapted individually to patient's needs with respect to general condition, age and growth. This case report describes both the underlying pathology as well as the interdisciplinary management of a female patient from birth to 6years of age affected by this rare combination of vertical craniofacial clefting and bilateral auriculo-mandibular dysplasia.
Journal of cranio-maxillo-facial surgery: official publication of the European Association for Cranio-Maxillo-Facial Surgery 01/2012; · 1.25 Impact Factor
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ABSTRACT: A 34-year-old patient with asymmetric hyperostosis of the craniofacial skeleton much more pronounced on the right side is presented. A long-term follow-up of 16 years showed progression of overgrowth even after skeletal maturity and despite repeated surgical corrections focusing on regional reduction of the hyperostoses. Clinical situation during infancy, adolescence, and adulthood is documented. Although the craniofacial hyperostosis in the patient was indicative of the Proteus syndrome, the applicable criteria were not met. Proportionate mild hemihypertrophia/hyperplasia of the patient's right extremities also exclude an oligosymptomatic variant of the Proteus syndrome limited to the skull. Both surgical strategies and difficulty of a diagnostic classification are discussed.
The Journal of craniofacial surgery 11/2011; 22(6):2135-40. · 0.81 Impact Factor
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ABSTRACT: Damage to dentoalveolar structures related to general anaesthesia is a well-known complication and may represent a relevant morbidity for affected patients. Central documentation of perioperative dentoalveolar injuries was performed since 1990 in the Department of Anaesthesiology and Intensive Care Medicine in cooperation with the Department of Oral and Maxillofacial Surgery at the Charité Universitätsmedizin Berlin, Campus Virchow-Klinikum. Documentation of perioperative dentoalveolar injury consisted of anaesthesia charts, reports of the anaesthesiologists and consultant maxillofacial surgeons.
Retrospective analysis of the data from 1990 to 2004 was performed according to this documentation with respect to incidence, matter, distribution of dental injury and therapeutic consequences.
Within 14 years 82 'dental injuries' with 103 affected teeth were documented in calculated 375,000 general anaesthesias. Incidence of 0.02% was very constant with an average of 5.5 events/year. Eighty-nine percent of the documented injuries occurred during scheduled operative procedures. Only 32.9% of the injuries took place during endotracheal intubation. In about 50% the injury was not related to intubation or extubation but happened during general anaesthesia. In 80% the dental injury was estimated by the anaesthesiologist as 'not avoidable'. In 83% pre-existing affection or structural injury of intraoral tissues was documented, in 32.7% of the affections sufficient therapy could be provided already during inhospital stay.
Perioperative dentoalveolar injury is surely an annoying complication of general anaesthesia. However incidence is rare and seems to be unavoidable. Pre-existing damage to dentoalveolar structures is the main risk for additional injuries related to general anaesthesia. Adequate therapy can be provided by interdisciplinary concepts. There should be a fair balance between the benefit of the surgical procedure and the risk of dental injury related to general anaesthesia. Awareness of the problem and proper documentation are important factors for adequate management in liability cases.
Dental Traumatology 02/2011; 27(1):10-4. · 1.20 Impact Factor
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ABSTRACT: In 1960, Gorlin, Chaudhry and Moss described a syndrome consisting of craniofacial dysostosis in association with hypertrichosis, cardiac, genital, dental and ocular anomalies. Diagnosis is based on typical clinical findings and cannot be performed by molecular genetic analysis until now. There is little in the clinical literature concerning this rare craniofacial syndrome. For functional and psychosocial reasons, surgical correction of the complex craniofacial malformation in a 7-year old Hungarian girl with Gorlin-Chaudhry-Moss syndrome was performed by fronto-facial advancement using internal distraction devices. Postoperatively necrotizing soft tissue infection of the scalp developed leading to termination of the distraction process ahead of schedule and requiring aggressive surgical management. Typical physiological and clinical characteristics were observed both during the initial craniofacial correction as well as during the management of the infectious complication suggesting that the linking of different conditions (surgical trauma plus the selection of toxic microorganisms) has caused tissue destruction rather than the syndromal disorder or the surgical technique of distraction osteogenesis. Although skeletal improvement was achieved residual damage from the infectious complication must be considered as severe.
Journal of cranio-maxillo-facial surgery: official publication of the European Association for Cranio-Maxillo-Facial Surgery 01/2011; 39(8):554-61. · 1.25 Impact Factor
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ABSTRACT: In 1975 Antley and Bixler described an unusual syndromal disorder consisting of complex craniosynostosis with midfacial hypoplasia, dysplasia of ears and nose, radiohumeral synostosis, congenital fractures of the femur and upper airway impairment in a newborn. Additional urogenital and cardiac malformations can be associated however diagnosis is based on a characteristic craniofacial deformity in association with humeroradial synostosis. Complex disturbance of craniofacial growth due to premature synostoses of the cranial base and vault results in a characteristic phenotype. Steroidogenesis due to intrinsic or extrinsic disturbance by maternal fluconazole ingestion during early pregnancy may be impaired. The mode of inheritance is supposed to be autosomal recessive. Mutations in the fibroblast growth factor receptor 2 (FGFR2) as well as mutations in the cytochrome P450 oxidoreductase (OR) gene have been verified. Like in other craniofacial dysostosis syndromes malformation of neuro- and viscerocranium is complex and requires a staged age- and growth-related interdisciplinary management with respect to the individual situation. This case report of a female patient born in 1994 suffering from that rare syndrome describes the interdisciplinary long-term management in one craniofacial centre over 16 years from birth to adolescence.
Journal of cranio-maxillo-facial surgery: official publication of the European Association for Cranio-Maxillo-Facial Surgery 12/2010; 39(7):487-95. · 1.25 Impact Factor
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ABSTRACT: Benign symmetrical lipomatosis (Madelungs disease) is a rare disease of unknown etiology which is characterized by the diffuse growth of non-encapsulated lipomas, usually on the neck and upper trunk. Given the benign nature of the lesion, surgical debulking is the treatment of choice, with liposuction reserved for smaller lesions. A 60-year-old man presented in our clinic with progressive tumor growth especially in the cheek. Intraoperatively all branches of the facial nerve were found to be covered by the lipoma. Although Madelungs disease is a benign lesion, the location of lipoma with possible involvement of vital structures may influence the choice of treatment.
European Journal of Plastic Surgery 11/2004; 27(6):306-308.
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ABSTRACT: The unusual case of a mandibular arteriovenous malformation in a patient with severe hemophilia A and hepatitis C is reported. Supplementary substitution of various coagulation factors allowed direct puncture and intralesional injection of a liquid adhesive, resulting in complete anatomic and clinical cure without peri- or postoperative bleeding. Replacement therapy providing normal levels of relevant coagulation factors enables endovascular treatment in a safe and effective manner in hemophiliac patients.
American Journal of Neuroradiology 05/2004; 25(4):614-7. · 2.93 Impact Factor
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ABSTRACT: We report on our experience in surgical exposure and direct cannulation of the superior ophthalmic vein (SOV) for embolization of dural cavernous fistulas. Surgical incision of the eyelid and direct cannulation of the superior ophthalmic vein was performed in 5 patients. All patients had undergone previous attempts of arterial embolizations and/or transfemoral venous approaches which had failed to reach the fistula site. We were able to occlude the fistulas in all patients in 1 session, resulting in complete angiographic disappearance. In 1 patient surgical cannulation of the superior ophthalmic vein failured due to the specific anatomy, so that it was necessary to perform a direct puncture in the depth of the orbit. All patients showed resolution of clinical symptoms within 2 to 6 months. No complications were observed. The approach via the superior ophthalmic vein is safe and effective for endovascular occlusion of dural cavernous sinus fistulas. Particularly in patients with visual deterioration and unsuccessful on incomplete transfemoral arterial or venous embolizations it represents a valuable alternative. If catheterization of the superior ophthalmic vein is not possible, the direct puncture of the vein deep in the orbit is feasible and may be a last resort. Zusammenfassung: Wir berichten ber unsere Erfahrungen mit dem transophthalmischen Zugang in der Behandlung duraler Fisteln des Sinus cavernosus nach chirurgischer Freilegung und Kanlierung der Vena ophthalmica superior bzw. der Vena angularis bei fnf Patienten. Es gelang in allen Fllen, die Fistel in einer Sitzung vollstndig zu verschlieen. Bei einem Patienten war aufgrund besonderer anatomischer Verhltnisse eine Punktion der Vena ophthalmica superior im hinteren Orbitadrittel erforderlich. Alle Patienten zeigten eine vollstndige Rckbildung der klinischen Symptomatik innerhalb von zwei bis sechs Monaten ohne Komplikationen. Der transophthalmologische Zugang zum Sinus cavernosus ist eine sichere und effektive Methode in der endovaskulren Therapie duraler Sinus-cavernosus-Fisteln und stellt insbesondere nach erfolgloser transfemoraler Katheterisierung und bei drohender klinischer Verschlechterung eine wertvolle Alternative dar.
Clinical Neuroradiology 07/2000; 10(3):101-107. · 1.09 Impact Factor
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ABSTRACT: This study sought to develop treatment strategies for managing percutaneous infection around craniofacial implants. The present general pathogen situation together with a bacterial resistance were determined in 57 infected peri-implant sites. Forty-four implants were randomly assigned for wound cleaning and split into three groups-two with local antibiotics of proven efficacy and one with 3% hydrogen peroxide (H2O2). The pathogen spectrum differed depending on the severity of the infection, with Staphylococcus aureus clearly correlated with the degree of inflammation (positive correlation: R = 0.72). It was observed that the use of additional local antibiotics was not superior to conventional wound cleaning with 3% H2O2. It is suggested that sulcus fluid flow rate measurements could serve as a simple and reliable objective parameter for recall examinations.
The International journal of prosthodontics 22(6):594-6. · 1.38 Impact Factor
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ABSTRACT: Patients who have undergone enucleation of the eye can be treated with glass eye prostheses, provided retention is adequate. Inadequate retention due to contraction of the conjunctival mucosa is a common problem which frequently affects the lower fornix first. This can be corrected using a free full-thickness buccal mucosa graft. Indications, operative procedures and postoperative results are presented.ZusammenfassungNach Enukleation eines Auges können Patienten mit Augenprothesen aus Glas rehabilitiert werden. Voraussetzung ist allerdings ein ausreichender Halt der Prothese. Unzureichender Halt bedingt durch die Kontraktion der Konjunktiva ist ein häufiges Problem, welches die untere konjuktivale Umschlagsfalte meist zuerst betrifft. Der Halt kann durch ein freies buccales Mukosatransplantat verbessert werden. Indikationen, operatives Vorgehen, postoperative Ergebnisse werden dargestellt.
International Journal of Oral and Maxillofacial Surgery.
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ABSTRACT: Carcinoma cuniculatum (CC) is a rare variant of squamous cell carcinoma usually occurring in the lower extremity. Occurrence in the oral cavity is unusual and was reported in the literature less than 20 times. A 81 year old female presented with pain in the left mandible. Clinical examination showed no pathologic result but panoramic radiograph showed osteolytic lesion. Result of histological study was CC and local resection was done. Eight month later patient presented again with pain in the contralateral side of the mandible and follow up X-ray showed a new osteolytic lesion. Histology confirmed second occurrence of CC. CC is rare entity especially in the head and neck showing local aggressive behavior but should be considered as differential diagnosis.
Oral Oncology Extra 42(4):173-175.
