Impact of microscopic orbital periosteum invasion in orbital preservation surgery.

OBJECTIVE: The orbital periosteum is considered to be a barrier to tumor spread; however, it is difficult to evaluate microscopic tumor spread during surgery. This study aimed to assess the impact of pathological status in orbital preservation surgery. METHODS: We retrospectively analyzed the 3-year local control rate and treatment outcomes of patients with malignant tumors invading the orbit who were treated between 2006 and 2012. RESULTS: In total, 27 patients were reviewed over a median follow-up period of 36 months. Pathologically, 19 had carcinomas and 8 had sarcomas. Treatment was by orbital exenteration in 6 patients and orbital preservation surgery in 21 patients. After orbital preservation surgery, poorer 3-year local control rates were significantly associated with positive surgical margins (negative vs. positive: 91% vs. 41%, P = 0.040) and microscopic orbital periosteum invasion (negative vs. positive: 90% vs. 39%, P = 0.010). These factors were independent risk factors in multivariate analysis. The locations of the positive margin were most common at the horizontal and vertical margins of the orbital periosteum and the posterior margin of the orbital apex. Moreover, in 24% of patients, invasion evaluation by preoperative imaging study was underestimated compared with postoperative microscopic evaluation. CONCLUSIONS: The positive surgical margin and microscopic orbital periosteum invasion were the risk factors of orbital recurrence. It is difficult to determine the indications for orbital preservation surgery by preoperative imaging studies because of the unpredictable accurate pathological status before surgery and the limitations of preoperative imaging evaluation.

Skull Subsidence due to Periosteum Defect following Craniotomy in a Child.

We report a case of a 7-year-old child with a cranial deformity secondary to a craniotomy for an intracranial hematoma. He suffered from an acute epidural hematoma with a lineal fracture of the right temporal bone following a severe head injury. A large question mark-shaped skin flap with the periosteum and temporal muscle was created for a decompressive craniectomy; however, neither the acute epidural hematoma nor brain swelling was severe, and we performed a small craniotomy compared with the skin flap without a decompressive craniectomy. Eighteen months after the operation, he complained of skull deformity with a mild depression of the forehead. Axial and 3-dimensional computed tomography showed thinness and subsidence of the frontoparietal bone around the replaced bone flap, corresponding to the skin flap with the periosteum defect. We suggest that the periosteum defect following creation of the skin flap during surgery prevented normal growth of the skull, which caused the thinness and subsidence of the frontoparietal bone. Preservation and cooptation of the periosteum should be performed during neurological surgeries in babies and children to prevent skull deformity.

Ontogenetic explanation for tegmen tympani dehiscence and superior semicircular canal dehiscence association. / Explicación ontogénica para la asociación entre dehiscencia del tegmen tympani y dehiscencia del canal semicircular superior.

OBJECTIVES: To analyze the ontogeny of the superior semicircular canal and tegmen tympani and determine if there are common embryological factors explaining both associated dehiscence. METHODS: We analyzed 77 human embryological series aged between 6 weeks and newborn. Preparations were serially cut and stained with Masson's trichrome technique. RESULTS: The tegmental prolongation of tegmen tympani and superior semicircular canal originate from the same structure, the otic capsule, and have the same type of endochondral ossification; while the extension of the squamous prolongation of tegmen tympani runs from the temporal squama and ossification is directly of intramembranous type. The nuclei of ossification of the superior and external semicircular canals and accessory of tegmen collaborate in the ossification of the tegmental extension and by growth extend to the tegmental prolongation. This fact plus the fact that both structures share a common layer of external periosteum could explain the coexistence of lack of bone coverage in tegmen and superior semicircular canal. CONCLUSION: The development of the semicircular canal and tegmen tympani could explain the causes of the association of both dehiscences.

Tratamento cirúrgico da paquidermoperiostose primária - relato de dois casos / Surgical Treatment of Primary Pachydermoperiostosis: Report of Two Cases / Surgical Treatment of Primary Pachydermoperiostosis: Report of Two Cases

A paquidermoperiostose primária é uma doença rara, caracterizada por aposição excessiva do periósteo do crânio, coexistindo com espessamento da epiderme e derme (paquidermia), provocando deformidades grosseiras. Devido à diversidade de estruturas acometidas, há várias opções cirúrgicas e métodos complementares que são utilizados no tratamento das alterações faciais desses pacientes. Esse trabalho apresenta o lifting subperiosteal como uma opção de tratamento estético para a face de pacientes portadores dessa síndrome, através do relato de dois casos operados no Hospital das Clínicas da Universidade Federal de Minas Gerais.

Primary pachydermoperiostosis is a rare disease characterized by excessive skull affixing of the periosteum, coexisting with thickening of the epidermis and dermis (pachydermia), thereby causing gross deformities. Owing to the variety of affected structures, there are several surgical options and complementary methods that are used in the treatment of facial alterations in these patients. This report describes the use of subperiosteal detachment as an aesthetic treatment option for the faces of two patients with primary pachydermoperiostosis, operated at the Hospital das Clínicas of the Federal University of Minas Gerais.

Gracile bones, periostal appositions, hypomineralization of the cranial vault, and mental retardation in brothers: milder variant of osteocraniostenosis or new syndrome?

We report on two brothers with ossification anomalies of membranous and cranial bones, remodeling defect of long bones leading to dense, overtubulated, narrow diaphyses, metaphyseal flare, periostal hyperosotosis that increased during the first months of life, thoracic dystrophy and severe hypotonia. One boy had hypospadias and cleft palate. Follow-up of the surviving boy documented progressive osteopenia, slow healing of the periostal anomalies, liver angiomatosis, mental and motor delay, thoracic deformity, delay in tooth eruption, and progressive microcephaly with enlargement of the cerebral ventricles. This disorder shares some traits with osteocraniostenosis, but lacks the cranial deformity and acromelic micromelia of the latter, in which periostal anomalies are not described. The syndrome reported here may represent a milder form of osteocraniostenosis, or a new entity belonging to the same "family." Genealogical data are consistent with AR or XLR inheritance. No mutations were found in the coding sequence of filamin A.

Oto-palato-digital syndrome, type II: report of three cases with further delineation of the chondro-osseous morphology.

Oto-palato-digital syndrome type II (OPD II) is a lethal X-linked skeletal dysplasia with pleiotropic manifestations. The basic defect is not known. There has been only one detailed report of the chondro-osseous abnormalities in this condition describing abnormal periosteal ossification in a single case [1990: Am J Med Genet 36:226-231]. We report on three cases of OPD II emphasizing the chondro-osseous morphology. Although endochondral ossification was normal, periosteal ossification was defective with islands of cortical bone aplasia and hyperplasia of the periosteum. The trabecular bone was also extremely poorly formed and markedly hypercellular. Both membranous ossification and bone remodeling appear to be defective in OPD II and should account for part of the observed phenotype. The biglycan gene maps to Xq28 and is involved in bone formation, but was excluded as a candidate by direct sequencing of cDNA in one case.

Periosteal tether causing angular growth deformity: report of two clinical cases and an experimental model.

Two human cases of periosteal tethering causing a disturbance of longitudinal bone growth are presented. Production of a periosteal tether in an experimental rabbit model supported a causal relationship between the tether and the growth disturbance. Excision of the periosteal tether (with or without an osteotomy) produced some correction of the growth disturbance in these two cases. One should keep this entity in mind as a possible cause for an acquired growth disturbance in a long bone.

Does interference with mucoperiosteum and palatal bone affect craniofacial growth? An experimental study in beagles.

This study was designed to assess the effects of raising mucoperiosteal flaps and exposing palatal bone at the time of palatoplasty. Using 62 beagle puppies as subjects, we tested the hypothesis that raising mucoperiosteal flaps does not interfere with craniofacial growth. We further hypothesized that the size of the area of bone exposed following palatoplasty does affect subsequent craniofacial growth. The animals were divided into four groups: two control groups (unoperated and unrepaired) and two experimental groups. In the first experimental group, two-flap palatoplasty was used to close the surgically induced palatal defect, leaving narrow strips (0 to 2.5 mm) of bone exposed lateral to the flaps. In the second group, one flap was raised to close the defect, leaving a wide area (5 to 6 mm) of palatal bone exposed on one side. Thirty-four direct craniometric measurements were analyzed. Animals that had elevation of both mucoperiosteal flaps with narrow strips of denuded bone on both sides had less severe craniofacial growth aberrations than those in which the defect was left unrepaired or was repaired with one mucoperiosteal flap leaving a wider area of bare bone exposed. These findings suggest that raising mucoperiosteal flaps is less detrimental to craniofacial growth than leaving large areas of exposed palatal bone.

[Two unusual periosteal exotoses]. / Zwei seltene periostale Exostosen.

After maceration of the skeleton of 2 persons we found 2 different solitary exostoses; one at the sciatic tuber, in the other at the sciatic tuber, in the other at the ventral side of the distal fibula. The exostoses reach a length of 2 cm. Prescher (1987) described a cartilaginous exostosis with a length of 2 cm at the proximal tibia. In our cases the exostoses belong to the periosteal form. We consider them to be bony origins of ligaments: at the sciatic tuber--the bony origin of the sacrotuberal ligament, at the distal fibula--the bony origin of the peroneal compartment of the retinaculum mm extensorum inferius.