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Biomedical subjects

Ibrahim Vargel

Publications and source records attributed to Ibrahim Vargel.

18 recordsLinked to original sources
PubMed2006

Ring chromosome 4 and Wolf-Hirschhorn syndrome (WHS) in a child with multiple anomalies.

We report on a 16-month-old male patient with ring chromosome 4 and deletion of Wolf-Hirschhorn syndrome (WHS) region with multiple congenital anomalies including unilateral cleft lip and palate, iris coloboma, microcephaly, midgut malrotation, hypospadias, and double urethral orifices. Peripheral chromosome analysis of the patient showed 46,XY,r(4)(p16.3q35) de novo. Multicolor fluorescence in situ hybridization (FISH) study was also performed and according to multicolor banding (MCB) a r(4)(::p16.3 --> q34.3 approximately 35.1::) was found in all metaphases. Subtelomeric 4p region, subtelomeric 4q region, as well as, Wolf-Hirschhorn critical region were deleted in ring chromosome 4. Genomic microarray analysis was also performed to delineate the size of deletion. Cranial magnetic resonance imaging (MRI) showed hypoplastic corpus callosum, delayed myelinization, and frontal and occipital lobe atrophies. Both maternal and paternal chromosomal analyses were normal. We compare the phenotypic appearance of our patient with the previously reported 16 cases of ring chromosome 4 in the medical literature.

Abnormalities, Multiple↗
PubMed2006

Results of botulinum toxin: an application to blepharospasm Schwartz-Jampel syndrome.

Schwartz-Jampel syndrome (SJS) is a rare congenital disorder of continuous myotonia, causing visual and eyelid problems such as blepharospasm, acquired ptosis, and blepharophimosis. We report the management of blepharospasm in two sisters with Schwartz-Jampel syndrome with application of botulinum toxin type A (BTX-A), as an alternative to the surgical treatments, such as orbicularis oculi myectomy, levator aponeurosis resection, and lateral canthopexy as described in the literature. Three consequent doses of commercially available BTX-A (Botox-Allergan, Inc. 100 u/via) were injected to orbicularis oculi muscle in two sisters with Schwartz-Jampel syndrome. At the least, the authors waited for a six-month interval to prevent development of sensitivity to BTX-A in patients. No significant improvement was observed after the injection of first dose of BTX-A, with the total dose of 25 units of BTX-A to each orbicularis oculi muscle of the eyes. The muscle tone weakened after the second dose, which was the twice as such of the first applied total dose (50 units of BTX-A to each orbicularis oculi muscle of the eyes) and significant functional and cosmetic improvements were achieved after injection of the third dose, in which the total applied dose was the same as the one applied in second dose. The blepharospasm and eyelid alterations caused by Schwartz-Jampel syndrome should be treated to provide functional and cosmetic improvements. Application of BTX-A to orbicularis oculi muscle for the treatment of blepharospasm could be considered as an alternative to levator resection, and lateral canthopexy in Schwartz-Jampel syndrome.

Adolescent↗
PubMed2005

Blood loss and transfusion rates during repair of craniofacial deformities.

Surgical procedures for correction of craniofacial deformities resulted in unavoidable and extensive blood loss in small children and infants. Almost all of the patients undergoing these procedures will undergo a blood transfusion either during or immediately after the operation. A retrospective review of 30 patients who underwent craniofacial surgery was performed in this study to determine the magnitude of transfusion required for craniofacial surgery, document transfusion morbidity, and identify variables associated with the transfusion. The mean estimated blood loss was 566.8 mL, the mean intraoperative transfusion was 394.8 mL, the mean postoperative transfusion was 103.2 mL, and the mean total transfusion was 505 mL. The mean operative time was 450 minutes, the mean preoperative hemoglobin and the mean postoperative hemoglobin before hospital discharge were 11.6 g/dL and 10.3 g/dL, respectively. Craniofacial surgical procedures involve extensive scalp dissection and calvarial and facial bone osteotomies in patients with a low total blood volume. Every medical and surgical strategy for minimizing the need for blood transfusion should be considered.

Blood Loss, Surgical↗
PubMed2004

Use of a water-vapour permeable polyurethane film (omiderm) in the non-surgical treatment of aplasia cutis congenita.

Aplasia cutis congenita is characterised by congenital absence of all layers of skin, and most commonly affects the scalp. It is often associated with absence of the bone and dura underlining the defective scalp. In this report, we present the result of use of a water-vapour permeable polyurethane film (Omiderm) together with absorbant fine mesh gauze impregnated with 3% bismuth tribromophenate (Xeroform) in the treatment of a cranial defect in a newborn baby of low birth weight, who could not be operated on because of associated medical problems.

Bandages↗
PubMed2004

Stoppers in RED II distraction device: is it possible to prevent pin migration?

Distraction osteogenesis has become popular for the treatment of hypoplastic congenital craniomaxillofacial anomalies. Rigid external distraction (RED II) after Le Fort III osteotomy was shown to be a highly effective treatment for the management of midface hypoplasia. This device is used with a halo vest, which is placed at the cranial equator. Intracranial penetration of the fixation pins of the halo is one of the complications of an external distraction device. To prevent pin penetration in rigid external distraction, the authors designed polylactic acid/polyglycolic acid (PLA/PGA) plates that were circular; the plates were 0.8 cm in diameter and had 1.5-mm holes in the center, through which the tip of the pins would pass. To quantify the applied torque by manual tightening of the screws of the distraction device and to measure intraosseous cone depth created by the penetration of the conical part of the screws with and without the PLA/PGA composite stopper, first an in vitro experiment was undertaken on cadaver. Then these PLA/PGA plaques, or stoppers, were placed over the bone surfaces of the cranium of the patients where the tip of the pins press. PLA/PGA stoppers are malleable and adapt their shape to the interactive forces between bone and the pins. They act as a second barrier, and spread the pressure of the screws to larger surfaces, thus securing better stabilization. The penetration of wider portions of the screw into scalp is reduced, minimizing the scalp damage caused by the screws. Biodegradable and biocompatible PLA/PGA stoppers avoid intracranial migration of the fixation pins, especially in children.

Adolescent↗
PubMed2004

Isolated supraorbital rim fracture displaced out of scalp: a case report.

Isolated fractures of the supraorbital rim are rarely seen. In this report, an isolated supraorbital rim fracture is presented in which the segment of the rim was completely removed from its location onto the frontal bone in a traffic accident. It was thought to be a foreign body and was delivered outside the scalp in the emergency service. It was replaced 24 hours after the trauma, and the patient healed without any problem.

Child, Preschool↗
PubMed2004

Correction of facial deformity using a Red III device in a patient with Antley-Bixler syndrome.

Antley-Bixler syndrome was first described in 1975 and to date at least 44 cases have been reported. In addition to brachycephaly, this syndrome is associated with midface hypoplasia, bilateral radiohumeral synostosis, multiple joint contractures, long bone fractures, dysplastic ears, "pear shaped" nose, and occasionally urogenital or cardiac defects. The authors report a case in a 23-year-old man with severe midface deficiency treated using distraction osteogenesis. A modified Le Fort III osteotomy was performed, and nasal subunit and remaining Le Fort III segment was distracted separately. The classic RED II design was found to be insufficient, so the authors added an extra horizontal bar to the system and converted it to a RED III design. Midface advancement was 11 mm from the porion to the orbitale and 31 mm at point A. Nasal advancement at the nasal root level was 5 mm. There were no complications, and at 4 months after surgery, advancement was stable without any relapse.

Abnormalities, Multiple↗
PubMed2004

Solvent-dehydrated calvarial allografts in craniofacial surgery.

Craniofacial surgery almost always requires the use of bone grafting. Although autografts are the standard procedure for bone grafting, it is sometimes not possible to harvest bone, and autografts have particular risks. The use of allograft bone provides a reasonable alternative to meet the need for graft material. Solvent dehydration is a multistage procedure in which human cadaveric bone is processed by osmotic exchange baths and gamma sterilization. This processing avoids the risk of infection transmission, decreases antigenicity, and does not weaken the mechanical properties of the bone. Solvent-dehydrated, gamma-irradiated human calvarial bone allografts were used for reconstruction of craniofacial deformities in 24 patients between 1988 and 2002. Resorption of the allografts and results of the surgical intervention were evaluated with plain radiographs and three-dimensional computed tomography 12 months after surgery, in 21 patients. Serologic tests for human immunodeficiency virus-1 antibody, hepatitis B surface antigen, and hepatitis C antigen were also performed. Biopsy specimens were taken from the allografts. Average follow-up in this group was 30 months (range, 8 to 60 months), and results of serologic tests were negative in all patients. Seventy-one percent of the patients (15 of 21) showed no resorption, with partial and complete allograft fusion. One patient had nearly total graft loss and the remaining five patients had 10 to 25 percent graft resorption. Rigid fixation of the allograft, contact with the dura and periosteum, and prevention of dead spaces around the allograft are the most important factors in achieving a satisfactory result. In solvent-dehydrated bone allografts, sterilization and antigenic tissue cleaning are achieved after several steps with a minimal dose of radiation. The result is a nonantigenic, sterile mechanical scaffold that can tolerate external forces. Although autografts are the standard in craniofacial surgery, solvent-dehydrated calvarial bone allografts produced successful results in selected cases.

Adolescent↗
PubMed2003

Rigid external distraction of the midface with direct wiring of the distraction unit in patients with craniofacial dysplasia.

The development of craniofacial distraction techniques represents a significant advancement in the management of craniofacial dysplasias. For distraction of the midface after Le Fort III osteotomy, two levels of anchorage to the moving segments are necessary. In this report, the authors connected the distraction forces directly to the infraorbital rims, using a surgical wire for superior anchorage. They performed this technique in four patients with craniofacial dysplasia for the management of severe maxillary hypoplasia using RED II system. This configuration not only simplified the placement of the system but also made the removal of the device much easier. The authors did not have any problems with the wire traction unit, such as wire breakdown or bony tunnel disruption. It is a cheap, simple, and effective configuration that can be easily performed without any complication.

Bone Wires↗
PubMed2003

[Role of three dimensional spiral CT in the evaluation of craniofacial abnormalities in pediatric patients].

Technological advances in computer image analysis have made possible three-dimensional surface reformations of anatomic structures from contiguous axial CT silices. Three-dimensional spiral CT is a relatively new method and it is very useful in the diagnosis and management of craniofacial abnormalities. The purpose of this pictorial review is to assess the role of three-dimensional spiral CT in the evaluation of craniofacial abnormalities including craniosynostosis, hypertelorism, frontonasal dysplasia, hypoplasia of mandible, encephalocele and trauma in paediatric patients.

Child↗
PubMed2002

Hereditary intraosseous vascular malformation of the craniofacial region: an apparently novel disorder.

Primary intraosseous vascular anomaly, previously called intraosseous hemangioma, is a very rare malformation that is usually seen in the vertebral column and in the skull. It is exclusively described in sporadic cases and no hereditary component has yet been reported. The most commonly affected bones in the skull are the mandible and the maxilla, and life-threatening bleeding after a simple tooth extraction is frequently observed. Here, we report two consanguineous families containing a total of four affected patients manifesting primary intraosseous vascular malformation (VMOS (vascular malformation osseous)) of the craniofacial region. The phenotypic expression is remarkably similar in both families. The characteristic findings include severe blood vessel expansions within the craniofacial bones and midline abnormalities such as diastasis recti, supraumbilical raphe, and hiatus hernia. Malformation is restricted to the mandibular and maxillary area in the prepubertal age, and rapid expansion starts after age 12 or 13. A 15-year follow-up of one of the patients demonstrated that the vascular malformation did not extend beyond the craniofacial region despite severe involvement of almost all bones in the skull. Detailed clinical and radiological evaluation provided neither evidence of soft-tissue involvement nor any sign of gross arterial, venous, or combined malformations, indicating that bone changes are a primary rather than a secondary effect due to any other vascular anomaly in the craniofacial region. An antibody against a universal proliferation marker, Ki-67, detected nonproliferative, single-layered endothelial cells, suggesting that this abnormality is a vascular malformation rather than a hemangioma. alpha-actin staining (antibody against perivascular tissue such as smooth muscle cells (SMCs) and/or pericytes) demonstrated that pathologic vessels lost their surrounding supportive tissues, as was previously seen in other types of vascular anomaly. Homozygosity mapping excluded the following loci and/or genes: multiple cutaneous venous malformation (VMCM1; gene, TIE2) on chromosome 9p21; venous malformation with glomus cells (VMGLOM) on chromosome 1p22-p21; hereditary hemorrhagic telangiectasia type 1 (HHT1; gene, endoglin) and type 2 (HHT2; gene, activin) on chromosomes 9q34.1 and 12q11-q14, respectively; and cerebral cavernous malformation type 1 (CCM1; gene, KRIT1), type 2 (CCM2), and type 3 (CCM3) on chromosomes 7q11.2-q21, 7p15-p13, and 3q35.2-q27, respectively. To the best of our knowledge, this is a new disorder, which we call hereditary intraosseous vascular malformation of the craniofacial region.

Antigens, CD34↗
PubMed2002

Tissue response to N-butyl-2-cyanoacrylate after percutaneous injection into cutaneous vascular lesions.

Despite the fact that cyanoacrylates, a group of rapidly polymerizing adhesives, are used widely in general surgery, neuroradiology, otolaryngology, and plastic surgery, scientific data on histopathological changes resulting from the deposition of -butyl-2-cyanoacrylate (NBCA), a new-generation cyanoacrylate derivative, in human tissues is based largely on experimental observations in animals and sporadic postmortem studies in humans. The authors report the consecutive pathological findings of a patient who underwent surgery for facial hemangioma after percutaneous injection of NBCA for devascularization of a lesion, and underwent additional surgery 1 and 6 months after the initial operation for the removal of the residual NBCA cast from the injection site. Acute inflammatory findings after injection of NBCA and the development of a chronic granulomatous foreign body reaction support the histological findings of experimental animal studies and postmortem examinations on humans. Additionally, their findings support the proposed hypothetical sequence of events for the recanalization of cyanoacrylate-embolized vascular structures.

Arteriovenous Malformations↗
PubMed2002

Reinnervation of denervated muscle in a split-nerve transfer model.

This study was performed to quantify the reinnervation of denervated muscle in a split-nerve transfer model and to determine any possible downgrading effects on the donor nerve and its end organ. Fifty-four adult Wistar rats weighing 200 to 250 g were used. The experimental design consisted of two groups. The motor nerve branch to the anterior tibial muscle and gastrocnemius muscle of the right hind limb were dissected in all rats. In the experimental group (N = 36), the motor nerve branch of the tibial nerve to the gastrocnemius muscle was exposed, cut, and ligated. The motor nerve branch to the anterior tibial muscle was split and transected longitudinally, and the medial half was routed posteriorly. End-to-end neural anastomosis was performed between this medial half of the split nerve and the distal stump of the gastrocnemius nerve. In the control group (N = 18), while the same surgical preparation was performed, the motor nerve branch to the anterior tibial muscle and gastrocnemius nerve were exposed and transected, and the nerve endings were ligated, but neural anastomosis was not performed between these nerves. The left hind limb of all rats served as a normal comparison side without any surgical intervention. Both of the groups were divided into three subgroups (12 rats each for the experimental groups and 6 rats each for the control group) to evaluate the results after periods of 1, 3, and 6 months. Electromyography, light microscopic and morphometric examination, and muscle weight measurements were used to document the results. Although stimulation of the peroneal and tibial nerves did not produce any compound muscle action potential (CMAP) recordings from either the anterior tibial or the gastrocnemius muscle in the control group, the normalized CMAP areas of the tibial nerve were (mean +/- standard deviation) 16.2 +/- 30.8% in the 1-month group, 63.4 +/- 34.7% in the 3-month group, and 72.4 +/- 16.3% in the 6-month group. For the peroneal nerve, the normalized CMAP areas were 17.0 +/- 32.2%, 53.4 +/- 29.4%, and 54.4 +/- 14.5% for the 1-, 3-, and 6-month groups in the experimental groups respectively. A high number of regenerating myelinated nerve fibers was identified in the distal part of the coapted motor nerve branch to the gastrocnemius muscle. The average number of myelinated fibers in the lateral half of the split nerve in the experimental group was 15,108 fibers per square millimeter, 14,167 fibers per square millimeter, and 19,830 fibers per square millimeter at months 1, 3, and 6 respectively. The average number of fibers proximal to the nerve anastomotic site was 15,423 fibers per square millimeter, 19,200 fibers per square millimeter, and 20,774 fibers per square millimeter. Distal to the nerve anastomotic site, the number of myelinated fibers was 17,941 fibers per square millimeter, 18,885 fibers per square millimeter, and 18,895 fibers per square millimeter at 1, 3, and 6 months respectively. There were no myelinated fibers in the control group sections. There were significant differences in muscle weight between the experimental and control groups at the end of month 6. The difference between the experimental side and the untouched normal healthy side was not significant in the weight measurements of both muscles. The results show acceptable reinnervation by split-nerve transfer with minimal functional impairment of the donor muscle. This study confirms that split-nerve transfer is a reliable method of reconstruction for paralyzed muscle with minimal donor area morbidity.

Animals↗
PubMed2002

Effects of growth factors on doxorubicin-induced skin necrosis: documentation of histomorphological alterations and early treatment by GM-CSF and G-CSF.

Extravasation of vesicant antineoplastic agents such as doxorubicin into the skin or subcutaneous tissues may result in loss of the full thickness of the skin or underlying structures. Several treatment methods have been advocated but none has demonstrated any superiority to the others. The authors designed a controlled animal study in 88 rats to test three methods of early treatment of extravasation of the vesicant antineoplastic agent doxorubicin. The first step of the study included 48 Sprague-Dawley rats. All animals received intradermal injections of 1 mg doxorubicin superficially to the panniculus carnosus in the dorsum. The rats were then divided into four groups of 12 rats each, as follows: group 1, no treatment; group 2, immediate intradermal injection of 0.1 ml saline to the same site; group 3, immediate intradermal injection of 10 microg granulocyte macrophage-colony stimulating factor (GM-CSF) in 0.1 ml saline to the same site; group 4, immediate intradermal injection of 10 microg granulocyte-colony stimulating factor (G-CSF) in 0.1 ml saline to the same site. During the next 6 weeks the rats were observed for the development of necrosis. Ulcers developed and reached maximum size two weeks after the injections. The largest ulcers according to area were observed in group 1 and the mean value was 21.25 mm (p < 0.05). Although wound areas were significantly smaller in the saline group than in the control group and the mean value was 7.58 mm (p < 0.05), the smallest lesions were observed in groups 3 and 4, and the mean values were 1.08 mm and 0.83 mm respectively (p < 0.05). There was statistically no difference with regard to mean ulcer area between groups 3 and 4. During the second step of the experiment, the remaining 40 Sprague-Dawley rats were used. Groups containing 10 rats each were designed similarly after all animals received intradermal injections of 1 mg doxorubicin into the back. On the 10th day after the injection, the entire area of the ulcer together with the underlying panniculus carnosus was excised for pathological examination and for determination of glucose 6-phosphate dehydrogenase (G6PD) activity. On microscopic examination, the extravasated ulcer consisted of a large area of ischemic necrosis. There was marked damage to small blood vessels in the form of fibrinoid necrosis and vasculitis. Injured vessel counts were higher in the control group (group 1; p < 0.05). No difference was observed in G6PD activity between the groups. The authors conclude that both saline and tissue growth factors (GM-CSF and G-CSF) are useful for the early treatment of doxorubicin extravasation; however, GM-CSF and G-CSF are more beneficial.

Analysis of Variance↗
PubMedDate not supplied

A new experimental hypertrophic scar model in guinea pigs.

Many aspects of the biology and effective therapy of proliferative scars remain undefined, in part due to a lack of an accurate, practical, reproducible, and economical animal model for systematically studying hypertrophic scars. This study was designed to investigate whether hypertrophic scar formation could be induced in guinea pigs by removal of the panniculus carnosus alone, and by a combination of the removal of the panniculus carnosus with application of coal tar afterwards. Whole thickness skin excision or deep partial thickness injury was used to create the lesions on intact skin. Different anatomic locations were tested in different groups. Scars thus developed were examined morphologically by light microscopy and electron microscopy (TEM and SEM) and biochemically by measuring the activity of glucose-6-phosphate dehydrogenase (G6PD) to check whether these scars had morphological and biochemical properties specific to hypertrophic scars. The albino guinea pigs used in this study were divided into three groups. Removal of the panniculus carnosus was performed from the ventral aspect of the torso in animals in groups I and II. On the skin overlying the area of panniculectomy, circular skin excision was performed in group I, and deep partial thickness burn injury was inflicted in group II, to see whether wounds would heal with hypertrophic scars. In group III, dorsal aspect of the torso were used and wounds were produced by circular skin excisions followed by panniculectomy on both sides but coal tar was applied to only one side. Tissue samples were taken from the scars that were hypertrophic in appearance, and from normal scars and normal skin for comparison. Light and electron microscopic examinations and G6PD activity measurements were performed on these samples. While hypertrophic scar development was not seen in group I and group II, scars with morphological and biochemical properties specific to hypertrophic scars developed in one third of animals in group III after healing of the wounds treated with coal tar. In conclusion, it is shown that it is possible to develop experimental hypertrophic scars in guinea pigs with morphological and biochemical properties similar to those of human proliferative scars. Therefore this model is a new, practical, and economical experimental animal model to study proliferative scars, although improvements are needed to increase yield.

Animals↗
PubMedDate not supplied

Simultaneous open rhinoplasty and alar base excision for secondary cases.

BACKGROUND: Simultaneous open rhinoplasty and alar base excision are a very safe procedure for protecting the vascular supply of the nasal dip and the columellar skin in primary cases when surgical dissection is performed below the musculoaponeurotic layer of the nose. Major arteries of the external nose lie above the musculoaponeurotic layer. However, secondary cases may pose increased risks to the blood supply of the nasal tip and columella skin because of the decreased vascular supply and increased scar tissue from the previous rhinoplasty. We studied our secondary cases of simultaneous open rhinoplasty and alar base excision, to assess the real risk for necrosis of the nasal tip and columellar skin. METHODS: A total of 12 secondary patients (6 men and 6 women) underwent simultaneous open rhinoplasty and alar base excision in the past 3 years. Their average age was 27 years (range, 21-35 years). The average follow-up period was 15 months (range, 1-35 moths). A modified grading system, originally described by Bafaqeeh and Al-Qattan, was used for assessment of the blood supply in the nasal tip and the columellar skin. RESULTS: Satisfactory results were obtained for our patients, with the exception of one case. Grade 3 vascular compromise to the nasal tip and the columella was observed in one case, but the patient healed well with wound care treatment. CONCLUSION: Simultaneous alar base excision and open rhinoplasty can be performed safely in secondary cases. However some surgical maneuvers such as subcutaneous pocket preparation for the tip graft in closed rhinoplasty and subdermal defatting in the first rhinoplasty as well as previous scarring on the nasal lobule can disrupt the vascular supply of the nasal tip and columella skin. Under these conditions, alar base excision should be deferred and then performed as an isolated excision procedure.

Adult↗