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[Behavioral response to an acute pain in newborn children with surgical diseases].

The article is dedicated to studying the behavioral response to an acute pain in the newborns with surgical pathologies. The videotaped facial activity (a total of 12 parameters), the general locomotive activity (6 parameters) and cry were studied in 93 newborn children with different surgical pathologies. It was found that the newborn children response differentially to the painful and non-painful stimuli. A combination of 6 signs, i.e. frowning eyebrows, tight eyes, aggravated nasolabial folds, open mouth, and motion of foot, was detected in 93.5% of cases (87 of the 93 children) as a response to a painful stimulus. Such behavior indicates the presence of the painful syndrome in the newborn with a sensitivity of 93.5% and with a specificity of 78.5%. The combination of these signs can signify a characteristic painful behavior in the newborn children and it can be used as a criterion of the painful syndrome in the neonatal period.

Acute Disease↗

[Endoscope-assisted supraorbital keyhole approach for excision of suprasellar region tumor].

OBJECTIVE: To study endoscope-assisted microsurgical technique with supraorbital keyhole approach for the treatment of suprasellar region tumor so as to maximize tumor removal and minimize operative-trauma. METHODS: According to high resolution CT and MR images before operation, individual operation schemes were worked out. Skin incision was made in the eyebrow, and the diameter of supraorbital craniotomy was approximately 2 cm. Endoscope-assisted microsurgical technique was used to resect lesions in 16 consecutive patients. RESULTS: Total Tumors were removed in all of the 16 patients via the supraorbital keyhole approach. No postoperative complications occurred. CONCLUSIONS: Supraorbital keyhole approach may diminish tissue injury considerably and has proven to provide sufficient operating space in the suprasellar region for tumor removal.

Adolescent↗

[Orbicularis oculi muscle and crow's feet. Pathogenesis and surgical approach].

Crow's feet is one of the characteristic signs of the aging face. Minor techniques designed to obliterate these lesions rapidly demonstrate their limits. The orbital fibers of the orbicularis oculi muscle play a fundamental role in the pathogenesis of wrinkles. Their contracture will create wrinkles in a similar way to the spokes of a wheel perpendicularly to the orientation of the muscle fibers. The dynamic action of the zygomatic muscles contributes to exaggerate these wrinkles. Statically, ptosis of the lateral brow and the aging skin also contributes to these lesions. Surgical treatment can be accomplished by a temporal lift or via a blepharoplasty approach. The vertical fibers of the orbicularis oculi muscle must be corrected by muscle incision or resection, muscle incision and suspension, muscle redraping and fixation, covering of the orbicularis oculi muscle with the malar SMAS. In patients with ptosis of the tail of the eyebrow one must either do a forehead lift or a mask-lift that will redrape the skin. The surface of the skin may require peeling by dermabrasion. Of course, these surgical techniques require a detailed knowledge of the anatomy of this region and especially the distribution of the branches of the facial nerve. The author analyses and compares the results of the different techniques.

Esthetics↗

Actinic prurigo, tropical (South-East Asian) variant.

Actinic prurigo is a chronic familial photodermatitis found predominantly among the Amerindians. It has been reported from North and South America, Britain and Japan. We report a case of actinic prurigo seen in Singapore. A 20-year-old Malay female presented with a persistent pruriginous eruption in the sun-exposed parts and on her abdomen. She also had lower lip cheilitis and thinning of the outer eyebrows, features often seen in actinic prurigo. The minimal erythema dose to ultraviolet A (UVA) and UVB were persistently lowered. We propose that this condition be called actinic prurigo, tropical (South-East Asian) variant.

Adult↗

[Surgical treatment of the pneumosinus dilatans of the frontal sinus (author's transl)].

Two cases of pneumosinus dilatans of the frontal sinus with cosmetic deformation are reported. In both cases one big hole without any interfrontal septum has been found. In the first case no efferent ducts of the frontal sinus have been visible, the second one showed only one. Surgical treatment by incision from ear to ear, and forming of a scalp flap with opening of the frontal sinus in the upper margin of the bony bulging. This bulging was left pedicled on the periosteum and fractured in many pieces. Forming of an artificial efferent duct to the nose by an incision below the left eyebrow. Good cosmetic results in both cases. X-rays fotos, pictures of the operation and postoperative results are demonstrated. Examinations of the human growth hormones (HGH) of the 24-years-old German and the 28-years-old Turkish man presented normal values. The author believes in a dysregulation of the efferent ducts.

Adult↗

[Remission of schizophreniform psychosis after brain tumor surgery].

A 19-year-old female was admitted to hospital due to a schizophrenia-like psychosis of the paranoid type including delusions and various hallucinations. Neurologically she only showed tics of the eyebrows with increased eye blinking. 30 months before an astrocytoma located on the left basal temporal lobe had been resected after the patient suffered from several psychomotor and two grand mal seizures. Following post-operative anticonvulsant therapy seizures had completely disappeared and the patient had been free of symptoms of any kind. After the acute onset of the psychosis another follow-up MRI of the brain using coronary sections revealed a small relapse-tumor. Symptoms disappeared after high-dose neuroleptic therapy. Finally another surgical intervention led to a lasting remission of the psychotic symptomatology (so far 18 months). Postoperatively neuroleptics could be discontinued. Clinical picture and MRI findings will be discussed with a focus on possible etiological factors in schizophrenia.

Adult↗

[Peri-orbital surgical approaches].

After having made a definitive diagnosis in patients with orbito-frontal pathology, the choice of incision is an important factor to achieve good intra-operative exposure of the region, as well as to obtain a good functional and aesthetic result. In the majority of cases we prefer to utilize the incisions commonly used for blepharoplasty, especially the transconjunctival approach popularized by Tessier. Other more classic accesses such as the superolateral, infra-orbital and intra-oral incisions, are reserved for treatment of cases of orbitomalar (communition) fractures. We feel there are limited indications for use of incisions through the eyebrows with medial extensions for exposure of isolated lesions of the medial orbital wall, orbital roof, and the anterior wall of the frontal sinus. However they may become very useful as the primary incisions in the treatment of open fractures of the orbital region. The bicoronal incision, a classical approach in neurosurgery, is also being used by us in cases of tumors and complicated middle face fractures which appear to extend to the orbito-frontobasal region in 40% of our patients. In addition it provides excellent access for post-traumatic reconstruction of orbito-frontal bone defects and impacted fractures. A combination of different incisions: bicoronal, peri-orbital and intra-oral, may be indicated for the correction of syndromal anomalies such as Treacher-Collins, for treatment of complicated facial fractures, or for pure aesthetic surgery procedures, such as the "mask-lift" introduced by Tessier.

Humans↗

A Turkish case of subcortical/subependymal heterotopia associated with corpus callosum dysgenesis, craniofacial dysmorphism, severe eye abnormalities, and growth-mental retardation.

The patient is a 12-year-old boy with a history of learning disability, growth retardation, and strabismus. Weight, height and head circumference were below the 3rd percentile. A café-au-lait spot, 1x1 cm a diameter, on the back region and pectus excavatum deformity were diagnosed. He had facial asymmetry, a broad nose, sparse eyebrows and eyelashes, a rudimentary frontal sinus, deviation of the nasal septum, and bilateral small maxillary bones. The left orbital fossa was also mildly rudimentary. On eye examination the movements of the left globe to the upward and lateral side were limited and internal strabismus was noted at this side. Visual acuity was 1/10, bilaterally. Bilateral choroid coloboma, glaucoma, vertical and horizontal nystagmus were diagnosed. Fundoscopic examination revealed bilateral optic atrophy and macular and paramacular granulation tissues on the left side. Intelligence quotient was 46. Electroencephalography revealed bilateral frontal slow-wave activity. Visual evoked potential revealed prolonged p100 wave latencies bilaterally. Magnetic resonance imaging of the brain demonstrated corpus callosum dysgenesis, bilateral subcortical heterotopia in the frontal lobes and subependymal heterotopia in the posterior horn of the left ventricle. Chromosomal analysis revealed a normal male karyotype, 46, XY. Although several cases of heterotopia in association with mental retardation, craniofacial dysmorphism, cerebral, and eye abnormalities have been described the combination of abnormalities diagnosed in our case has not previously been reported. We hypothesize that the combination of subcortical/subependymal heterotopia, corpus callosum dysgenesis, craniofacial dysmorphism, severe eye abnormalities, and growth-mental retardation may be a new syndrome.

Abnormalities, Multiple↗

Mixing politics with pleasure. An AHA vice president organizes a fund-raiser for the Bush-Cheney campaign. Does this constitute a conflict of interest?

An American Hospital Association lobbyist's fund-raiser for the Bush-Cheney re-election campaign last week at Washington's Capitol Hill Club raised eyebrows, along with questions about the gray area between personal advocacy and the industry's political agenda. The AHA was not involved in the event, association officials say, but some observers remain skeptical.

American Hospital Association↗

Interstitial deletion of the proximal region of the long arm of chromosome 18, del(18q12) a distinct clinical entity? A report of two new cases.

Two unrelated mentally retarded patients were found to have an interstitial deletion of 18q12. They were a 2-year-old, short, macrocephalic and autistic girl, and a 5-year-old boy. Six other liveborn patients with comparable deletion have been so far identified. The common findings are mild dysmorphic features (telecanthus, epicanthal folds, flaring eyebrows, small mouth with thin upper lip), hypotonia, behavioural disorders, mental retardation with speech delay and lack of major malformation.

Child, Preschool↗

Phenotypic variability of mannosidosis type II: report of two Greek siblings.

Two patients, a 13-year-old boy and his 24-year-old sister, were diagnosed as mannosidosis type II cases, on the basis of both presenting extremely reduced plasma and white blood cell acid-alpha-mannosidase are reported. With the exception of mental retardation and neurosensory deafness the two siblings manifested a wide phenotypic variability. The boy had several facial features indicating a lysosomal storage disorder, as well as spondylolisthesis. His sister, apart from heavy eyebrows and lower jaw prognathism appeared normal.

Adolescent↗

[KID syndrome: a cause of pachydermatoglyphia].

INTRODUCTION: The KID syndrome is a rare ectodermal dysplasia associating erythrokeratodermia, deafness and keratitis. Other disorders such as sensitivity to infections or hypohidrosis may be associated. Pachydermatoglyphia is characterized by diffuse hyperkeratosis in which the dermatoglyphe crests are accentuated in thickness and height. CASE REPORT: We report the case of a KID syndrome in a young girl, born of non-consanguine parents and without any familial context. The classical elements of this dysplasia were present: erythrokeratoderma with dryness and roughness of the whole tegument, plicatured pachydermia of the knees, facial erythema, grooves around the mouth, hypotrichosis of the lashes and eyebrows, deafness, and ophthalmologic involvement. Involvement of the palms was characterized by the pachydermatoglyphic aspect. DISCUSSION: In this case report, the palmar keratoderma corresponded to the original description of pachydermatoglyphia and we consider that the KID syndrome should be integrated as a possible etiology of pachydermatoglyphia.

Child↗

Trichophytosis by double infection of Trichophyton schoenleinii and Trichophyton violaceum.

Chronic generalized trichophytosis in a 44-year-old female is reported. The disease began when she was 15 and gradually spread from the scalp. Surviving hairs on the scalp and eyebrows showed an endothrix invasion. Enlargement and fistulization of the cervical and axillary lymph nodes were also present. Trichophyton schoenleinii was cultured from the scalp while Trichophyton violaceum was isolated from the body skin, the nail and lymph nodes. Griseofulvin produced significant improvement with occasional relapses. The possible role of the basic cell-mediated immune defect, as revealed by the decreased number of the T and B cells, is discussed.

Adult↗

The clinical significance of the branching pattern of the facial nerve in Malaysian subjects.

Fine dissection was carried out in 79 facial halves from formalin fixed Malaysian adult cadavers of various races, to trace the extracranial part of the facial nerve and its peripheral branches. The facial nerve trunk, after leaving the stylomastoid foramen was located at a depth of 1 to 2 cm from the skin in the vagino-mastoid angle. It bifurcates at the posterior border of the ramus of the mandible and in 3.8% trifurcation was found. Mean distance of bifurcation from the angle of the mandible was 28.06 mm and 81.0% were within the range of 21 to 35 mm. The branching patterns were classified into six types, and the frequency of occurrence was type I 11.39%, type II 15.9%, type III 34.18%, type IV 18.98%, type V 7.59% and type VI 12.67%. Type I, a classical text book pattern was found to be one of the least common patterns. There is no significant difference in percentage of each type between the present study and that of Koreans, though some differences with Caucasians were noted in three uncommon types. The frontal branch could be outlined between the two diverging lines from the earlobe to the lateral ends of the eyebrow and the highest frontal crease. Posterior to the facial artery, the mandibular branch was seen passing below the inferior border of the mandible in 20%; anterior to the artery, this nerve divides into one to four branches. In almost all the cases, branches to the mentalis and the depressor labii inferioris muscles and infrequently branches to the depressor angular oris were seen below the inferior border of the mandible.(ABSTRACT TRUNCATED AT 250 WORDS)

Asian People↗

[Congenital anhidrotic ectodermal dysplasia in a female infant].

Fever of unknown origin, unability to sweat, hypotrichosis, absent eyebrows and thick everted lips were symptoms in a 3 month old female infant and raised the suspicion of anhidrotic ectodermal dysplasia. After several days of high fever and enteritis our patient presented with convulsions which could hardly be interrupted for a couple of days. Two months later brain scanning revealed serious brain damage. Finally the diagnosis of anhidrotic ectodermal dysplasia was confirmed by the unability to sweat by means of the quantitative pilocarpin iontophoresis test. Skin biopsy showed absence of the eccrine glands.

Biopsy↗

[Neuroendocrine tumors of visual system--Merkel cell carcinoma].

PURPOSE: To describe cases of Merkel cell carcinoma. Belonging to the APUD-system tumors, the highly malignant Merkel cell carcinoma affects in 10% the ocular adnexes, 50-60% of the patients develop a metastasis to the lymph nodes, and the five year survival rate is only 38%. 30% of the tumors recur after one year. MATERIAL AND METHODS: In the last eight years the Merkel cell carcinoma was diagnosed in four patients and was treated by wide resection, radiotherapy and cytostatic drugs. Histological and immunohistological examination was performed. RESULTS: In three cases the carcinoma was diagnosed in the upper lid and in one case in the eyebrow. Two patients could not be cured. Characteristic is clinical uniform appearance of the tumor as painless, reddish nodule with smooth surface, telangiectatic blood vessels, fast growing and fast leading to metastasis. Furthermore, the histological characteristics of the tumor were found as well as NSE, S100 proteins and neuroendocrine granula, which allow to classify to the APUD--system and to distinguish from the more benign tumors. CONCLUSIONS: As neuroendocrine tumor the Merkel cell carcinoma represents a high malignant tumor in ophthalmology. The certain diagnosis is only made by histological and immunohistological examination. A full-thickness resection followed by radiation, should be performed as soon as possible.

Aged↗

Hypertrichosis, hyperkeratosis, abnormal corpus callosum, mental retardation and dysmorphic features in three unrelated females.

We report three unrelated patients with hypertrichosis, mild to moderate mental retardation, and dysmorphic facial features including low anterior hairline, thick arched eyebrows, nose with broad tip and columella below alae nasi, short philtrum, thick drooping lower lip and simple posteriorly rotated ears. They also had rough skin with hyperkeratotic plaques. Feet and finger tips were broad. All of them had personality problems like aggressiveness, stubborn temperament or tendency to withdraw. Brain MRI showed thick and short corpus callosum. We believe that these patients represent a new syndrome of unknown aetiology.

Adolescent↗

[Sebaceous gland carcinoma of the eyelids].

Sebaceous gland carcinoma is considered a relatively rare tumor and represents approximately 1 and 5% of all eyelid tumors. These tumors usually arise in the Meibomian glands of tarsus, in the Zeis glands, and the sebaceous glands of caruncle or eyebrow. In some instances it had multicentric origin, while in other cases the initial location was unable to be determined. It occurs more frequently in women than in men and presentation age varies from 50 to 90 years. Clinical diagnosis is difficult because is can be confused with chalazion, blepharoconjunctivitis, conjunctivitis, and basocellular carcinoma. Sebaceous gland carcinoma could go unnoticed; thus, it is necessary to carry out excision of conjunctiva as well as to inform the pathologist of the possibility of malignancy, because pagetoide spread can be easily incorrectly interpreted or not observed even by expert pathologists (up to 50%). Five-years mortality has been estimated at 30% due delayed diagnosis and treatment. The present work intends to describe incidence, and clinical and histopathologic presentation of sebaceous gland carcinoma during the last 40 years at the Dr. Luis Sanchez Bulnes Hospital, Association to Prevent Blindness in Mexico, as well as to review treatment modalities forecast for this entity.

Diagnosis, Differential↗