[Development of "harelip surgery" up to the end of the 18th century].
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Cleft Lip↗
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[11-year old Anna with harelip "was surgically treated according to curent practice"].
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Child↗
Congenital respiratory tract defects in lambs.
One hundred and forty-seven newborn lambs with congenital defects of the respiratory tract were examined during a 3-year investigation into the causes of perinatal lamb mortality. Respiratory defects were found in 3.3% of the 4,417 lambs examined and in one-third (36.6%) of the 401 congenitally malformed lambs. All the defects were secondary to other more extensive malformations. The upper respiratory tract was involved 3 times more than the lower tract. Males were involved more than females (4:3). The upper tract defects were harelip, cleft palate, atelorrhinia, arrhinia, proboscis, fused nostrils and choanal atresia. One of the facial clefts was a rare median harelip associated with cleft palate and fused nostrils. The lower tract defects were pulmonary agenesis, hypoplasia and duplication. The 2 most common defects were choanal atresia and secondary pulmonary hypoplasia. It was concluded that primary defects of the respiratory tract of sheep are rare and that secondary defects are relatively common.
Abnormalities, Multiple↗
[The functional and surgical rehabilitative treatment of cleft lip and cleft palate in a case of the syndrome of the deletion of the long arm of chromosome 18 diagnosed in utero].
Following an introductory illustration of the clinical characteristics of the 18q syndrome (De Grouchy syndrome), the paper describes the treatment carried out in a young patient with harelip and cleft palate in addition to chromosomopathy. Although the facial malformation and the karyotype with the deletion of a portion of the long arm of chromosome 18 were diagnosed in utero, the parents, having been correctly informed of the mental retardation usually associated with this syndrome and the possibility of surgical correction of the facial malformation, decided not to abort. The case offers an occasion for analysing the various stages and results obtained to date using a multi-specialised diagnostic approach and functional and surgical treatment of the harelip and cleft palate which have enabled much of the congenital handicap to be corrected.
Abnormalities, Multiple↗
[VDT pulse magnetic field enhances teratogenic effect of ara-c in mice].
Impregnated NIH mice were irradiated 4 hrs daily on days 16th-17th of gestation with 15.6 kHz pulse magnetic field at 40 microT peak to peak. Significant enhancement of ara-c induced teratogenic potency by the magnetic field can be observed. The incidence of harelip fetuses bearing harelip and/or cleft palate was significantly higher in the combined exposure group, in which mice were exposed to the magnetic field and given an intraperitoneal injection of ara-c on the 9th day of gestation, than that of mice treated with ara-c alone.
Abnormalities, Drug-Induced↗
Pediatric surgery in Islamic medicine from the Middle Ages to the Renaissance.
Albucasis lived throughout the tenth century, practicing medicine for more than 40 years in Zahra at the outskirts of Cordoba. This was the period of the greatest splendour of Arab domination of Spain. Albucasis taught medicine at the university of Cordoba and published an encyclopedia of medicine comprising 30 volumes, the last one dealing with surgery. This work was translated into all civilized languages and was the standard medical textbook right till the eighteenth century. This paper deals with many pediatric surgical conditions as described in Albucasis' 30th volume. The pathology and treatment of harelip, hydrocephalus, the adenoids, ranula, gynecomastia, imperforated external urinary meatus, circumcision, hermaphrodites, imperforate anus, and supernumerary and webbed finger were all described by Albucasis, and his remarks are discussed here in some detail.
General Surgery↗
Membrane characterization of cultured human keratinocytes by freeze-fracture electron microscopy.
Human skin explants obtained from 2 to 5-year-old patients with harelips were cultured in NCTC 168 medium at 37 degrees C, in a humidified atmosphere of 5% CO2 in air. After a 2-week incubation period, plasma membranes of the newly grown cells were characterized by freeze-fracture electron microscopy. Desmosomal diameter in cultured keratinocytes was much smaller, ranging from 0.08 to 0.19 micron, than that in vivo, where it ranged from 0.3 to 0.7 micron in diameter. On the E-face of the plasma membranes, attached to the bottom of the culture dish, small particle aggregations were observed. These were thought to be half-desmosomes, each consisting of 10 to 30 particles. Small gap junctions were also observed. These ranged in size from 0.05 to 0.1 micron in diameter. Membranous structures were found adhering to the plasma membrane from the intercellular spaces. These membranous structures may be lipid vesicles, since they are similar in freeze-fracture electron-microscopic features to lamellar lipid structures seen in the intercellular spaces of horny cells in vivo.
Cell Membrane↗
Partial distal trisomy 13q resulting from familial reciprocal 5/13 translocation.
A female infant with partial trisomy 13, 46,XX,der(5),t(5;13) (p15;q22)mat, for the distal part of the long arm is reported. The clinical and autopsy findings were similar to those of complete trisomy 13, except for harelip and cleft palate, and sloping forehead. Fetal hemoglobin and nuclear appendages in polymorphonuclear leukocytes were normal. Loci for these traits are discussed.
Abnormalities, Multiple↗
[Early contributions from Erlangen to the theory and practice of ether and chloroform anesthesia. 1. Heyfelder's clinical trial with ether and chloroform].
The era of modern anaesthesia in Germany began on January 24th, 1847. This day, professor in ordinary Johann Ferdinand Heyfelder anaesthetized a patient with sulphuric ether in the clinic of surgery and ophthalmology of the University of Erlangen. By March 17th, 1847, Heyfelder had performed 121 surgical procedures under ether. The operations in majority were teeth-extractions, and a few more complex operations such as the treatment of a harelip or of lip cancer or the resection of the shoulder joint. Heyfelder described in detail 108 of these inhalations in a little book entitled The experiments with sulphuric ether. This monograph published in March, 1847, represents one of the first complete dissertations on sulphuric ether in the German literature. In a special chapter he analyzed the development of various physiological and psychological parameters during etherization. Heyfelder also examined blood and urine of some etherized patients and reported that he did not find any important or specific alterations. In 1847, Heyfelder was probably the first to apply salt-ether in man. After 4 administrations he concluded that salt ether acted more quickly but shorter than sulphuric ether. Advantageous were its application without problems and ease of induction. Disadvantageous were its high volatility, its price and the difficulty of getting it in a pure form. From December, 1847, on Heyfelder started to use chloroform. He was now able to perform more major operations, for example, the total resection of the hip-joint. In his book The experiments with sulphuric ether, salt ether, and chloroform he describes a great number of anaesthetic administrations using these 3 agents. In his summary Heyfelder concluded, that chloroform was undoubtly superior to sulphuric ether mainly because it was a quicker acting and longer lasting agent and leads to deeper narcosis. Moreover its application was much easier for it needed no special apparatus. However, because of its great anaesthetic potency, Heyfelder particularly demanded great caution in the application of chloroform. Explicitely he expected an assistant for chloroformizations, whose only duty was to supervise the inhalations and the patient--a forerunner of the modern specialized anaesthesiologist.
Anesthesiology↗
Colored illustrations of pediatric otorhinolaryngologic surgical techniques of a Turkish surgeon, Serefeddin Sabuncuoglu, in the 15th century.
OBJECTIVES: Serefeddin Sabuncuoglu is known to be the author of the first illustrated surgery book, Cerrahiyyetu'l Haniyye (Imperial Surgery), which was written in Turkish in 1465 a.d. at the age of 80. The purpose of this paper is to describe his contributions to pediatric otorhinolaryngology. RESULTS: Cerrahiyyetu'l Haniyye consists of three chapters totaling 412 pages, in which there are 191 sections dealing with a wide range of surgical specialties, including pediatric otorhinolaryngologic surgery. Sabuncuoglu explains some of child diseases and their treatment in a whole section, and gives information on the same subject in relevant part of sections. The illustrations he provides are not at a state of the art level with those of his contemporaries, but they bear a great importance considering the attitudes of Islam to the art of painting. For each section, a single, poetic sentence describes in detail the diagnosis, classification and surgical technique. Some authors have claimed that he only translated Ebu Kasim-al Zahravi (Albucasis)'s Al-Tasrif (Textbook of Surgery) and added the illustrations of the surgical techniques. Even if this is accepted, the illustrations are enough to entitle this work as a milestone. Serefeddin Sabuncuoglu describes medical and surgical management pediatric otorhinolaryngologic diseases such as harelip, hypertrophy of adenoids, atresia of external auditory canal, a foreign body in external auditory canal, short frenulum, abscess of head, mass and abscess of neck in children and unpleasant odor in nose in his textbook. CONCLUSIONS: Although not recognized and rewarded in his time, Serefeddin Sabuncuoglu was a great surgeon in Turkish medical history. The sections on children in his book are of great importance in pediatric otorhinolaryngology which became a different speciality in the last century. Writing his book at his 80's, he deserves to be considered as a distinguished scientist, medical doctor and illustrator of his era, considering the insulting and discriminating ideas toward women and children, especially girls, at that period.
Books, Illustrated↗
Tight junctions of human keratinocytes in primary culture: a freeze-fracture study.
Human skin explants obtained from 2- to 5-yr-old patients with harelips were cultured in NCTC 168 medium at 37 degrees C, in a humidified atmosphere containing 5% CO2 in air. After a 2-week incubation period, the newly grown cells were studied with special reference to tight junctions by freeze-fracture electron microscopy. Many completely formed tight junctions were observed between the uppermost living cells of migrating epithelium, and fragmented tight junctions were seen between the lower layer cells. The tight junctions in the uppermost cells developed so well that they formed a belt-like network consisting of two to six rows of strands. This observation may suggest that human keratinocytes have the ability to produce tight junctions perfectly enough to serve as a barrier, although no complete tight junctions were formed in situ.
Cells, Cultured↗
Lactate, pyruvate, and excess lactate during ether and halothane anesthesia in infants and children.
Arterial blood lactate, pyruvate, and excess lactate (XL) were measured and calculated in 33 patients aged 7 months to 6 years over a period of 2 hours during repair of harelip or cleft palate. When the anesthetic was diethyl ether, lactate and pyruvate levels rose significantly 60 minutes after induction of anesthesia but did not rise thereafter. Excess lactate appeared 60 minutes after induction of ether anesthesia, but it, too, showed no further increase. When the anesthetic was halothane there was no significant change in lactate, pyruvate, or XL. Changes with ether were not age-related, nor could they be correlated with changes in rectal or skin temperature. The responses of lactate, pyruvate and XL to ether anesthesia in the present study were not significantly different from those found by others in adults.
Age Factors↗
Rotation advancement in traumatic upper lip ectropion.
Rotation advancement is a well-known technique in cleft lip repair. This procedure corrects lip length and abnormal muscle insertion, and allows for accurate approximation of lip structures. It is a widely accepted standard of practice for congenital harelip repair that teaches fundamental principles of reconstructive surgery. An innovation is presented in which this procedure is used to correct a traumatic lip ectropion. The anatomic design is identical to a congenital cleft lip repair. The benefits of this approach are discussed in an interesting patient report.
Adult↗
Congenital symblepharon (abortive cryptophthalmos) associated with meningoencephalocele.
An 18-year-old male patient was first seen with right congenital symblepharon (abortive cryptophthalmos) and right frontal meningoencephalocele. There were no other affected members in the pedigree. Computed tomograms revealed that the right orbital roof was absent. Apart from a right harelip on the affected side, there were no other systemic abnormalities. The eye had light perception only. The upper eyelid connected with the globe and the cornea was totally opaque. Echographic studies showed that the eye was improperly developed. Electroencephalography detected slow-wave activity over the right frontoparietal region.
Adolescent↗
A new case of the trisomy 9P syndrome. Report of a patient with unusual chromosome findings (46,XX/47,XX, + i (9p) and a peculiar congenital heart defect.
A girl is described for whom an unusual chromosome constitution was found and who had a peculiar congenital heart defect. The girl showed the main clinical features of the trisomy 9p syndrome, such as psychomotor retardation, microcephaly and brachycephaly, enophthalmos, antimongoloid eye slant, hypertelorism, abnormal ears, a globulous nose, downward slanting mouth, hypoplasia of phalanges and abnormal palmar creases. In addition, the girl had an incomplete harelip, a cleft palate and a peculiar congenital heart defect, a ventricular septal defect with pulmonary valve stenosis and a marked hypoplasia of the pulmonary trunk, including the bifurcation. Chromosome analysis revealed a mosaicism with normal and abnormal mitoses (47 chromosomes). The extra chromosome was a metacentric E16-like chromosome, which on the basis of the G, R, Q and Giemsa-11-banding could be identified as an isochromosome of the short arm of chromosome 9 (46,XX/47,XX, +i (9) (pter leads to cen leads to pter)). The patient consequently had a partial tetrasomy of the short arm of chromosome 9.
Abnormalities, Multiple↗
A, 1;6, translocation associated with congenital glaucoma and cleft lip and palate.
A translocation of a part of the long arm of a chromosome No. 1 onto the long arm of a chromosome No. 6 was observed in a 2 1/2-year-old boy with mental retardation, harelip, cleft palate and congenital glaucoma. Different banding methods revealed that the translocation t(1;6)(q23;q27) apparently was balanced. The conncection between the patients' symptoms and the chromosomal rearrangement might be fortuitous or produced by the chromosome aberration.
Child, Preschool↗
Comparison of attitudes of regular and adapted physical educators toward disabled individuals.
The Attitudes Toward Disabled Persons Scale (Form B) and a ranking survey of 10 disabling conditions were administered to 38 physical education teachers and 47 adapted physical education teachers. Both groups of teachers' attitudes toward the disabled were below the scale median. Both groups of teachers were more accepting of individuals with physical disabilities such as, amputee, harelip, and epilepsy, than individuals who were mentally retarded, cerebral palsied, and emotionally disturbed.
Adult↗
Waardenburg's syndrome: case reports in two Nigerians.
We saw two cases of Waardenburg's syndrome in Nigerian children. A 9-year-old girl had congenital deaf-mutism, high-arched palate, complete heterochromia iridis with pigment mottling in the posterior pole of the eye with the hypochromic iris, dyspigmented frontal scalp hair, a history of vitiligo, but with no dystopia canthorum. An 11-year-old boy had a harelip with cleft palate, heterochromia iridis, blonde fundus on the eye with blue iris, and dystopia canthorum.
Abnormalities, Multiple↗