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anomalies and curiosities of medicine-第95章

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 the groove by a pulsatile swelling which occupied the position of the right auricle。 The clavicle and the two margins of the sternum had no connections whatever; and below the groove was a hard substance corresponding to the ensiform cartilage; which; however; was very elastic; and allowed the patient; under the influence of the pectoral muscles; when the upper extremity was fixed; to open the groove to nearly the extent of three inches; which was more than twice its natural width。 By approximating his arms he made the ends of his clavicles overlap。 When he coughed; the right lung suddenly protruded from the chest through the groove and ascended a considerable distance above the clavicle into the neck。 Between the clavicles another pulsatile swelling was easily felt but hardly seen; which was doubtless the arch of the aorta; as by putting the fingers on it one could feel a double shock; synchronous with distention and recoil of a vessel or opening and closing of the semilunar valves。

Madden pictures (Figs。 134 and 135) a Swede of forty with congenital absence of osseous structure in the middle line of the sternum; leaving a fissure 5 3/8 X 1 3/16 X 2 inches; the longest diameter being vertical。 Madden also mentions several analogous instances on record。 Groux's case was in a person of forty…five; and the fissure had the vertical length of four inches。 Hodgen of St。 Louis reports a case in which there was exstrophy of the heart through the fissure。 Slocum reports the occurrence of a sternal fissure 3 X 1 1/2 inches in an Irishman of twenty…five。 Madden also cites the case of Abbott in an adult negress and a mother。 Obermeier mentions several cases。 Gibson and Malet describe a presternal fissure uncovering the base of the heart。 Ziemssen; Wrany; and Williams also record congenital fissures of the sternum。

Thomson has collected 86 cases of thoracic defects and summarizes his paper by saying that the structures deficient are generally the hair in the mammary and axillary regions; the subcutaneous fat over the muscles; nipples; and breasts; the pectorals and adjacent muscles; the costal cartilages and anterior ends of ribs; the hand and forearm; he also adds that there may be a hernia of the lung; not hereditary; but probably due to the pressure of the arm against the chest。 De Marque gives a curious instance in which the chin and chest were congenitally fastened together。 Muirhead cites an instance in which a firm; broad strip of cartilage resembling sternomastoid extended from below the left ear to the left upper corner of the sternum; being entirely separate from the jaw。

Some preliminary knowledge of embryology is essential to understand the formation of branchial fissures; and we refer the reader to any of the standard works on embryology for this information。 Dzondi was one of the first to recognize and classify congenital fistulas of the neck。 The proper classification is into lateral and median fissures。 In a case studied by Fevrier the exploration of a lateral pharyngeal fistula produced by the introduction of the sound violent reflex phenomena; such as pallor of the face and irregular; violent beating of the heart。 The rarest of the lateral class is the preauricular fissure; which has been observed by Fevrier; Le Dentu; Marchand; Peyrot; and Routier。

The median congenital fissures of the neck are probably caused by defective union of the branchial arches; although Arndt thinks that he sees in these median fistulas a persistence of the hypobranchial furrow which exists normally in the amphioxus。 They are less frequent than the preceding variety。

The most typical form of malformation of the esophagus is imperforation or obliteration。 Van Cuyck of Brussels in 1824 delivered a child which died on the third day from malnutrition。 Postmortem it was found that the inferior extremity of the esophagus to the extent of about two inches was converted into a ligamentous cord。 Porro describes a case of congenital obliteration of the esophagus which ended in a cecal pouch about one inch below the inferior portion of the glottidean aperture and from this point to the stomach only measured an inch; there was also tracheal communication。 The child was noticed to take to the breast with avidity; but after a little suckling it would cough; become livid; and reject most of the milk through the nose; in this way almost suffocating at each paroxysm; it died on the third day。

In some cases the esophagus is divided; one portion opening into the bronchial or other thoracic organs。 Brentano describes an infant dying ten days after birth whose esophagus was divided into two portions; one terminating in a culdesac; the other opening into the bronchi; the left kidney was also displaced downward。 Blasius describes an anomalous case of duplication of the esophagus。 Grashuys; and subsequently Vicq d'Azir; saw a dilatation of the esophagus resembling the crop of a bird。

Anomalies of the Lungs。Carper describes a fetus of thirty…seven weeks in whose thorax he found a very voluminous thymus gland but no lungs。 These organs were simply represented by two little oval bodies having no lobes; with the color of the tissue of the liver。 The heart had only one cavity but all the other organs were perfectly formed。 This case seems to be unique。 Tichomiroff records the case of a woman of twenty…four who died of pneumonia in whom the left lung was entirely missing。 No traces of a left bronchus existed。 The subject was very poorly developed physically。 Tichomiroff finds four other cases in literature; in all of which the left lung was absent。 Theremin and Tyson record cases of the absence of the left lung。

Supplementary pulmonary lobes are occasionally seen in man and are taken by some authorities to be examples of retrogressive anomalies tending to prove that the derivation of the human race is from the quadrupeds which show analogous pulmonary malformation。 Eckley reports an instance of supernumerary lobe of the right lung in close connection with the vena azygos major。 Collins mentions a similar case。 Bonnet and Edwards speak of instances of four lobes in the right lung。 Testut and Marcondes report a description of a lung with six lobes。

Anomalies of the Diaphragm。Diemerbroeck is said to have dissected a human subject in whom the diaphragm and mediastinum were apparently missing; but such cases must be very rare; although we frequently find marked deficiency of this organ。 Bouchand reports an instance of absence of the right half of the diaphragm in an infant born at term。 Lawrence mentions congenital deficiency of the muscular fibers of the left half of the diaphragm with displacement of the stomach。 The patient died of double pneumonia。 Carruthers; McClintock; Polaillon; and van Geison also record instances of congenital deficiency of part of the diaphragm。 Recently Dittel reported unilateral defect in the diaphragm of an infant that died soon after birth。 The stomach; small intestines; and part of the large omentum lay in the left pleural cavity; both the phrenic nerves were normal。 Many similar cases of diaphragmatic hernia have been observed。 In such cases the opening may be large enough to allow a great part of the visceral constituent
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