Anomalies and Curiosities of Medicine

by George M. Gould; Walter L. Pyle

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 constituents to pass into the thorax, sometimes seriously interfering with respiration and circulation by the pressure which ensues. Alderson reports a fatal case of diaphragmatic hernia with symptoms of pneumothorax. The stomach, spleen, omentum, and transverse colon were found lying in the left pleura. Berchon mentions double perforation of the diaphragm with hernia of the epiploon. The most extensive paper on this subject was contributed by Bodwitch, who, besides reporting an instance in the Massachusetts General Hospital, gives a numerical analysis of all the cases of this affection found recorded in the writings of medical authors between the years 1610 and 1846. Hillier speaks of an instance of congenital diaphragmatic hernia in which nearly all the small intestines and two-thirds of the large passed into the right side of the thorax. Macnab reports an instance in which three years after the cure of empyema the whole stomach constituted the hernia. Recently Joly described congenital hernia of the stomach in a man of thirty-seven, who died from collapse following lymphangitis, persistent vomiting, and diarrhea. At the postmortem there was found a defect in the diaphragm on the left side, permitting herniation of the stomach and first part of the duodenum into the left pleural cavity. There was no history of traumatism to account for strangulation. Longworth cites an instance of inversion of the diaphragm in a human subject. Bartholinus mentions coalition of the diaphragm and liver; and similar cases are spoken of by Morgagni and the Ephemerides. Hoffman describes diaphragmatic junction with the lung.

Anomalies of the Stomach.—The Ephemerides contains the account of a dissection in which the stomach was found wanting, and also speaks of two instances of duplex stomach. Bartholinus, Heister, Hufeland, Morgagni, Riolan, and Sandifort cite examples of duplex stomach. Bonet speaks of a case of vomiting which was caused by a double stomach. Struthers reports two cases in which there were two cavities to the stomach. Struthers also mentions that Morgagni, Home, Monro, Palmer, Larry, Blasius, Hufeland, and Walther also record instances in which there was contraction in the middle of the stomach, accounting for their instances of duplex stomach. Musser reports an instance of hour-glass contraction of the stomach. Hart dissected the stomach of a woman of thirty which resembled the stomach of a predaceous bird, with patches of tendon on its surface. The right extremity instead of continuously contracting ended in a culdesac one-half as large as the greater end of the stomach. The duodenum proceeded from the depression marking the lesser arch of the organ midway between the cardiac orifice and the right extremity. Crooks speaks of a case in which the stomach of an infant terminated in a culdesac.