Special Article &&&&&&&&&&&&&& The History of Congenital Diaphragmatic Hernia from 1850s to the Present Sergio G. Golombek, MD, FAAP stomach, small intestines, and part of the larger omentum were lying in the left pleural cavity; both the phrenic nerves were normal. Alderson reported a fatal case of diaphragmatic hernia with Congenital diaphragmatic hernia (CDH) is believed to result from symptoms of pneumothorax. The stomach, spleen, omentum, and incomplete fusion of the pleuroperitoneal membrane, and passage of the transverse colon were found lying in the left pleura. abdominal contents into the chest. A historical review of the literature on The most extensive paper on this subject was contributed by 5 this subject shows a wide divergence of opinion on the etiology of the Bodwitch, reporting an instance in the Massachusetts General various types of CDH, and on the recommended treatment. A variety of Hospital, giving a numerical analysis of all the cases of this affection theories regarding its causes and the optimal way of approaching it have found recorded in the writings of medical authors between the years been published from the mid 19th century through the 20th century, and 1610 and 1846. are reviewed in this article. Hillier described an instance of CDH in which nearly all the small Journal of Perinatology (2002) 22, 242–246 DOI: 10.1038/sj/jp/7210701 intestines and two thirds of the large intestines passed into the right side of the thorax. O’Dwyer,7 a New York surgeon, eloquently described an operation for the relief of CDH. INTRODUCTION Congenital diaphragmatic hernia (CDH) is a life-threatening ‘‘A child, at 3 1/2 years, had symptoms supposed to indicate condition that occurs with a frequency of approximately 1/3000 extensive empyema of left side, for the relief of which an opening was made in the 6th intercostal space. The escape of births, when the fetal diaphragm fails to develop normally. The a loop of small intestine through this opening showed the survival rate for infants with hernias of the foramen of Bochdalek real nature of the case. varies from institution to institution; but overall, it has remained at ...When the diaphragm was allowed to resume its position, about 50% for nearly half a century, despite advances in neonatal after the completion of the operation, the pressure from 1 intensive care, anesthesia, and surgery. below was so great that it bulged upward, so as to fill at least half the pleural cavity. The hernia being probably EARLY ANATOMIC AND SURGICAL UNDERSTANDING congenital, and the whole mass of intestine, with the exception of the descending colon, having occupied the chest It was believed that, after birth, progressive compression of the so long, there was no sufficient room for them in the lung and shift of the mediastinum by gaseous expansion of the abdominal cavity. herniated intestine was the sole pathological mechanism. ... Death occurred rather suddenly six hours after the Bochdalek’s hernia was reported by Lawrence as a ‘‘congenital operation, at the very time that he appeared to be doing well. deficiency of the muscular fibers of the left half of the diaphragm At the autopsy the edges of the wound in the diaphragm were with displacement of the stomach.’’ The patient died of double found somewhat separated, and in all probability the sutures 2 would have cut completely through in twenty-four hours pneumonia. This was followed by several case reports in the last 7 half of the 19th century.3–7 Dittel reported unilateral defect in had the patient lived so long. ’’ the diaphragm of an infant who died soon after birth. The On the basis of a case successfully treated, and a study of others reported, Walker8 came to the interesting conclusions summarized in Table 1, considering they were written at the beginning of the 20th The Regional Neonatal Center, New York Medical College, Westchester Medical Center, Valhalla, century. NY, USA. Of the 118 cases collected by Livingston, 83 were on the left side, Address correspondence and reprint requests to Sergio G. Golombek, MD, FAAP, The Regional 18 on the right, 4 were central, 2 were double, in 1 the diaphragm Neonatal Center, New York Medical College, Westchester Medical Center, Valhalla, NY 10595, USA. was absent. Journal of Perinatology 2002; 22:242 – 246 # 2002 Nature Publishing Group All rights reserved. 0743-8346/02 $25 242 www.nature.com / jp Congenital Diaphragmatic Hernia Golombek Table 1 The Conclusions of Walker8 on the Treatment of CDH The embryological development of CDH was well described by Keith ,13 confirming Holt’s summary of symptoms. (1) In all cases of so-called internal strangulation, one should always think of the possibility of a diaphragmatic hernia. (2) When the physical examination reveals the diaphragmatic hernia as ‘‘In the course of the development the mouth of the probable, an operation is imperative when the symptoms of pleural diverticulum comes to lie in front of the neck of obstruction are present. the 12th rib — its site being marked by the hiatus (3) In recent cases, if the diagnosis had been made early, laparotomy is between the crural and costal fibers of the diaphragm. probably the better operation. The mouth of this sac becomes closed by the thickening (4) In old cases, on account of the difficulty of reducing the gut, ligating the sac, and stitching the rent in the diaphragm, the transpleural of the peritoneum at the pleuroperitoneal junction before route is preferable. the end of the 2nd month of development. Here again the (5) If, after making a laparotomy, the gut cannot be reduced, a plastic properties of the peritoneum secure the closure of thoracotomy is justifiable. the hernial sac. If the opening fails to close, then the contents of the abdomen grow or are pressed through the pleuroper- ‘‘If a large deficiency exist, infants may live but a few itoneal passages, compressing the lungs. It is exceedingly hours; with smaller ones, life may be prolonged rare for a hernia to occur on the right side, for the indefinitely. Booker’s patient lived 2 1/2 months with liver serves to block the passage; but it is otherwise on nearly all the small intestine and omentum and the the left side; hence the usual CDH lies on the left side, transverse colon in the thorax [Arch Pediatrics vol 14 p and the protruding organs are those which occupy the 649] and Northup’s patient [Arch of Pediatrics vol 9 p left hypochondrium and umbilical regions of the 130], who died at 3 1/2 years of intercurrent disease, had abdomen.’’ several coils of the ileum, the cecum, and the appendix in the chest. The symptoms are in all cases obscure, the only Few years later, Hedblom,14 a surgeon from Madison, WI, reported frequent one being dyspnea, sometimes constant, some- a study on 378 cases in which operation was performed, from under 1 times in severe paroxysms resembling asthma, those being year up to 80 years of age. His cases were collected from the Mayo apparently produced by an accumulation of gas in the Clinic (19 patients) and from the literature (359 patients). He thoracic part of the intestine. The physical signs are those described the classification of diaphragmatic hernias (congenital, of pneumothorax, generally on the left side, with acquired, traumatic, and indeterminate); the age and sex of the displacement of the heart to the right. The condition is 9 patients; etiology; contents of hernia; symptoms; physical signs; not amenable of treatment. ’’ diagnosis; indications and types of operation; site of hernia; operative Gilmore10 described one case where the defect was located at the procedure; results, including mortality (127 deaths, a surprisingly left anterior border of the diaphragm. ‘‘These cases are very unusual. low operative mortality of 33.6%); causes of death and recurrence. As may be imagined, the physical chest signs thus produced are most This was ‘‘one of the most comprehensive papers on this subject,’’ unusual and puzzling.’’ according to Dr. Leon T. LeWald, from New York, who discussed the Feer11 incorrectly concluded that it occurs immediately after birth study in the same issue of the Journal of the American Medical and that nothing was to be hoped for from treatment. He described Association. the symptoms as those of severe asphyxia, occurring paroxysmally, and in many cases resulting in death soon after birth. Table 2 Summary and Conclusions of Hume12 ‘‘Tympany and absence of respiratory sounds are noted on (1) Diaphragmatic hernia may be classified as: the affected side of the thorax, and cardiac dullness (a) Traumatic: (i) true (ii) false disappears or is pushed to the right. The retraction of the (b) Congenital: (i) true (ii) false abdomen is characteristic. In some cases, the condition gives (2) Congenital hernia occurs through the left dome most commonly, to a no premonitory symptoms but develops suddenly with less extent through the esophageal orifice. In other situations, it is evidence of internal strangulation. In the newborn, nothing but rarely met with in adult life. (3) Hernia through the hiatus pleuroperitonealis takes place before the is to be hoped for from treatment; in older children only very closure of that opening in the embryo. extensive operations, involving resection of ribs, replacement (4) Hernia though the dome of the diaphragm is due to some primary of the hernial content and closure of the opening, are congenital ectopia of viscera, occurring at the time of the successful.’’ formation of the diaphragm, and sometimes associated with incomplete rotation of the intestine. 12 (5) Hernia through the esophageal orifice is due to failure of the tailward Hume, a London surgeon, collected 35 cases of undoubted CDH migration of the stomach, or in some cases to the persistence of a found in the English, French, and American publications during the para-esophageal recess.