Peritoneopericardial Diaphragmatic Hernia with a Small Intestinal Obstruction Within the Pericardial Sac Isaac M. Angell Clinical Advisor: H. Jay Harvey Basic Science Advisor: Linda A. Mizer Senior Seminar Paper Cornell University College of Veterinary Medicine September 22, 2010 Key Words: Hernia, Peritoneopericardial, Small Intestinal Obstruction, Foreign body, English Bulldog Abstract: A two year old male, castrated English Bulldog, was presented to Cornell University Hospital for Animal’s Emergency Service for lethargy, vomiting, inappetance, and diarrhea in May of 2010. On presentation, the dog was quiet, alert, and responsive. His heart sounds were mildly muffled but there were no murmurs or arrhythmias noted. Decreased lung sounds were noted on auscultation, particularly on the right. Intestinal loops could not be abdominally palpated, and his abdomen appeared grossly small. Based on orthogonal thoracic radiographs, a diagnosis of a Peritoneopericardial Diaphragmatic Hernia (PPDH) was made. The presenting clinical signs and diagnosis of a PPDH warranted surgery. This case was found to be unique and complicated at surgery because not only did the pericardial sac contain an entire length of jejunum and a small portion of the quadrate lobe of the liver, but there was also a foreign body in the ileum within the pericardial space. The patient handled the surgery well and recovered successfully. Introduction: According to Saunder’s Dictionary, a Peritoneopericardial Diaphragmatic Hernia (PPDH) is an, “[A]nomalous development of the diaphragm and pleuropericardial membranes that allows herniation of variable amounts of abdominal contents into the pericardial sac.”1 Although PPDHs are the most frequently occurring congenital pericardial and diaphragmatic anomaly in dogs and cats2, the difference between them 1 and what often leads to their diagnosis is not the primary disease itself, but a secondary disease process or an incidental finding upon a physical examination 3. The etiology of a PPDH according to The Embryology of Domestic Animals, “[H]as often been explained erroneously as a direct, persistent connection between the peritoneal and pericardial cavities. However, there is never a natural communication here to be closed.”4 Since there is never a natural communication, it therefore means that the PPDH is formed due to a secondary phenomenon. There are many different theories on the exact mechanism of a PPDH formation. One theory that has been hypothesized is that as the liver grows out of the septum transversum and the peritoneal space extends between the two, a faulty separation process occurs that either results in a small rent in the septum transversum or the septum tranversum becomes so thin, that at some point in time it ruptures and thus results in the communication between the peritoneum and pericardial space4. Some others suggest that the lateral pleuroperitoneal folds and the ventromedial pars sternalis fail to unite during the division of the coelom, and still others think that there is a prenatal injury to the septum transversum and/or injury at the fusion site of the septum transversum and pleuroperitoneal folds5. A PPDH predisposes the affected animal to secondary sequellae. The issue is such that a hernia becomes a gateway to trouble. Many abdominal organs can travel through the hole in the ventral aspect of the diaphragm and into the pericardial sac. These organs include, but are not limited to, the liver and small intestines. Depending on the extent of the defect, many or a few organs become malpositioned. With such peculiarities come problems: abdominal viscera can compress the heart and or the lungs, or abdominal 2 organs can become trapped and compromised. These are the states in which many animals that have a PPDH are found6. Case History: An almost two year old male, castrated English Bulldog was presented to Cornell University’s Hospital for Animal’s (CUHA) Emergency Service for lethargy, inappetance, vomiting, diarrhea, and a possible diaphragmatic hernia on May 19, 2010. He was brought to his primary veterinarian on May 18, 2010 because he had become very lethargic and vomited once the previous night. The referring veterinarian (rDVM) obtained abdominal and thoracic radiographs which were suggestive of either a diaphragmatic hernia (DH) or PPDH. The rDVM’s radiographs were not diagnostic to determine whether or not the dog had a DH or a PPDH. He was being treated with antibiotic ophthalmic drops for an ocular infection, due to keratoconjunctivitis sicca resulting from bilateral removal of his third eyelid glands in October of 2008 and January of 2009. Since that time he had been receiving artificial tears. Recently, he had also been treated topically for mites. He was up-to-date on his vaccinations and was on heartworm and flea and tick preventatives. 3 Chief Complaints:  Inappetance  Vomiting  Lethargy  Diarrhea Clinical Findings: On physical examination, he was quiet, alert, and responsive with a Body Condition Score (BCS) of 5/9. His heart sounds were mildly muffled, but no murmurs or arrhythmias were noted. His mucous membranes were pink and moist, and his Capillary Refill Time (CRT) was less than two seconds. Decreased lung sounds were ausculted, particularly on the right side of the thorax. Intestinal loops could not be palpated on abdominal palpation and his abdomen appeared grossly small. Bilateral entropion was present and a purulent discharge coated both globes. His coat was healthy with no evidence of seborrhea or ectoparisites. No evidence of orthopedic problems, peripheral lymphadenopathy, or nervous system abnormalities were observed. Orthogonal thoracic radiographs were taken to distinguish between a DH (usually traumatic) and a PPDH (usually congenital). The best way to determine whether or not the PPDH is clinical, is to determine if the patient has corresponding clinical signs. Radiological evidence that, indeed, points to a PPDH is an enlarged cardiac silhouette which often has a heterogeneous opacity, other abdominal organs absent from the abdomen, and a small appearing liver7. One of the hallmark radiographical signs is air- 4 filled intestines within the pericardial sac8. Both the lateral and ventral dorsal radiographic views of the thoracic cavity showed air-filled intestines which were confined within the pericardial sac. Based on this information a diagnosis of a PPDH was made. Of concern was not the fact that indeed there was a PPDH, but the fact that the patient had other signs such as lethargy, inappetance, and diarrhea with an episode of vomiting, in conjunction with a PPDH. Based on these factors, the decision was made to do surgery if he was doing well enough systemically. Surgery was scheduled to correct the PPDH, and to determine the cause of the other clinical signs. Blood was drawn for a CBC, Chemistry Panel, and Gaslyte to ensure that the patient was healthy enough for surgery. Abnormal findings consisted of hypochloremia of 103mEq/L (reference range 107-117) consistent with a third space loss, hypokalemia of 3.4mEq/L (reference range 3.9-5.3) most likely due to gastrointestinal losses and third space losses/sequestration, hypoferremia of 52ug/dL (reference range 98-220)/decreased Fe saturation of 15ug/dL (reference range 28-62) seen with inflammation, thrombocytopenia of 122thou/uL (reference range186-545) can be low with bacterial infections, decreased HCT of 40% (reference range 41-60), decreased hemoglobin of 13.9g/dL (reference range 14.1-20.2) also seen with infectious processes, and neutrophilia of 11.6thou/uL (reference range 2.7-9.4) consistent with infection9. Gaslyte results were consistent with a mild respiratory acidosis with metabolic compensation. 5 Signalment: The patient was an English Bulldog, a breed that is known for having numerous congenital anomalies. Although PPDHs are not known to have a positive correlation with this breed, other genetically predisposed conditions were on the differential diagnosis list and many this patient was ultimately diagnosed with these, including:  Cardiovascular conditions – Ventricular septal defect, Tetralogy of Fallot, Aortic stenosis, and Pulmonic stenosis.  Neoplastic conditions – Mast cell tumors, Primary brain tumors, and Lymphosarcoma.  Ocular conditions – Entropion, Distichiasis, Trichiasis, Keratoconjunctivitis Sicca, Prolapse of the gland of the nictitating membrane, Refactory corneal ulceration, and Multifocal retinal dysplasia.  Renal and urinary conditions – Ectopic ureters, Urethrorectal fistula, Urethral prolapse, Sacrocaudal dysgenesis, Cystine urolithiasis, and Urate urolithiasis10. Problem List: 1) Peritoneopericardial Diaphragmatic Hernia 2) Ulcers - corneal 3) Entropion 4) Ectopic cilia 5) Neutrophilia 6) Mild respiratory acidosis with metabolic compensation 6 Differential Diagnosis:  Impacted/obstructed intestine  Infectious gastritis  Traumatic injury  Diaphragmatic Hernia  Pericardial effusion Prognosis: PPDHs are not a death sentence. In fact, many affected animals can be asymptomatic throughout all or part of their whole lives. Many pets are not diagnosed with a PPDH until it is found as an incidental finding during a radiographic or ultrasonagraphy exam. Coincidentally, one-third of dogs and cats are not diagnosed with a PPDH until they are greater than four years old11. The decision to perform corrective surgery for an animal should be weighed against the perceived benefits. In an aged and asymptomatic animal, it may be the wrong choice to do surgery. The prognosis following a successful surgery, however, is excellent12. In one study that was done, the most common owner complaints after surgical repair of a PPDH included: lethargy, vomiting, weight loss, diarrhea, and coughing. Upon a successful corrective herniorrhapy, very few complications were reported. Often when there were complications, they were related to the primary reason why the animal presented for examination, not a disease process involving the PPDH. Some complications that were reported include a gastrointestinal obstruction due to a foreign 7 body, excessive internal bleeding from a gastrotomy incision, and development of a pneumothorax. The outcomes for those animals not taken to surgery are less sure. Although animals with PPDHs can often live for years without clinical signs, there is always a certain amount of risk for future complication when the abnormality is not corrected. Surgical repair should always be held as a viable option; a corrective herniorrhapy is a relatively simple procedure with few or no complications and postoperative survivals are excellent13. The patient was given an excellent prognosis for recovery from his emergency heriorrhapy and enterotomy surgery. Two days after he recovered from surgery he was given a poor prognosis for an unrelated disease process that threatened his eyesight, unless he had emergency eye surgery. His owners elected for bilateral conjunctival grafts which were performed. After the surgery, he was given a good prognosis for the return of eyesight. Treatment: The treatment for the patient’s presenting issues was a herniorrhapy and abdominal exploratory surgery. He was placed under general anesthesia and a standard ventral midline celiotomy was performed from the xiphoid to the caudal caudal aspect of his scrotum. A defect was found in the ventral muscular portion of the diaphragm, communicating with the pericardial sac. It was necessary to make defect in the diaphragm larger so that all the contents from within the pericardial sac could be removed, including a focal dilation of the small intestines which was trapped between the 8 heart and the pericardium. An entire length of jejunum, ileum, and a small portion of the quadrate lobe of the liver were removed from the pericardial space. The PPDH was closed in a simple, interrupted fashion. All abdominal viscera were examined for abnormalities. A small portion of the quadrate lobe of the liver appeared discolored, yet due how small the focal ischemia was no corrective treatment was deemed necessary. An enterotomy was preformed along the antimesenteric border of the ileum where it was focally dilated with a foreign body, and a 1”x 3” plastic foreign body was removed from the site. The enterotomy was closed in a standard fashion. The patient recovered from surgery without complication. After surgery he was started on the following treatments: NaCl IV fluids to help with hydration, Fentanyl continuous rate infusion (CRI) and Dexmedetomidine to aid in the control of pain, Baytril and Unasyn to help prevent infection, and Famotidine, Sucralfate, and Cerenia to help heal and rest his gastrointestinal system. Incidentally, two days later after presentation he was taken back to surgery because of deep infected corneal ulcers in both eyes (OU). Conjunctival grafts were performed OU, entropion in the left eye (OS) was repaired, and a nest of ectopic cilia on the dorsal lid margin OS was removed. Sutures were placed at the lateral margin of both eyelids to reduce excessive eye movements that could damage the graft. He recovered from anesthesia uneventfully. At the time of discharge the patient was sent home on Tramadol, Famotidine, Clavamox, Baytril, 2% Cyclosporine ophthalmic drops, Ciprofloxacin ophthalmic drops, Atropine ophthalmic ointment, Neo-Poly-Bacitracin ophthalmic ointment, and Cefazolin ophthalmic drops. 9 Outcome: The patient was discharged from the hospital five days after he was presented on emergency for his initials signs of inappetence, letheragy, vomiting, and diarrhea. At the time of discharge the patient seemed to have fully recovered from the presenting gastrointestinal issues. At subsequent visits for suture removal and ophthalmology appointments, it was reported that he was healthy and that his eyes were healing very well. At the last visit it was suggested that he was doing so well that the next re-check appointment was scheduled in one year. Discussion: This case was interesting in the fact that the patient had a chronic persistent disease, in fact a life-long disease. He had lived in harmony with it, until an acute disease process interrupted the unique familiarity the abdominal viscera had with a vital thoracic organ. What makes this case unusual is not the congenital abnormality itself, but how the secondary intestinal blockage interplayed with the original issue. To diagnose a PPDH it was important to differentiate between that and a DH. This was done based on the fact that a majority of DHs are usually traumatic, there was no know trauma associated with this dog, and that on radiographic examination all abdominal viscera were contained within the pericardial sac. PPDHs are very well characterized, however, the etiology is not specifically known although there have been several ideas hypothesized. Despite an uncertain etiology, the diagnosis and treatment would still dependent on an attentive diagnostician 10 or an attention grabbing incident. Surgery was the only intervention available to this patient and, luckily in this case, the surgical result was curative. The surgical approach that is most commonly used for PPDHs, and was used in this case, is a midline celiotomy. An incision was made through the linea alba being sure to avoid the rectus abdominis muscle on either side. This approach allows for viewing of all the abdominal organs as well as being less painful than a sternotomy. One disadvantage is that the ventral aspect of the diaphragm can be hard to visualize. Of course, the main goal with a herniorrhapy is to repair the defect, but to do this in the case of a PPDH it is necessary to remove all the abdominal viscera from the pericardial sac. To remove the viscera it is often necessary to enlarge the actual opening through the diaphragm. The defects in the diaphragm and thus in the pericardial sac, since the two structures are conjoined, can be sutured together either with a simple continuous or interrupted pattern. The suturing should start dorsally and proceed ventrally. If there is a large amount of air in the pericardial sac at closing it may be necessary to drain off the air and shrink down the potential space14. In this case a concurrent enterotomy procedure had to be done to remove a plastic foreign body within the ileum. An enterotomy should be performed on the antimesenteric border distal to the foreign body to have the suture line in as healthy of tissue as possible. After the foreign body is removed, the incision is closed with a simple interrupted or continuous longitudinal single-layer appositional pattern. The key is to have the holding layer, the submucosa, incorporated into every suture15. The uniqueness of this case is found in the extremely rare cavity that a small intestinal foreign body was found within the small intestine. The primary congenital 11 abnormality could have never been noticed if he had not had this secondary damning pathology. Luckily for him, his problems were able to be fixed with a surgical treatment that was curative and resulting prognosis excellent16. 12 References: 1. Blood DC, Studdert VP, Gay CC. Peritoneopericardial hernia. Saunder’s comprehensive veterinary dictionary. 3rd ed. Philadelphia: Saunders Co. 2007;1362. 2. Hunt GB, Johnson KA. Diaphragmatic, pericardial, and hiatal hernia. In: Slatter D, ed. Textbook of small animal surgery. 3rd ed. Philadelphia: Saunders Co. 2003; 473. 3. Merck Vet Manual Web site. Peritoneopericardial Diaphragmatic Hernia (PPDH). Availble at: http://www.merck vetmanual.com/mvm/index.jsp?cfile=htm/bc/11112.htm. Accessed Sept 2, 2010. 4. Noden DM, De Lahunta A. Malformations of the diaphragm. 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English bulldog. Breed predispositions to disease in dogs and cats. Ames: Blackwell Publishing Ltd. 2004; 38. 11. Sisson DD, Thomas WP. Pericardial disease and cardiac tumors. In: Fox PR, Sisson DD, Moise NS, ed. Textbook of canine and feline cardiology: principles and clinical practice. 2nd ed. Philadelphia: WB Saunders Co, 1999; 683. 12. Quintavalla C, Zannetti G. A case of diaphragmatic peritoneal pericardial hernia in a dog. Available at: http://www.unipr.it/arpa/facvet/annali/1998/quintavalla/quintavalla.htm Istituto di Clinica Medica Veterinaria 1998. Accessed Sept 2, 2010. 13. Wallace J, Mullen HS, Lesser MB. A technique for surgical correction of peritoneal pericardial diaphragmatic hernia in dogs and cats. J Am Anim Hosp Assoc 1992;28:503510. 14. Hunt GB, Johnson KA. Diaphragmatic, pericardial, and hiatal hernia. In: Slatter D, ed. Textbook of small animal surgery. 3rd ed. Philadelphia: Saunders Co. 2003; 407, 481. 15. Brown DC. Small intestines. In: Slatter D, ed. Textbook of small animal surgery. 3rd ed. Philadelphia: Saunders Co. 2003; 650. 16. Tilley LP, Smith FWK. Peritoneopericardial diaphragmatic hernia. Blackwell’ Five Minute Veterinary Consult: Canine and Feline. 4th ed. Ames: Blackwell Publishing 2007;1065. 13