the diaphragmatic defect. Using a tapered non-cutting needle and a running Goretex® suture, the patch was circumferentially an- chored tension-free to the ribs, ...
CASO CLINICO/ CASE REPORT
A rare case of left diaphragmatic agenesis in an elderly patient
LANDINO FEI,VINCENZO TRAPANI, FRANCESCO MOCCIA, MARCO CIMMINO Unit of General and Gastrointestinal Surgery “F. Magrassi-A. Lanzara” – Department of Clinical and Experimental Medicine and Surgery – Second University of Naples – School of Medicine – Naples Correspondence to: Prof. Landino Fei – Corso Vittorio Emanuele, 494/F – 80135 Napoli
Riassunto
Introduction
Parole chiave: agenesia diaframmatica, riparazione erniaria protesica
Partial or complete agenesis of the hemidiaphragm is a rare congenital malformation whose embryological basis is unknown1,2. The defect may be associated with herniation of the abdominal contents into the thoracic cavity and pulmonary hypoplasia, that results in progressive respiratory and cardiocirculatory failure and death of the neonate. Late presentation is extremely rare and older patients are asymptomatic for a long time without developing ventilatory insufficiency,perhaps - and paradoxically - precisely because of the associated lung hypoplasia1-6. Thus, only 7 cases of hemidiaphragmatic agenesis have been reported in adult patients, four of which were left sided1,4,5,two right sided3,6 and one bilateral2. Our elderly patient is,
Gli Autori riportano un raro caso relativo ad un paziente di 71 anni affetto da agenesia completa dell’emidiaframma sinistro e che ha sviluppato solo tardivamente stipsi ostinata ed episodiche crisi subocclusive. Alla laparotomia sottocostale sinistra lo stomaco, il colon trasverso con la flessura splenica e la milza erano completamente risaliti ed allocati nell’emitorace sinistro. La riparazione è stata effettuata utilizzando un patch di politetrafluoroetilene espanso di 2 mm di spessore (Gore-Tex®): la protesi sagomata come una neocupola diaframmatica è stata fissata circonferenzialmente con una sutura continua in PTFE ai bordi del difetto. Non sono state osservate complicazioni maggiori nell’immediato periodo postoperatorio né recidiva a 34 mesi di follow-up. Gli Autori puntualizzano non solo la rarità dell’agenesia vera dell’adulto (solo 8 casi riportati in letteratura), ma anche che il paziente è il più anziano tra quelli trattati chirurgicamente. L’uso, infine, di un patch in PTFE, grazie alla sua resistenza e malleabilità, può garantire una buona riparazione sia anatomica sia funzionale.
Summary A rare case of left diaphragmatic agenesis in an elderly patient. L. Fei, V. Trapani, F. Moccia, M. Cimmino A 71-year-old man affected by left hemidiaphragmatic agenesis developed late severe constipation and occasional episodes of bowel obstruction. At left subcostal laparotomy, the stomach, transverse colon, splenic flexure, and spleen were located in the left hemithorax. Repair was performed with a 2-mm-thick expanded polytetrafluoroethylene (Gore-Tex®) patch secured in place circumfer-
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CASO CLINICO/CASE REPORT
2008 - vol. 60 n. 3 pp 483-486
therefore, the eighth such diagnosed case in the literature.The patient had been asymptomatic for a long time and required surgical treatment only after developing severe gastrointestinal symptoms.
Case report A 71-year-old man presented to our Surgical Unit with features of dyspepsia, severe constipation and occasional but increasing episodes of incomplete bowel obstruction accompanied by abdominal distension and pain.The patient did not report any previous trauma or chest surgery and his family history was unremarkable. Chest examination revealed bowel sounds and absent air entry in the left hemithorax. Standard chest X-rays showed intestinal gas associated with a reduced diminished pulmonary volume in the left hemithorax (Fig.1A). Contrast medium X-ray of the upper gastrointestinal tract and enema documented the herniation of the stomach and the colon into the thoracic cavity (Fig. 1B). CT scan confirmed these findings and demonstrated the absence of a diaphragmatic remnant. Spirometry and blood gas analysis were normal.
Fig. 1. Features of the left diaphragmatic agenesis and positioning of the prosthesis.A) detail of chest x-ray showing the bowel herniation into the left hemithorax; B) contrast medium x-ray enema showing herniation of the colon into the left thoracic cavity; C) intraoperative picture showing left diaphragmatic agenesis and the e-PTFE Gore-Tex“ patch in place; D) postoperative standard chest x-ray showing correct position of the new prosthetic diaphragmatic dome.
Surgical treatment was carried out through a left subcostal laparotomy extended on the right side.The stomach, transverse colon, splenic flexure, spleen and part of the small bowel were found in the left thoracic cavity, while there was no evidence of the diaphragmatic remnant. Repair was performed with a 2-mm-thick expanded poly-
entially as a new diaphragmatic dome. No early major complications and no recurrence at 34 months’ follow-up were observed. To the best of our knowledge, this is the oldest treated patient with a true hemidiaphragmatic agenesis and is the eighth case reported in the literature. The use of the ePTFE soft tissue patch, thanks to its strength and pliability, affords good anatomical and functional repair.
Key words: diaphragmatic agenesis, prosthetic hernia repair Chir Ital 2008; 60, 3: 483-486
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tetrafluoroethylene (ePTFE) soft tissue patch (Gore-tex ® ) (W.L. Gore & Associates, Inc, Flagstaff, AZ, USA), which was shaped on the diaphragmatic defect. Using a tapered non-cutting needle and a running Goretex ® suture, the patch was circumferentially anchored tension-free to the ribs, intercostal muscles and endothoracic fascia (Fig.1C). Postoperatively,the patient received intravenous administration of analgesic drugs (ketorolac 30 mg, b.i.d.) for 72 hours. Chest X-rays showed good placement of the new diaphragmatic dome and the fundic air bubble in its correct position (Fig. 1D). No early major or late postoperative complications were observed, and no recurrence was found at 34 months’ follow-up.
A rare case of left diaphragmatic agenesis in an elderly patient
Discussion The main questions relating to the management of large diaphragmatic defects, such as agenesis, concern when and how to operate. As regards the former question, in cases of partial defects of smaller size, immediate repair is unanimously recommended even in asymptomatic subjects, because of the risk of bowel incarceration and strangulation1,4. On the other hand, the real need for surgical repair of large diaphragmatic defects (e.g. agenesis) in asymptomatic adults is a matter of debate because of the low risk of bowel complications thanks to the free scope for movement of the herniated viscera4. As far as surgical technique is concerned, it is worth noting that the aim of this surgery is to repair the defect, restoring anatomical and functional integrity with minimal stretching of the diaphragm, as well as to assure adequate protection of the lung and the mediastinum from infection and prevent the risk of adhesions or recurrences7,8.Many diaphragmatic hernias can be repaired by primary closure. In contrast, in patients
with large defects or agenesis of the diaphragm, direct suturing may be impossible because of the absence of adequate muscular tissue6,7. Among the 7 adult patients with hemidiaphragmatic agenesis reported in the literature, 3 were treated with a polypropylene mesh 5,6, one with ePTFE mesh 2, one without mesh 1 , and two asymptomatic patients were not operated on3,4.We employed an ePTFE patch, which is currently used for repairing others types of wall defects7,9. It is likely that the use of ePTFE mesh is more appropriate than other types of prosthetic materials the use of which carries a risk of postoperative adhesions 9 . At the same time, absorbable meshes should be avoided because of the lack of appropriate life-long support due to their rapid absorption. Non-absorbable meshes, such as the Gore-Tex® soft tissue patch, are endowed with full pliability,as confirmed by the fact that the sutures have been anchored close to the graft edge with a low risk of tearing9,10.This microporous mesh possesses good biocompatibility and produces a low inflammatory and fibrous reaction with a peripheral and interstitial arrangement of collagen fibers that allows the forma-
tion of a smooth surface. This surface is able to support a continuous layer of mesothelial cells on the peritoneal surface, resulting in increased fibrinolytic activity which is able to degrade fibrous adhesions9,10.Its employment in an intraperitoneal position induces formation of very few loose adhesions, thus reducing the risk of visceral erosion and fistulas8. On the other hand, macroporous material, such as polypropylene meshes, induces the formation of strong adhesions on the peritoneal surface because it produces inconsistent and disordered mesothelial proliferation. This feature, together with its relative rigidity, can be considered the major disadvantage of the intraperitoneal use of polypropylene mesh8,9. In summary, the Gore-Tex® soft tissue patch, thanks to its high pliability, strength and low adhesiveness, is a good prosthesis for intraperitoneal and intrathoracic use.This mesh seems to be an adequate and satisfactory diaphragmatic substitute,which can be easily used to repair large diaphragmatic defects, ensuring the most anatomical and physiological conditions possible with only a low risk of recurrence.
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5. Singh G, Bose SM. Agenesis of hemidiaphragm in adults. Aust N Z J Surg 1993; 63: 327-8. 6. Glavas M, Drazinic I, Pikot D, Altarac S. Patch reconstruction of hemidiaphragm agenesis by the polypropylene mesh prosthesis. Croat Med J 2000; 41: 333-5. 7. Menezes SL, Chagas PS, MacedoNeto AV, Santos VC, Rocco PR, Zin WA. Suture or prosthetic reconstruction of experimental diaphragmatic defects. Chest 2000; 117: 1443-8. 8. Klinge U, Klosterhalfen B, Muller M, Shumpelick V. Foreign body reaction to meshes used for the repair of abdomi-
nal wall hernias. Eur J Surg 1999; 165: 665-73. 9. Matthews BD, Mostafa G, Carbonell AM, Joel CS, Kercher KW, Austin C, Norton HJ, Heniford BT. Evaluation of adhesion formation and host tissue response to intraabdominal polytetrafluoroethylene mesh and composite prosthetic mesh. J Surgical Research 2005; 123: 227-34. 10. Isaka M, Nishibe T, Okuda Y, Saito M, Seno T, Yamashita K, Izumisawa Y, Kotani T, Yasuda K. Experimental study on stability of a high-porosity expanded polytetrafluoroethylene graft in dogs. Ann Thorac Cardiovasc Surg 2006; 12: 37-41.
