{"paper_id":"eedc128f-c22c-40e9-a845-38bcc87fe1b9","body_text":"Endometriosis, which is characterized by the incidence of \nstroma and endometrial glands outside of the uterine cavity, is \ncommon in approximately 10% of women during their child \nbearing age ( 1 ). Most common site of endometriosis is pelvic \nperitoneum has been reported in extra-pelvic locations, \nlike upper abdominal cavity and diaphragm, as well ( 2 ). In \nfact, diaphragmatic endometriosis involving the full thickness \nof the diaphragm includes 1-1.5% of patients diagnosed with \nendometriosis ( 1 ). This rare condition can be asymptomatic \nand has been discovered accidentally. A patient with diaphragmatic \nendometriosis experiences the following symptoms: upper \nabdominal pain on the right side, pain under the lower ribs, \npainful breathing, and sometimes nausea or vomiting ( 3 ,  4 ).\nHere in, we present a case of diaphragmatic endometriosis \nassociated with pelvic endometriosis in a 20-year-old \nfemale patient with chronic pelvic pain and dysmenorrhea \nwith a high score. In a preliminary investigation, she was \ndiagnosed with deep pelvic endometriosis. However, during \nlaparoscopic surgery of the entire abdominal and pelvic cavity, \ndiaphragmatic endometriosis was discovered incidentally, \nwhich had spread through the center and right parts of \ndiaphragm. In this case report, we introduce a rare case of \ndiaphragmatic endometriosis along with pelvic endometriosis \nand discuss its symptoms and therapeutic methods.\n\nIn March of 2017, a 20-year-old virgin female with \nachronic pelvic pain was referred to our center. The patient \ncomplained of severe pelvic pain with verbal numerical \nrating scale (VNRS) of 9 during the menstrual \ncycle. This chronic pain had lasted for almost one year. \nThe patient did not mention dyschezia, pain during or afterurination, \norother symptoms associated with diaphragmatic \nendometriosis, such as chest pain, shoulder pain, or \nright upper abdominal pain. Furthermore, she had used no \nhormone replacement therapy.\nIn abdominal examination, there was fullness on the \nleft side, while in both rectal examination and abdominal \nexamination, there was fullness in the posterior cul-de-\nsac. An immobile 10-cm mass wasfelt on the left side, \nwhereas another immobile 5-6-cm mass was on the right \nside that was fixed to the uterus.\nPelvic ultrasonography results indicated a cyst with an \napproximate size of 12×7 cm consisting of thick contents \nin the left ovary with internal septae, raising suspicion \nregarding formation of the tubo ovarian complex in endometrial \ncavity. Furthermore, the ultrasound findings \nshowed an endometrium a cyst with an approximate dimension \nof 4 cm on the right side with adhesion and endometrial \nnodule of the posterior fundus with moderate \nadhesion to the rectosigmoid. Therefore, magnetic resonance \nimaging (MRI) was performed to exclude the left \nmass from adenocarcinoma, while the results showed normal \nupper abdominal organs, including liver, spleen, pancreas, \nkidneys, adrenal, as well asthe lungs. In pelvic MRI \nfindings, there was endometrium in both adnexae along \nwith hydrosalpinx on the left side, whereas enhancement \nwas not reported in the left adnexal masses.\nIn addition, the blood test showed an anti mullerian hormone \n(AMH) of 1.82 and CA-125 of 125.1, while other \ntumor markers, including risk of ovarian malignancy algorithm \n(ROMA) and HE4 were normal.\nDuring laparoscopy, we noticed extensive endometriosis \nthat involved the anterior and posteriorcul-de-sac, both pelvic \nside walls, both ovaries, and sigmoid colon. The left \novary contained a cyst measured 10-12 cm with severe adhesion \nto the rectum, while the right ovary contained a cyst \nmeasured approximately 6 cm with moderate adhesion to \nthe tube and the right ovary. There was also no evidence \nof endometriosis in ureters. Anatomy of pelvis restored, \npelvic Die corrected and a 2-cm endometriotic nodule attached \nto the rectovaginal septum (RVS) was shaved.\nOn exploring the upper abdomen, 5 to 6 areas of superficial \nendometriosis were discovered in the, anterior and center \nof the right hemi-diaphragm (Figes .1 ,  2 ), but the left hemi-\ndiaphragm was intact. The tota Redwine l surface area of the \ndiaphragm (left side, center, and right side) was thoroughly \ninvestigated when the patient was put into reverse Trendelenburg \nposition. The fulguration was performed using bipolar \nenergy for endometriotic lesions of the diaphragm. The \nendoscopic exploration of thoracic cavity was not performed \nbecause the patient had no symptoms of shoulder or chest \npain, no history of catamenial hemothorax or pneumothorax \nand diaphragmatic involvement was superficial.\nLesions of endometriosis on the rightside and the center of the \ndiaphragm.\nLesion of endometriosis on the surface on right hemi diaphragm.\n\nIt has been reported that the incidence of endometriosis \namong the women of child bearing age is about 10% \n( 5 ). Peritoneal cavity, especially the pelvic peritoneum, is \nthe most common site of involvement, but endometriosis \nhas been shown in almost all parts of the body ( 1 ). Endometriosis \nmainly occurs at the age of 30 to 45 years \n( 6 ). The mean age for extra-pelvic endometriosis has been \nreported between 35 and 40 years with the prevalence of \n12% ( 2 ,  3 ). In addition, the average age for pelvic endometriosis \nis 25 to 30 years ( 3 ).\nDiaphragmatic endometriosis is a rare serious disorder, \nwhich has been reported for the first time as a separate \nterm by Brews ( 7 ). Most diaphragmatic lesions occur on \nthe right side. The pathogenesis of a higher prevalence \nof endometriosis in the subphrenic region is sampsons \nretrograde menstruation theory, which indicates that refluxed \nendometrium may be caught by falciform ligament \nin the right side of the diaphragm ( 1 ). Classical symptoms \nof diaphragmatic endometriosis are chest pain (pleuritic \npain, especially on the right side), dyspnea, epigastric \npain, shoulder pain, and upper abdomen painthat is sudden \nonset in many patients ( 2 ). It is noteworthy that although \nsymptoms are usually periodic, some patients with \ndiaphragmatic endometriosis experience continued symptoms, \nwhich are not associated with the menstrual cycle. \nTherefore, despite of atypical clinical symptoms, especially \nin patients with pelvic endometriosis, there should \nbe a strong clinical suspicion to different types of thoracic \nendometriosis.\nThe pain in diaphragmatic endometriosis is due to \nstimulation of a sensory branch of the C5 nerve root. The \nseverity of the symptoms varies depending on the location \nand depth of the lesions.It has been reported that \ndiaphragmatic endometriosis can be asymptomatic, while \nsome women may experience no clinical symptoms or an \nobscure pain ( 8 ). Some serious and life-threatening conditions \nassociated with diaphragmatic endometriosis are the \nresults of the expansion of the fenestrations or holes in the \ndiaphragm due to necrosis of endometriosis lesions ( 9 ). \nThese conditions are as follows: i. Catamenial pneumothorax \n(CPT) is a rare condition causing the lungs to collapse \nduring menses and responsible for about one third of \nspontaneous pneumothorax in women ( 10 - 12 ). It occurs \nalone or with different manifestations of thoracic endometriosis \nsyndrome (TES), including hemopneumothorax \nand catamenial hemoptysis, ii. Hemopneumothorax is \nknown as presence of blood and air in the chest cavity \n( 13 ), as well as iii. Intrathoracic endometriosis nodules.\nDiaphragmatic endometriosis isdiagnostic error due to \nthe similarity of clinical symptoms with other benign or \nmalignant disorders. About 95% of diaphragmatic lesions \noccur in the right side of the diaphragm, although it has \nbeen previously seen in the left side alone or both sides \nof the diaphragm, even in some vital structures, like the \nphrenic nerve. Furthermore, in most of the reported cases, \nthe lesions occur in the anterior or posterior portion of the\ndiaphragm and behind the liver. Therefore, due to the diversity \nof an organ site involvement, diaphragms and their \nsurrounding areas should be thoroughly examined ( 3 ).\nIn terms of macroscopic appearance, lesions may appear \nin different colours and shapes that are mostly reported \nas bruised, purple and purple red. Computerized \ntomography (CT) scan or MRI may play an important \nrole in diagnosis. Thoracic endometriosis may appear as \nsmall cystic lesions in chest radiography or CT scan ( 2 ). \nHowever, it has been shown that MRI may provide better \ndetails to diagnose endometriosis ( 6 ). In our case, MRI \nreport showed no pulmonary endometriosis lesions.\nTherapeutic measures for diaphragmatic endometriosis or \nsuspicious thoracic endometriosis may be mainly based on \nthe patient’s medical history. It has been strongly indicated \nthat the best treatment choice is the expectant approach as \ncompared to the other interventions for those patients with \nasymptomatic diaphragmatic endometriosis ( 14 ).\nHowever, for symptomatic patients, surgery will be beneficial, \nif the medicationis deemed to have failed ( 8 ,  15 ). \nGiven a possibility of damage to the diaphragm, phrenic \nnerve, lungs, vessels or heart, it is crucial to choose a surgical \nplan after an informed consent is obtained from the \npatient. Also, alternative therapeutic options should be \nexplained to the patient.\nThe patient’s age, the type of treatment andthe medication \nas well as the surgeon’s expertise should be also considered \nin this regard. Although there is still uncertainty \nabout the efficiency of laparoscopic surgery in diagnosis \nand treatment of diaphragmatic endometriosis ( 3 ), this \nconcern is being resolved in consultation with an expert \nlaparoscopic surgeon regarding the use of different techniques \nsuch as proper patient positioning for an optimum \nview of the diaphragm and associated structures. The involvement \nof hidden area including the junction of the \ndiaphragm and the posterior edge of the liver is common \nin the invasive conditions. In addition, the application of \nright sub-conundrum port or flexible laparoscope ( 16 ) \nmay provide a precise view of the diaphragm. Simultaneous \napplication of laparoscopy and thoracoscopic surgery \n(VATS) is also considered as an effective therapeutic plan \nin diagnosis and treatment of women with diaphragmatic \nendometriosis, suffering intolerable pain in the right upper \nabdomen and chest (due to hemopneumothorax).\nThe use of hormonal medications, such as danazol (oral \ncontraceptives), has been suggested to the patients who \nare not interested in VATS or believe the thoracoscopy is \nnot safe enough. The segmental resection is needed during \nVATS for the following disorders: tension pneumothorax, \nhemopneumothorax, lesions of pulmonary endometriosis, \nchemical pleurodesis, as well as pleurectomy ( 2 ). VATS \nas a procedure also provides the following abilities: resection \nof diaphragm implants, restoration of diaphragmatic \nfenestration, resection of apical blebs, and implants \nof lung parenchyma. This invasive procedure is known \nas an excisional technique dueto the complete removal of \nendometriosis lesions. In a number of control-randomized \nstudies, it has been shown that complete disease eradication \nis the only definitive way to relieve pain, while the \nrecurrence is negligible ( 17 ,  18 ). There are several different \nand effective methods for excision of the lesions, \nlike vaporization, ablation, hydrodissection, and surgical \nscissor excision.\nIn our case, diaphragmatic endometriosis was discovered \nafter inspection of the upper abdomen. In addition, \nthe patient had nosymptoms, such as shortness of breath, \nshoulder pain, and right upper quadrant (RUQ) pain. \nTherefore, due to laparoscopic examination of the diaphragm, \nand appearance of lesions, the endometriosis lesions \nablate. We decided to apply no other interventions \nfor the patient.\nIt has been reported that the asymptomatic diaphragmatic \nendometriosis can be safely treated with use of laparoscopic \nsurgery instead of laparotomy, diaphragmatic \nresection, or other interventions ( 3 ,  8 ,  9 ). Furthermore, \nVATS is known as a diagnostic and therapeutic method \nin selected symptomatic patients as compared to the laparotomy \nand thoracotomy. Medical treatment after surgery \nis different depending on the patient’s decision for future \npregnancies. In those patients who tend to getpregnant, \nthere is no need for further medications after surgery, but \nassisted reproductive technology (ART) is recommended \nIn contrast, for those who do not want to get pregnant, \nsuppressive hormonal treatment is used.\nAfter discharge, considering the virginity, we prescribed \nsuppressive hormonal medicationsfor the patient. \nFurthermore, we advised her to go to a hospital immediately \nif she experience shoulder or RUQ pain, shortness of \nbreath and other catamenial symptoms.\n\nEndometriosis is considered as a clinical puzzle for both \nphysicians and patients. Although many efforts have been \nmade for both diagnosis and treatment of this disease, it \nis still controversial in terms of clinical symptoms, pathophysiology, \ndisease progression as well as management. \nThe surgeon should be fully aware of the clinical symptoms, \npatient’s medical history, and endometriosis lesions \nduring a laparoscopic surgery. It is noted that if chest pain, \nshoulder pain, hemothorax, pneumothorax, and hemoptysis \noccur during the reproductive age, especially with \nacyclic pattern, then diaphragmatic endometriosis should \nbe considered. Therefore, a close inspection of both anterior \nand posterior parts of hemi-diaphragm and applying \na combined VATS/laparoscopy procedure are needed. \nThis is especially true if there are lesions present. In order \nto achieve better outcomes with prevention of recurrence \nand re-occurring clinical symptoms, resection of any suspicious \nlesion is also recommended.\nIt is noteworthy that in contrast to conservative treatment \nwhich will be applied when endometriosis is detected \nduring laparoscopic surgery in asymptomatic patients,\nhowever, an interventional approach is needed in symptomatic \npatients with post-surgical complications.","source_license":"CC0","license_restricted":false}