Pneumatosis Cystoides Intestinalis Complicated during Chemotherapy for Pulmonary Nontuberculous Mycobacterial Disease

Background: Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by the presence of gas in the intestinal wall. Aim: We report two rare cases of PCI that are complicated during the chemotherapy for pulmonary nontuberculous mycobacterial (NTM) disease. Case Presentation: In this report, we described two cases (a 72-year-old woman and a 60-year-old woman) of PCI that appeared during the combined chemotherapy consisting of rifampicin, ethambutol and clarithromycin. Because there were few clinical symptoms and increased inflammatory responses, the diagnosis of PCI was delayed. However, there were fortunately no severe complications in both cases. Conclusion: Respiratory physicians should be aware of the potential development of PCI in patients during the chemotherapy for pulmonary NTM disease. It is important to detect PCI in the early stage through radiological examinations to avoid severe complications.

A systematic analysis of pneumatosis cystoids intestinalis

AIM:To increase the understanding,diagnosis and treatment of pneumatosis cystoides intestinalis(PCI)and to find the characteristics and potential cause of the disease in China.METHODS:We report here one case of PCI in a 70-year-old male patient who received a variety of treatment methods.Then,we systematically searched the PCI eligible literature published from an available Chinese database from May 2002 to May 2012,including CBM,CBMDisc,CMCC,VIP,Wanfang,and CNKI.The key words were pneumatosis cystoides intestinalis,pneumatosis,pneumatosis intestinalis,pneumatosis coli and mucosal gas.The patients' information,histories,therapies,courses,and outcomes were reviewed.RESULTS:The study group consisted of 239 PCI cases(male:female = 2.4:1)from 77 reported incidents.The mean age was 45.3 ± 15.6 years,and the median illness course was 6 mo.One hundred and sixty patients(66.9%)were in high altitude areas.In addition,43.5%(104/239)of the patients had potential PCI-related disease,and 16.3% had complications with intestinal obstruction and perforation.The most common symptom was abdominal pain(53.9%),followed by diarrhea(53.0%),distention(42.4%),nausea and vomiting(14.3%),bloody stool(12.9%),mucous stool(12.0%)and constipation(7.8%).Most multiple pneumocysts developed in the submucosa of the colon(69.9%).The efficacy of the treatments by combined modalities,surgery,endoscopic treatment,conservative approach,oxygen,and antibiotics were 100%,100%,100%,93.3%,68.3% and 26.3%,respectively.CONCLUSION:PCI can be safely managed by conservative treatments,presents more frequently in males,in the large bowel and submucosa,than in females,in the small intestine and subserosa.High altitude residence maybe associated with the PCI etiology.

Pneumatosis cystoides intestinalis following alpha-glucosidase inhibitor treatment:A case report and review of the literature

A 69-year-old man was diagnosed as having myasthenia gravis (MG) in September 2004,and treated with thymectomy and prednisolone. He was then diagnosed as having steroid-induced diabetes mellitus,and received sulfonylurea (SU) therapy in May 2005. An alpha-glucosidase inhibitor (αGI) was added in March 2006,resulting in good glycemic control. He experienced symptoms of abdominal distention,increased flatus,and constipation in October 2007,and was admitted into our hospital in late November with hematochezia. Plain abdominal radiography revealed small linear radiolucent clusters in the wall of the colon. Computed tomography (CT) showed intramural air in the sigmoid colon. Colonoscopy revealed multiple smooth surfaced hemispherical protrusions in the sigmoid colon. The diagnosis of pneumatosis cystoides intestinalis (PCI) was made on the basis of these findings. As the αGI voglibose was suspected as the cause of this patient's PCI,treatment was conservative,ceasing voglibose,with fasting and fluid supplementation. The patient progressed well,and was discharged 2 wk later. Recently,several reports of PCI associated with αGI therapy have been published,predominantly in Japan where αGIs are commonly used. If the use of αGIs becomes more widespread,we can expect more reports of this condition on a global scale. The possibility of PCI should be considered in diabetic patients complaining of gastrointestinal symptoms,and the gastrointestinal tract should be thoroughly investigated in these patients.

Pneumatosis cystoides intestinalis:A single center experience

AIM： To share our experience of the management and outcomes of patients with pneumatosis cystoides in- testinalis （PCI）. METHODS： The charts of seven patients who under- went surgery for PCI between 2001 and 2009 were re- viewed retrospectively. Clinical features, diagnoses and surgical interventions of patients with PCI are discussed. RESULTS： Seven patients with PCI （3 males, 4 fe- males; mean age, 50 ~ 16.1 years; range, 29-74 years） were analyzed. In three of the patients, abdominal pain was the only complaint, whereas additional vomiting and/or constipation occurred in four. Leukocytosis was detected in four patients, whereas it was within normal limits in three. Subdiaphragmatic free air was observed radiologically in four patients but not in three. Six of the patients underwent an applied lapa- rotomy, whereas one underwent an applied explorative laparoscopy. PCI localized to the small intestine only was detected in four patients, whereas it was localized to the small intestine and the colon in three. Three patients underwent a partial small intestine resection and four did not after PCI was diagnosed. Five patients were diagnosed with secondary PCI and two with pri- mary PCI when the surgical findings and medical his- tory were assessed together. Gastric atony developed in one case only, as a complication during a postopera- tive follow-up of 5-14 d. CONCLUSION： Although rare, PCl should be consid- ered in the differential diagnosis of acute abdomen. Diagnostic laparoscopy and preoperative radiological tests, including computed tomography, play an impor- tant role in confirming the diagnosis.

Gastrointestinal sarcoidosis associated with pneumatosis cystoides intestinalis

A 39-year-old male reported fevers,weight loss,watery loose stools,and decreased visual acuity in his right eye over the prior five years.He was pancytopenic,had an elevated American council on exercise level,total bilirubin,and alkaline phosphatase.Computed tomography revealed massive hepatosplenomegaly and emphysematous lung changes.Liver biopsy showed non caseating granulomas.The patient was diagnosed with extrapulmonary sarcoidosis and was treated with prednisone.The patient symptomatically improved but 5 mo later presented with abdominal pain caused by perforation of the cecum.He underwent a cecectomy and pathology revealed pneumatosis cystoides intestinalis.This represents the first reported association between pneumatosis cystoides intestinalis and sarcoidosis.The etiology of pneumatosis cystoides intestinalis in this case was likely multifactorial and involved both effects of the corticosteroids as well as the advanced nature of the gastrointestinal sarcoidosis.Furthermore this case has the unique features of emphysematous lung changes and pancytopenia which are uncommon with sarcoidosis.

Pneumatosis cystoides intestinalis associated with massive free air mimicking perforated diffuse peritonitis

While pneumatosis cystoides intestinalis （PCI） is a rare disease entity associated with a wide variety of gastrointestinal and non-gastrointestinal disorders, PCI associated with massive intra- and retroperitoneal free air is extremely uncommon, and is difficult to diagnose differentially from perforated peritonitis. We present two cases of PCI associated with massive peritoneal free air and/or retroperitoneal air that mimicked perforated peritonitis. These cases highlight the clinical importance of PCI that mimics perforated peritonitis, which requires emergency surgery. Preoperative imaging modalities and diagnostic laparoscopy are useful to make an accurate diagnosis.

Insights into the pathogenesis of cystoid macular edema: leukostasis and related cytokines

Cystoid macular edema(CME) is the abnormal collection of intraretinal fluid in the macular region, especially in the inner nuclear and outer plexiform layers. CME leads to severe visual impairment in patients with various retinal diseases, such as diabetic retinopathy, retinal vascular occlusion, choroidal neovascularization, and uveitis. Although various retinal conditions lead to CME, a shared pathogenesis of CME is involved in these diseases. Accordingly, the pathogenesis of CME based on vasogenic mechanisms is first discussed in this review, including vascular hyperpermeability, leukostasis, and inflammation. We then describe cytotoxic mechanisms based on retinal Müller cell dysfunction. This comprehensive review will provide an understanding of the pathogenesis of CME for potential therapeutic strategies.

Pneumatosis cystoides intestinalis after fluorouracil chemotherapy for rectal cancer

Pneumatosis cystoides intestinalis (PCI) is a relatively rare condition characterized by intraluminal gas in the gastrointestinal tract. Several chemotherapeutic agents have been reported to be associated with PCI, although fluorouracil-related PCI is extremely rare. We report a case of a 76-year old man who received adjuvant chemotherapy for rectal cancer with fluorouracil (FU) and leucovorin (LV). After 1 cycle of the treatment, he presented with diarrhea and abdominal pain. Abdominal radiogram revealed the presence of free air under the diaphragm and intramural gas in the intestine. Laparotomy was performed, showing a suspected diagnosis of perforation in the gastrointestinal tract. Intraoperative findings revealed penumatosis of the intestine without evidence of perforation. He was treated supportively and his symptoms improved. In conclusion, we should consider the possibility of PCI occurring in patients with malignancies during chemotherapy treatment.

Comparative study of the efficacy and safety of bromfenac, nepafenac and diclofenac sodium for the prevention of cystoid macular edema after phacoemulsification

AIM： To compare the efficacy, tolerability and safety of bromfenac 0.09%, nepafenac 0.1% or diclofenac 0.1% for the prophylaxis of the cystoid macular edema（CME） after phacoemulsification. METHODS： Group sequential observational comparative study. After phacoemulsification, patients received two months for topical treatment of either diclofenac sodium, bromfenac or nepafenac. All patients received concomitant topical tobramycin 0.3% and topical prednisolone 1%. We measured CME using optical coherence tomography（OCT） central foveal thickness, macular thickness and total macular volume. RESULTS： We enrolled 243 patients from January to June 2015, and 35% received diclofenac, 32.9% bromfenac and 32.1% nepafenac. When we compared pre-operative to three weeks to two months, bromfenac was more effective in reducing foveal volume（21.3 and 35.4 mm3, respectively）, compared with the diclofenac（1.3 and 11.5 mm3, respectively）, and the nepafenac group, became more edematous 6.4 and 5.3, respectively. Totally 133 patients completed the post-surgical satisfaction questionnaire. Patients complained of eye stickiness in 13.8% whom we gave nepafenac, versus 10.3% whom we gave diclofenac sodium, and in 0 whom we gave bromfenac. CONCLUSION： Bromfenac is the best tolerated and is more effective than diclofenac and nepafenac in reducing CME after phacoemulsification.

Pneumatosis cystoides intestinalis

Pneumatosis cystoides intestinalis(PCI) is a rare condition that may be associated with a variety of diseases.The presenting clinical picture may be very heterogeneous and represent a challenge for the clinician.In the present paper we describe both a common and an uncommon clinical presentation of PCI and review the pertaining literature.Our cases confirm that,apart from asymptomatic cases,the clinical presentation of PCI may be widely different and suggest that a new onset of stipsis might be the presenting symptom.Diagnosis might be suggested by a simple X-ray of the digestive tract showing a change in the characteristics of the intestinal wall in two-thirds of these patients.However,one third of the patients do not have a suggestive X-ray and require a computed tomography(CT) scan/nuclear magnetic resonance that may reveal a thickened bowel wall containing gas to confirm the diagnosis and distinguish PCI from intraluminal air or submucosal fat.CT also allows the detection of additional findings that may suggest an underlying,potentially worrisome cause of PCI such as bowel wall thickening,altered contrast mucosal enhancement,dilated bowel,soft tissue stranding,ascites and the presence of portal air.Our results also point out that clinicians and endoscopists should be aware of the possible presentations of PCI in order to correctly manage the patients affected with this disease and avoid unnecessary surgeries.The increasing number of colonoscopies performed for colon cancer screening makes PCI more frequently casually encountered and/or provoked,therefore the possible endoscopic appearances of this disease should be well known by endoscopists.

Combined Descemet stripping automated endothelial keratoplasty and intravitreal dexamethasone implant for concomitant pseudophakic bullous keratopathy and cystoid macular edema

Dear Editor,Endothelial cell density decreases with age and in various ocular conditions,including corneal endotheliitis,uveitis,pseudoexfoliation syndrome,and birth injury(1)The reduction of endothelial cell density is exacerbated over time after intraocular surgery(1)Descemet stripping automated endothelial keratoplasty(DSAEK)is considered the primary procedure for patients with only endothelial dysfunction.

Pneumatosis cystoides intestinalis associated with toxic epidermal necrolysis: A case report

Toxic epidermal necrolysis(TEN) is a severe adverse drug reaction, which is characterized by erythema, blisters, and/or erosions of the mucous membranes and skin, but intestinal involvement is rare. In contrast, pneumatosis cystoides intestinalis(PCI) is a rare condition associated with a wide variety of underlying diseases, but to date no patient has presented with PCI associated with TEN. A 55-year-old man was admitted to intensive care unit for treatment of TEN caused by phenobarbital. On day 8 after admission, he presented with progressive abdominal distention and hypotension. Computed tomography(CT) showed gas in the superior mesenteric vein and air filled cysts in the walls of the small intestine. He was suspected of having septic shock due to PCI. As there were no indications of bowel ischemia or necrosis, the patient was managed conservatively with antibiotics and oxygen therapy. On day 10 after admission, he was weaned off catecholamines, with CT on day 11 showing complete resolution of gas in the superior mesenteric vein and air filled cysts. To our knowledge, this article describes the first patient presenting with PCI associated with TEN.

Efficacy of topical dorzolamide 2% in diabetic cystoid macular edema

AIM:To study the effect of topical dorzolamide 2%on macular thickness reduction in diabetic cystoid macular edema(CME).METHODS:This was a prospective,non-randomized,open study including eyes with diabetic macular edema(DME).All eyes received topical dorzolamide 2%three times daily for one month.Changes in best-corrected visual acuity(BCVA),and central macular thickness(CMT)by optical coherence tomography were evaluated at 1 wk,1,and 3 mo post-treatment.RESULTS:Ninety-three eyes(84 patients)were included.Mean±SD(log MAR)BCVA improved significantly from 1.08±0.26 pretreatment to 0.66±0.24 at 1 mo and 0.87±0.26 at 3 mo post-treatment(P<0.001 both).The mean±SD CMT was significantly reduced from 535.27±97.4μm at baseline to 357.43±125.8μm at 1 mo and 376.23±114.5μm at 3 mo post-treatment(P<0.001 both).No significant ocular or systemic side effects were recorded.CONCLUSION:Topical dorzolamide 2%results in significant improvement of mean BCVA and reduction of mean CMT at 3 mo post-treatment.It can be used as an effective,affordable,and safe therapy for treatment of nonrefractory diabetic CME.

Effect of nepafenac 0.1% on retinal thickness after cataract surgery in patients without risk factors for cystoid macular edema

AIM:To evaluate the effect of topical preoperative nepafenac 0.1%treatment on postoperative macular edema using optical coherence tomography(OCT)after uncomplicated cataract surgery.METHODS:Ninety eyes of 90 patients without any risk factors were included in the study.The patients were assigned to three groups:group 1,treated with topical prednisolone acetate 1%;group 2,treated with topical nepafenac 0.1%in addition to prednisolone acetate(1%);and group 3,those who started receiving nepafenac 0.1%treatment 3 d prior to surgery and continued the treatment postoperatively in addition to prednisolone acetate(1%).Central retinal thickness(CRT)and macular volume values were recorded using OCT at weeks 3 and 6.RESULTS:The increases in macular volume in the central 1 mm area after 3 and 6 wk were significantly lower in patients who used prophylactic topical nepafenac preoperatively(group 3)compared with those in group 1(P=0.028 and 0.008,respectively).No significant differences in the increase in macular volume and CRT were noted between groups 2 and 3(P>0.05).In group 1,the increases in macular volume in the central 3 mm area at weeks 3 and 6 were significantly higher than that in group 2 and 3(3rd week,P=0.004;6th week,P=0.005).CONCLUSION:Nepafenac 0.1%treatment in addition to topical steroids after uncomplicated cataract surgery reduce the increase in macular volume in the early postoperative period.

Foveal regeneration after resolution of cystoid macular edema without and with internal limiting membrane detachment:presumed role of glial cells for foveal structure stabilization

AIM: To document with spectral-domain optical coherence tomography the morphological regeneration of the fovea after resolution of cystoid macular edema(CME) without and with internal limiting membrane(ILM) detachment and to discuss the presumed role of the glial scaffold for foveal structure stabilization. METHODS: A retrospective case series of 38 eyes of 35 patients is described. Of these, 17 eyes of 16 patients displayed foveal regeneration after resolution of CME, and 6 eyes of 6 patients displayed CME with ILM detachment. Eleven eyes of 9 patients displayed other kinds of foveal and retinal disorders associated with ILM detachment. RESULTS: The pattern of edematous cyst distribution, with or without a large cyst in the foveola and preferred location of cysts in the inner nuclear layer or Henle fiber layer(HFL), may vary between different eyes with CME or in one eye during different CME episodes. Large cysts in the foveola may be associated with a tractional elevation of the inner foveal layers and the formation of a foveoschisis in the HFL. Edematous cysts are usually not formed in the ganglion cell layer. Eyes with CME and ILM detachment display a schisis between the detached ILM and nerve fiber layer(NFL) which is traversed by Müller cell trunks. ILM detachment was also found in single eyes with myopic traction maculopathy, macular pucker, full-thickness macular holes, outer lamellar holes, and glaucomatous parapapillary retinoschisis, and in 3 eyes with Müller cell sheen dystrophy(MCSD). As observed in eyes with MCSD, cellophane maculopathy, and macular pucker, respectively, fundus light reflections can be caused by different highly reflective membranes or layers: the thickened and tightened ILM which may or may not be detached from the NFL, the NFL, or idiopathic epiretinal membranes. In eyes with short single or multiple CME episodes, the central fovea regenerated either completely, which included the disappearance of irregularities of the photoreceptor layer lines and the reformation of a fovea externa, or with remaining irregularities of the photoreceptor layer lines. CONCLUSION: The examples of a complete regeneration of the foveal morphology after transient CME show that the fovea may withstand even large tractional deformations and has a conspicuous capacity of structural regeneration as long as no cell degeneration occurs. It is suggested that the regenerative capacity depends on the integrity of the threedimensional glial scaffold for foveal structure stabilization composed of Müller cell and astrocyte processes. The glial scaffold may also maintain the retinal structure after loss of most retinal neurons as in late-stage MCSD.

Selective Laser Trabeculoplasty Complicated by Cystoid Macular Edema:Report of Two Cases

Purpose: Selective laser trabeculoplasty,a relatively novel treatment for open angle glaucoma,is frequently associated with mild post-operative intraocular inflammation. Methods: We report two uncommon cases of cystoid macular edema within a few weeks of routine selective laser trabeculoplasty. Results:Visual acuities and macular thicknesses of the two cases returned to baseline after medical treatment,but in one case, the cystoid macular edema persisted for months. Conclusion:Cystoid macular edema after selective laser trabeculoplasty is fortunately a rare complication, but it might be more common in patients with predisposing factors, and it can be resistant to treatment.

Combinative approach of transzonular triamcinolonemoxifloxacin and perioperative drops to minimize postoperative complications of cataract surgery

AIM: To investigate the effectiveness of combinationtherapy with transzonular triamcinolone-moxifloxacin andconventional perioperative drops in reducing postoperativecomplications of cataract surgery. METHODS: Electronic medical records of cataractsurgery patients (single surgeon) were reviewed fromJanuary 2018 to September 2021. The rate of postoperativecomplications including prolonged and/or recurrentpostoperative inflammation, endophthalmitis, cystoid macularedema (CME), and intraocular pressure (IOP) was comparedbetween the patients receiving combinative therapy andpatients receiving drops only. RESULTS: Totally 596 patients and 1057 eyes(Combinative-Therapy group 493 and Drop-Only group 564)were included in this study. Using combination therapyreduced the relative risk of postoperative inflammationby 26.9% (16.6% Combinative-Therapy vs 22.7% Drop-Only, P=0.013). The incidence of endophthalmitis was 0in Combinative-Therapy group vs 0.5% in Drop-Only group(relative risk reduction 100%), although not statisticallysignificant (P=0.10). The incidence of severe IOP spikeswas not significantly different between Combinative-Therapy (2.4%) and Drop-Only (1.6%) groups (P=0.33).The relative risk of postoperative CME was 51.4% less inthree months follow up visit in Combinative-Therapy group,although not statistically significant (P=0.07). The visualoutcome 1-month postop. (best corrected visual acuity) wassignificantly better in Combinative-Therapy (logMAR 0.10)compared to Drop-Only (logMAR 0.14) groups (P=0.02) whilethe baseline visual acuity was not significantly different. CONCLUSION: The combinative approach oftranszonular triamcinolone-moxifloxacin plus perioperativeeyedrops is an effective method to minimize postoperativeinflammation, with better visual outcomes. It couldpotentially reduce the risk of postoperative endophthalmitisand CME (near-significant P-values;larger studies couldanalyze better considering low incidence).

雷珠单抗与康柏西普治疗视网膜静脉阻塞继发黄斑囊样水肿的疗效比较

目的观察并比较雷珠单抗与康柏西普在视网膜静脉阻塞(retinal vein obstruction,RVO)继发黄斑囊样水肿(cystoid macular edema,CME)治疗中的疗效。方法非随机选取2022年1月—2023年12月东台市中医院收治的120例RVO继发CME患者为研究对象,依据治疗方法不同分成两组,研究组接受雷珠单抗治疗(n=61),对照组接受康柏西普治疗(n=59)。对比两组的视力水平及黄斑中心凹视网膜厚度的改善程度、并发症发生率。结果研究组疗后6个月最佳矫正视力水平为0.48±0.08,黄斑中心凹视网膜厚度为(295.24±10.85)μm,优于对照组的0.39±0.07、(315.12±11.69)μm,差异有统计学意义(t=2.449、2.323,P均<0.05)。两组临床疗效及并发症发生率比较,差异无统计学意义(P均>0.05)。结论雷珠单抗、康柏西普用于RVO继发CME的疗效差异性甚微,比较而言前者的效果较好,治疗中可根据患者的实际情况酌情选择。

Rare etiology of colonic intussusception involving an adult with emphysematous cystic enteropathy:A case report and review of literature

BACKGROUND Pneumatosis cystoides intestinalis(PCI),characterized by a collection of gas-filled cysts in the intestinal wall,is an uncommon but well-known condition in gastroenterology.Abdominal pain is the most frequent symptom associated with PCI.Intussusception represents a potential cause of recurrent abdominal pain or emergency presentation.However,the occurrence of colonic intussusception secondary to PCI is very unusual in adulthood.CASE SUMMARY A 52-year-old male,known with idiopathic PCI,presented seventeen months after initial diagnosis with a new right upper quadrant pain.A computed tomographyscan demonstrated a colonic intussusception at the hepatic flexure.PCI did not progress compared with initial investigation.The patient underwent an emergency right hemicolectomy.CONCLUSION Resection was recommended in this case because PCI proved to be persisting with no identified curable cause.Surgery allowed to address the underlying pathology,the potential relapse of intussusception,and the likely cause of recurrent abdominal pain,either invagination or PCI itself.

肠壁囊样积气症的MSCT征象及其在临床诊治决策中作用的探讨

目的:通过比较采用不同临床治疗方案的肠壁囊样积气症(PCI)患者多层螺旋CT(MSCT)征象的差异,探讨MSCT征象在PCI临床诊治决策中的作用,从而为其临床应用提供参考依据。方法:回顾性分析宁夏回族自治区人民医院2016年1月至2022年8月期间治疗的44例PCI患者资料,根据治疗方式分为保守组(34例)和手术组(10例),总结其MSCT征象特点,包括病变部位、气囊肿的大小、累计长度、在肠壁的分布、有无游离气体、门静脉积气、肠梗阻等,并比较两组间上述MSCT征象出现率的差异。结果:(1)MSCT征象:44例PCI肠壁囊样积气症气囊肿直径为0.1~3.7 cm,病变累计长度约为1.0~180 cm,1例为单发,其余为多发;浆膜下型13例(29.5%),黏膜下型15例(34.1%),混合型16例(36.4%);累及结直肠31例(70.5%),累及小肠19例(43.2%),累及胃壁、系膜处各1例(2.3%);伴有腹腔游离气体14例(31.8%);伴有肠梗阻14例(31.8%)。(2)两组间患者MSCT征象的差异:手术组与保守组之间病灶呈串珠样(90.0%vs 29.4%)、伴发门脉积气(60.0%vs 5.9%)、伴发肠梗阻(70.0%vs 20.6%)及伴发腹腔游离气体(70.0%vs 20.6%)等征象的出现率,差异均有统计学意义(P<0.05),而病变生长方式、生长部位在两组间则无统计学差异(P>0.05)。结论:PCI患者在MSCT上具有特征性表现;MSCT的征象特点可为PCI的临床治疗方案选择提供参考依据,当病灶呈串珠样改变,伴发门脉积气、肠梗阻及腹腔游离气体时要结合临床表现考虑手术可能性。