Aggressive Surgical Management Research Articles

Emergency decompressive surgery in patients with transtentorial brain herniation and pupillary abnormalities: the importance of improved pupillary response after osmotherapy and surgery.

The predictors of survival and functional recovery following emergency decompressive surgery in patients with transtentorial brain herniation, particularly those with pupillary abnormalities, have not been established. In this study, the authors aimed to assess the outcome of patients with intracranial mass lesions, transtentorial brain herniation, and nonreactive mydriasis, following emergency surgical decompression. A retrospective chart review was performed of all patients with transtentorial herniation and pupillary abnormalities who underwent craniotomy or craniectomy at two trauma and stroke centers between 2016 and 2022. The functional outcome was determined using the modified Rankin Scale (mRS). Forty-three patients, 34 men and 9 women with a mean age of 47 years (range 16-92 years), were included. The underlying etiology was traumatic brain injury in 33 patients, hemorrhagic stroke in 8 patients, and tumor in 2 patients. The median preoperative Glasgow Coma Scale score was 3 (range 3-8), and the median midline shift was 9 mm (range 1-29 mm). Thirty-two patients (74.4%) had bilaterally fixed and dilated pupils. The median time to surgery (from pupillary changes) was 133 minutes (mean 169 minutes, range 30-900 minutes). Eighteen patients (41.9%) died postoperatively. After a median follow-up of 12 months (range 3-12 months), 11 patients (26.8%) had a favorable functional outcome, while 10 remained severely disabled (mRS score 5). On univariate analysis, younger age (p < 0.001), less midline shift (p = 0.049), and improved pupillary response after osmotic therapy (p < 0.01) or decompressive surgery (p < 0.001) were associated with favorable outcomes at 3 months. With aggressive medical and surgical management, patients with transtentorial brain herniation, including those with bilaterally fixed and dilated pupils, may have considerable rates of survival and functional recovery. Young age, less midline shift, and improved pupillary response following osmotic therapy or decompressive surgery are favorable prognosticators.

The Current Challenges in Diagnosing and Treating Malignant Priapism: A Comprehensive Review.

Malignant priapism (MP) is defined as a condition of persistent erection of the penis without sexual stimulation due to the neoplastic process of the cavernous sinus and the efferent veins. The effectiveness of established therapeutic recommendations in priapism was ineffective in MP. Modalities of therapy for MP varied from medication treatment, nonsurgical treatment, surgical treatment, and radiotherapy. Despite aggressive surgical management combined with radiation therapy, chemotherapy, or targeted therapy, the survival rate remains low. Therefore, the treatment is usually palliative, focusing on the patient's quality of life improvement and symptom relief.

Risk factors associated with progression from papilloedema to optic atrophy: results from a cohort of 113 patients

BackgroundThe aim of this study was to assess the risk factors for atrophic progression of patients with papilloedema secondary to intracranial hypertension, using optical coherence tomography parameters.MethodsA retrospective study was...

Role of aggressive locoregional surgery in treatment strategies for ipsilateral supraclavicular lymph node metastasis of breast cancer: a real-world cohort study.

Background: Breast cancer patients with synchronous ipsilateral supraclavicular lymph node metastases (ISLNM) have unfavorable prognoses. The role of supraclavicular lymph node dissection (SLND) as a surgical intervention in the treatment of this condition remains controversial. In this study, we aimed to evaluate the prognostic factors associated with breast cancer with ISLNM and to assess the potential impact of aggressive locoregional surgical management on patient outcomes. Methods: We conducted a retrospective analysis of 250 breast cancer patients with ISLNM who were treated with curative intent at our institution between 2000 and 2020. The cohort was stratified into groups based on the extent of axillary surgery. The first group, comprising 185 patients, underwent level I/II axillary dissection. The second group, consisting of 65 patients, underwent aggressive locoregional surgery, including levels I/II/III (infraclavicular) dissection in 37 patients and levels I/II/III + SLND in 28 patients. Our study evaluated overall survival (OS) and disease-free survival (DFS) as primary endpoints, and locoregional recurrence-free survival (LRRFS) and distant metastasis-free survival (DMFS) as secondary endpoints. Results: The median follow-up time among all patients was 5.92years (1.05-15.36years). The 5-year OS rate was 71.89%, while the DFS rate, LRRFS rate, and DMFS rates were 59.25%, 66.38%, and 64.98%, respectively. A significant difference in OS, DFS, LRRFS, and DMFS was observed between the second group and the first group (p < 0.01). No beneficial impact on recurrence, metastasis, or survival outcomes was observed in the levels I/II/III + SLND group compared to the levels I/II/III dissection group. Multivariate logistic regression analysis revealed that levels I/II/III ± SLND surgery and T stage were associated with OS (p = 0.006 and p = 0.026), while levels I/II/III ± SLND surgery, ER+/HER2-, and histologic grade were associated with DFS (p = 0.032, p = 0.001, p = 0.032). Conclusion: Breast cancer with ISLNM may be considered a locoregional disease, requiring a combination of systemic and local therapies. Aggressive locoregional surgery has been shown to positively impact recurrence, metastasis, and survival outcomes. This approach may provide improved management of the ISLNM for breast cancer patients.

Long‐term clinical outcomes of Spitz‐type lesions in adults: A single institution retrospective cohort review

AbstractBackgroundThe lack of information on clinical outcomes of Spitz‐type lesions, means that their management remains a contentious issue, with some centres opting for a purely conservative, observational approach, while others mandate further surgery and long‐term follow‐up.ObjectivesOur aim was, therefore, to determine the long‐term clinical outcome of classic Spitz and Spitzoid naevi, atypical Spitz tumours (ASTs) and Spitzoid melanomas, with a view to finding evidence to support a unified management approach.MethodsWe conducted a retrospective cohort review of Addenbrooke's Hospital patients aged ≥18 years with a histopathologic diagnosis containing the term ‘Spitz’, from 1991 through 2020. Sentinel lymph node biopsy (SLNB) results, wide local excision (WLE) rates, metastases and fatality were assessed.ResultsOne hundred and twenty‐six patients with Spitz‐type proliferations and follow‐up information were identified (mean age: 35.9, SD: 11.6). There were 86 (68.3%) classic Spitz or Spitzoid naevi, 12 (9.5%) ASTs, 27 (21.4%) Spitzoid malignant melanomas and one (0.8%) melanoma that arose in a previously excised Spitz naevus. SLNB findings were positive in three of seven but none of the 10 individuals with ASTs and Spitzoid melanomas, respectively. Invasive non‐Spitz melanoma developed in four patients with classic Spitz or Spitzoid naevi. After a median follow‐up of 46 months (range: 1–304), only one patient with a Spitzoid melanoma, who had a different malignant melanoma (2.4 mm, level 4), developed distant metastases. No deaths were observed.ConclusionsSpitz‐type lesions are only rarely associated with mortality and distant metastasis, but CSNs may increase the risk of developing non‐Spitz‐type malignant melanoma. These findings support the suggestion that aggressive surgical management of these lesions such as SLNB and WLE may not be necessary, but surveillance should still continue to assess for subsequent development of malignant melanoma.

Adamantinomatous craniopharyngioma: evolution in the management.

In spite of the continuous progresses in pediatric neurosurgery, adamantinomatous craniopharyngioma (AC) remains a challenging tumor due to its proximity to optic pathways, pituitary gland, hypothalamus, and Willis' circle, which can result in significant endocrine, cognitive, and neurological morbidity after treatment with subsequent impact on the patient's quality of life (QoL). The relevance that QoL has today explains the changes in the management of AC observed over the time. The goal of the present article is to provide a historical background, to show the milestones in the changes of the AC treatment, and to analyze the current main options to manage such a challenging tumor. The pertinent literature has been reviewed. Moreover, a comparison between the past and recent personal series is reported. Three main eras have been identified. The first (named Cushing era) was characterized by the need to realize a harmless surgery and to define the best way to approach AC; the second (microscope era) was characterized by a tremendous technical and technological development, with remarkable results in term of safe tumor resection and control but relatively poor QoL outcomes; and the third one (current period) is characterized by an increasing integration between surgery and adjuvant treatments, with relatively minor tumor control but significant improvement of QoL (comparable overall survival). The authors' experience reflects these changes. Two groups of children were compared: 52 cases (mean follow-up: 17.5 years) belong to the historical series (group 1, 1985-2003, aggressive surgical management) and 41 (mean follow-up: 8.5 years) to the current one (Group 2, 2004-2021, integrated management). No significant differences between the two groups were detected about recurrence rate, surgical mortality, and overall survival. However, Group 2 showed significant lower rates of postoperative panhypopituitarism, obesity, and visual deterioration. Radical surgery allows for a good AC control with a low rate of recurrence but high risk of permanent morbidity. Despite the greater number of recurrences and surgeries, the more conservative policy, based on a combination of treatments, seems to provide the same tumor control with a better QoL. The advances in trans-nasal and trans-ventricular endoscopy, in proton therapy and in the management of the AC cyst are the main factors that allowed such an improvement.

Differentiated Thyroid Cancer in Adolescents: Single Center Experience and Considerations for Surgical Management and Radioiodine Treatment

Differentiated thyroid cancer (DTC) in adolescents rare but with a favorable outcome, despite higher rates of cervical lymph node and pulmonary metastasis compared to adults. The aim of this study was to critically evaluate treatment of adolescents with DTC at a single center. Patients receiving postoperative radioiodine treatment (RAIT) for DTC between 2005 and 2020 at our institution were screened to identify adolescents according to the World Health Organization definition (10-19 years of age). Demographics, clinico-pathological characteristics, treatment and outcome were analyzed. Among 1,897 DTC patients, 23 (1.3%) were adolescents with a median (range) age of 16 (10-18) years. The female to male ratio was 3.6:1. Sixty percent had classic papillary thyroid cancer, with follicular variant in 40%, which was higher than previously reported (15-25%) for this age group. pT-status was pT1 in 9 (39.2%), pT2 in 8 (34.8%), pT3 in 3 (13%) and pT4 in 3 (13%) patients. In 19 (82.6%) patients, central lymphadenectomy was performed and metastasis was seen in 57%. All patients received RAIT with initial activities of 1.2 (n=1, 4.3%), 2 (n=12, 52.2%) or 3.7 GBq (n=10, 43.5%). Eighteen (78.2%) patients were free of biochemical and radiologic disease at a median follow-up of 60.7 months. Second-line surgery for lymph node relapse was necessary in 3 (13%) cases. There was one disease-associated death. Despite high rates of metastasis, most patients were cured, and second-line surgery was rarely required. Further prospective studies are needed to determine whether less aggressive surgical management or omitting adjuvant RAIT are feasible in patients with limited stages at diagnosis.

Radical Cystectomy in the Treatment of Invasive Bladder Cancer: Long-Term Results in 1,054 Patients.

To evaluate our long-term experience with patients treated uniformly with radical cystectomy and pelvic lymph node dissection for invasive bladder cancer and to describe the association of the primary bladder tumor stage and regional lymph node status with clinical outcomes. All patients undergoing radical cystectomy with bilateral pelvic iliac lymphadenectomy, with the intent to cure, for transitional-cell carcinoma of the bladder between July 1971 and December 1997, with or without adjuvant radiation or chemotherapy, were evaluated. The clinical course, pathologic characteristics, and long-term clinical outcomes were evaluated in this group of patients. A total of 1,054 patients (843 men [80%] and 211 women) with a median age of 66 years (range, 22 to 93 years) were uniformly treated. Median follow-up was 10.2 years (range, 0 to 28 years). There were 27 (2.5%) perioperative deaths, with a total of 292 (28%) early complications. Overall recurrence-free survival at 5 and 10 years for the entire cohort was 68% and 66%, respectively. The 5- and 10-year recurrence-free survival for patients with organ-confined, lymph node-negative tumors was 92% and 86% for P0 disease, 91% and 89% for Pis, 79% and 74% for Pa, and 83% and 78% for P1 tumors, respectively. Patients with muscle invasive (P2 and P3a), lymph node-negative tumors had 89% and 87% and 78% and 76% 5- and 10-year recurrence-free survival, respectively. Patients with nonorgan-confined (P3b, P4), lymph node-negative tumors demonstrated a significantly higher probability of recurrence compared with those with organ-confined bladder cancers (P < .001). The 5- and 10-year recurrence-free survival for P3b tumors was 62% and 61%, and for P4 tumors was 50% and 45% , respectively. A total of 246 patients (24%) had lymph node tumor involvement. The 5- and 10-year recurrence-free survival for these patients was 35%, and 34%, respectively, which was significantly lower than for patients without lymph node involvement (P < .001). Patients could also be stratified by the number of lymph nodes involved and by the extent of the primary bladder tumor (p stage). Patients with fewer than five positive lymph nodes, and whose p stage was organ-confined had significantly improved survival rates. Bladder cancer recurred in 311 patients (30%) . The median time to recurrence among those patients in whom the cancer recurred was 12 months (range, 0.04 to 11.1 years). In 234 patients (22%) there was a distant recurrence, and in 77 patients (7%) there was a local (pelvic) recurrence. These data from a large group of patients support the aggressive surgical management of invasive bladder cancer. Excellent long-term survival can be achieved with a low incidence of pelvic recurrence.

Acute thrombosis of the lower limb arteries on the background of the XBB.1.5 ("Kraken") subvariant of omicron SARS-COV-2 - medical or surgical treatment?

Comparative analysis of the results of open thrombectomy and conservative therapy in patients with acute thrombosis of the lower limb arteries against the background of the XBB.1.5 ("Kraken") subvariant of Omicron Severe acute respiratory syndrome-related coronavirus-2 (Sars-cov-2). The present prospective, open, multicenter comparative study for the period 04/01/2022 to 12/01/2023 included 167 patients with acute thrombosis of the lower limb arteries against the background of the XBB.1.5 ("Kraken") subvariant of the XBB.1.5 ("Kraken") subvariant of Omicron Sars-cov-2.Depending on the treatment strategy, two groups were formed: group 1 - open thrombectomy (n = 136) and drug treatment with an anticoagulant (unfractionated heparin in a therapeutic dosage) and an antiplatelet (acetylsalicylic acid 125mg once a day); group 2 - only drug therapy (n = 31). This group consisted of patients who refused surgical revascularization and were on a prophylactic dose of unfractionated heparin (5000 U 3 times a day subcutaneously). On the development of acute arterial thrombosis, 80 U/kg (maximum 5000 U) of unfractionated heparin was injected intravenously in a bolus, followed by transfer to intravenous infusion using an infusion pump with an initial rate of 18 U/kg per hour under control of activated partial thromboplastin time. Analgesic and antiplatelet therapy was also prescribed (acetylsalicylic acid 125mg once a day). No significant intergroup differences either in mortality rates (group 1: n = 58, 38.2%; group 2: n = 7, 22.6%; p = 0.09) or limb amputation (group 1: n = 63, 46.3%; group 2: n = 9, 29.0%; p = 0.07) were found. However, there was a trend towards a decrease in the incidence of these events in the drug treatment group. After open thrombectomy, rethrombosis developed in 50.7% (n = 69) cases; and thrombosis after rethrombectomy followed by amputation in 46.3% (n = 63). There were no hemorrhagic complications in both groups. Myocardial infarction and ischemic strokes were not recorded. Anticoagulant therapy demonstrates several advantages over aggressive surgical management. However, probably due to the small size of the studied sample, significant differences were not obtained. Further study of this issue is required with the identification of the optimal reperfusion method in this complex cohort of patients.

Breast MRI for Extent of Disease: Association of Demographic Factors and Biopsy Compliance on Surgical Decisions in Patients with BI-RADS 4 and 5 Findings.

The decision to pursue bilateral mastectomy without pathological confirmation of additional preoperative MRI lesions is likely multifactorial. We investigated the association of demographic factors and biopsy compliance following preoperative breast MRI with changes in surgical management in patients with newly diagnosed breast cancer. A retrospective review of BI-RADS 4 and 5 MRIs performed across a health system from March 2018 to November 2021 for assessment of disease extent and preoperative planning. Patient characteristics, including demographics, Tyrer-Cuzick risk score, pathology from index cancer and biopsy of MRI findings, and pre- and post-MRI surgical plans were recorded. Analysis compared patients who underwent biopsy with those who did not. The final cohort included 323 patients who underwent a biopsy and 89 who did not. Of patients who underwent a biopsy, 144/323 (44.6%) had additional cancer diagnoses. MRI did not change management in 179/323 patients (55.4%) who underwent biopsy and in 44/89 patients (51.7%) who did not. Patients with a biopsy were more likely to have additional breast conservation surgery (P < .001) and patients without a biopsy were more likely to have a change in management to bilateral mastectomy P = .009). Patients without a biopsy who underwent a management change to bilateral mastectomy were significantly younger (47.2 vs 58.6; P < .001) and more likely to be white (P = .02) compared to those choosing bilateral mastectomy after biopsy. Biopsy compliance is associated with changes in surgical decisions, and younger, white women are more likely to pursue aggressive surgical management without definitive pathologic diagnoses.

Pott's Puffy: First Shot is the Best Shot.

Low incidence of Pott's Puffy tumor (PPT) has caused studying risk factors and recurrences of the disease to be difficult. We used the comparatively increased incidence at our institution to evaluate potential risk factors for the disease process itself and prognostic factors for recurrence of the disease. Single institutional retrospective chart review identified 31 patients from 2010 to 2022 with PPT compared with a control group of 20 patients with either chronic rhinosinusitis or recurrent sinusitis. Patient mean age of PPT was 42 (range of 5 to 90) with the majority of the patient population as male (74%) and Caucasian (68%) in the setting of rural West Texas. Patient mean age of the control group was 50.7 (range of 30-78) with majority of patient population as male (55%) and Caucasian (70%). Interventions studied were functional endoscopic sinus surgery (FESS), FESS with trephination, and cranialization with or without FESS to compare prognostic factors for recurrence rates of PPT. These patients' prognostic risk factors for recurrence and risk factors to develop PPT were analyzed using Analysis of Variance (ANOVA) χ 2 statistical analysis with Fischer exact testing. Mean age was 42 years (range of 5-90) with the majority of the PPT patient population as male (74%) and Caucasian (68%) with an overall incidence of about 1 in 300,000. Pott's Puffy tumor patients were significantly favored in the younger and male population compared with the control patients. Risk factors of no prior allergy diagnosis, previous trauma, medication allergy to penicillin class or cephalosporin class, and lower body mass index were significant in the PPT population compared with the control group. Significant prognostic factors for recurrence of PPT were prior history of sinus surgery and operative treatment choice. Fifty percent (3/6) of patients with prior sinus surgery had recurrence of PPT. Of our 4 treatment options (FESS, FESS with trephination, FESS with cranialization, or cranialization alone), ;FESS had a recurrence of PPT of 0% (0/13), FESS with trephination had a recurrence of PPT of 50% (3/6), FESS with cranialization had a recurrence of PPT of 11% (1/9), and cranizalization alone had a recurrence of PPT of 0% (0/3). Of note, postop chronic rhinosinusitis was seen in 46% (6/13) of FESS alone, 17% (1/6) with FESS with trephination, 0% (0/9) with FESS with cranialization, and 33% (1/3) with just cranialization alone. Pott's Puffy tumor patients were younger and predominately male when compared to the control patients. No prior allergy diagnosis, previous trauma history, medication allergy to penicillin class or cephalosporin class, and lower body mass index are risk factors for PPT. There are 2 prognostic factors that predict recurrence of PPT: first operative treatment choice and prior sinus surgery. History of prior sinus surgery tends to increase the recurrence of PPT. The first operative treatment plan is the best shot at definitively treating PPT. Correct management surgically can prevent recurrence of PPT as well as long-term recurrence of chronic rhinosinusitis. With early diagnosis and mild disease, FESS is sufficient to prevent recurrence of PPT but chronic sinusitis may continue to occur if frontal sinus outflow track is not well opened. If considering trephination, a definitive cranialization may be more suited for more advanced disease since our study showed 50% of recurrence of PPT with trephination and FESS along with 17% chronic sinusitis long term. More advanced diseases with higher WBCs and intracranial extension do better with more aggressive surgical management with a cranialization with or without FESS which shows to reduce rates of PPT recurrence significantly.

Disseminated uterine actinomycosis: Case report and review of literature

Actinomyces - It mainly affects the cervicofacial and abdominopelvic organs. These bacteria are of low virulence and their spread requires breakdown or alteration of the normal tissue barrier, which can be due to any surgery, trauma, or infection. Actinomyces infection involving the reproductive organs may often mimic a neoplasm and lead to aggressive surgical management for the patient; it is therefore necessary to recognize the infection which can be effectively managed using a combination of pharmaceutical agents. Imaging such as computed tomography and magnetic resonance imaging aid in determining the extent of the disease. Histopathological diagnosis/culture is necessary to make the final diagnosis. We present a case of actinomycosis of the uterus manifesting as a large uterine mass with dissemination to the pelvis and anterior abdominal wall.

A phase 2 study of neoadjuvant lenvatinib in locally advanced invasive thyroid cancer.

TPS6105 Background: Lenvatinib is a multiple receptor tyrosine kinase (RTK) inhibitor that has demonstrated meaningful benefit in radioiodine (RAI)-refractory differentiated thyroid cancer (DTC). In SELECT, lenvatinib significantly prolonged progression-free survival (PFS) and was associated with an overall response rate (ORR) of 65% in patients with RAI-refractory DTC, with the greatest degree of tumor shrinkage occurring in the first 2 cycles. The robust activity of lenvatinib in DTC suggests that neoadjuvant treatment in patients presenting with bulky neck disease may make surgery more feasible, allows for optimal surgical outcomes and less aggressive surgical management. Neoadjuvant lenvatinib to improve surgical outcomes in locally advanced DTC has not been studied to date. This phase 2 study will examine the efficacy and safety of lenvatinib prior to thyroidectomy, with the goal of achieving more favorable surgical outcomes. Methods: This is a Simon two-stage Phase 2 open-label multicenter clinical trial for adult patients who meet the following criteria: have been diagnosed with locally advanced or persistent/recurrent thyroid and/or cervical nodal DTC, deemed at risk for R2 resection (surgical margins with macroscopic tumor) (as evident by vocal cord paralysis by laryngoscopic exam, imaging documenting extrathyroidal/extranodal extension, or major vessels involvement), have measurable disease per RECIST v1.1, have an ECOG performance status ≤ 1, and no contraindication to surgery. A total of 28 patients will be enrolled from three centers (Massachusetts General Hospital/Massachusetts Eye &amp; Ear Infirmary (MGH/MEEI), Memorial Sloan-Kettering Cancer Center (MSKCC), and MD Anderson Cancer Center (MDACC)). Participants will receive up to 6 cycles of lenvatinib prior to surgery depending on response. Participants will undergo restaging at the end of cycle 2, 4, and 6. Participants with sufficient response to treatment who projected to achieve an R0 or R1 resection will stop lenvatinib and proceed to surgery within 7-10 days from the last dose. Participants who do not have a sufficient response who are tolerating treatment, will receive an additional 2-4 cycles of lenvatinib, followed by surgery. The primary objective is the R0 (surgical margins free from tumor)/R1 (surgical margins free from macroscopic tumor) resection rate. Secondary outcome measures include R0/R1 resection rates in each of 5 pre-specified anatomic target interfaces (perithyroid muscles, cartilage, esophagus, recurrent laryngeal nerve, major vessels), evaluation of the change in surgical morbidity complexity MGH/MEEI-MSKCC-MDACC (MMM) score and the Invasive Thyroid Class, ORR prior to surgery per RECIST v1.1, and the safety of lenvatinib in this patient population. The study is currently accruing with 11 patients enrolled at time of submission. Clinical trial information: NCT04321954 .

Pediatric Laryngeal Coccidioidomycosis: A Case Series in an Endemic Region.

Laryngeal coccidioidomycosis is a rare but life-threatening manifestation of coccidioidomycosis. Data in children are sparse and limited to case reports. We conducted this study to review the characteristics of laryngeal coccidioidomycosis in children. We performed a retrospective review of patients ≤21 years of age with laryngeal coccidioidomycosis who were treated from January 2010 to December 2017. We collected demographic data, clinical and laboratory studies and patient outcomes. Five cases of pediatric laryngeal coccidioidomycosis were reviewed. All children were Hispanic and 3 were female. The median age was 1.8 years and the median duration of symptoms before diagnosis was 24 days. The most common symptoms included fever (100%), stridor (60%), cough (100%) and vocal changes (40%). Airway obstruction requiring tracheostomy and/or intubation for airway management was present in 80%. The most frequent location of lesions was the subglottic area. Coccidioidomycosis complement fixation titers were frequently low and culture/histopathology of laryngeal tissue was necessary to make a definitive diagnosis. All patients required surgical debridement and were treated with antifungal agents. None of the patients had recurrence during the follow-up period. This study suggests that laryngeal coccidioidomycosis in children presents with refractory stridor or dysphonia and severe airway obstruction. Favorable outcomes can be achieved with a comprehensive diagnostic work-up and aggressive surgical and medical management. With the rise in cases of coccidioidomycosis, physicians should have a heightened awareness regarding the possibility of laryngeal coccidioidomycosis when encountering children who have visited or reside in endemic areas with stridor or dysphonia.

O143 Management strategies and outcomes for small bowel neuroendocrine tumours with involvement of the superior mesenteric vascular root: a systematic review

Abstract Introduction Small intestine neuroendocrine tumours (SI-NETs) are the most common small bowel tumours. A particularly challenging subset of these tumours are those that involve the superior mesenteric artery (SMA) or vein (SMV) for which the role and feasibility of surgery is often questioned. This review aimed to identify and evaluate the management strategies used for these complex SI-NETs. Methods Primary studies concerning SI-NETs with SMA and/or SMV involvement were identified from PubMed and Embase review and data abstraction was performed. Results Initial literature search identified 155 potentially eligible studies, from which nine non-randomised primary retrospective studies met inclusion criteria. Treatments identified include stenting the SMV, debulking and resection, auto-transplantation, and endovascular occlusion and tumour excision (EVOTE) procedures. The level of involvement of the SMA and SMV was associated with resectability such that more proximal involvement yielded a lower likelihood of successful resection. Auto-transplantation strategies may show promise in more proximal mesenteric root involvement. To date, one study reported a case of a patient with SMV encasement who was successfully treated with auto-transplantation of ileocolic and ileojejunal segments after resection of the affected structures. Conclusion The present study highlights a role for aggressive surgical and multimodal management in cases of SI-NETs with vascular involvement of the mesenteric root. The use of classification systems to stratify mesenteric masses by level of vascular involvement can help identify patients who could benefit from resection. Large scale prospective studies are needed to better elucidate the effectiveness of surgical management strategies in these challenging SI-NETs.

Infantile Fibromatosis of the Infratemporal Fossa Extending to Oropharynx

The Case Study describes the clinical radiographic Surgical Procedure with reconstruction and histopathology Infantile fibromatosis of infratemporal fossa extending to oral cavity and Oropharynx in a two-year-old male child. This condition is rarer in two-year-old child mainly in infratemporal fossa extending to Oropharynx and protruding into oral cavity leading to tremendous suffering of the child for breast feeding, eating with occasional profuse bleeding. The differential diagnosis, management and long term follow up for two years are also mentioned. The need for aggressive surgical management, rare site and operability in this region is very rarer of rarest. The absence of recurrence after 2 years follow up is significant and we can declare the patient is cured from the primary disease. This paper gives guide to how to approach the disease process.

Genetic Problems, Diagnosis, and Cardiovascular Manifestations of Loeys-Dietz Syndrome.

Loeys-Dietz Syndrome (LDS) is an autosomal dominant connective tissue disorder with multisystem involvement of wide spectrum, found to be associated with transforming growth factor-β pathway. LDS is characterized by craniofacial, skeletal, cutaneous, vascular abnormalities along with aortic aneurysm and aortic dissection contributing to mortality and morbidity at a young age. Therefore, timely diagnosis and intervention in patients with LDS is vital. Several gene mutations have been described as contributing factors of LDS, causing widespread and aggressive vascular disease. Based on these gene mutations, 5 types of LDS have been described so far. Besides aortic aneurysm and dissection, some of the other cardiac manifestations of LDS involve cardiomyopathy, valvular abnormality, atrial fibrillation, patent ductus arteriosus, atrial septal defects, etc. Routine imaging of patients' vasculatures and aggressive medical and surgical management are key factors in managing patients with LDS.

Epidemiology, management, and clinical outcomes of extrapulmonary Mycobacterium abscessus complex infections in heart transplant and ventricular assist device recipients

Nontuberculous mycobacteria are emerging pathogens, yet data on the epidemiology and management of extrapulmonary nontuberculous mycobacteria infections in orthotopic heart transplantation (OHT) and ventricular assist device (VAD) recipients are scarce. We retrospectively reviewed records of OHT and VAD recipients who underwent cardiac surgery at our hospital and developed Mycobacterium abscessus complex (MABC) infection from 2013 to 2016 during a hospital outbreak of MABC linked to heater-cooler units. We analyzed patient characteristics, medical and surgical management, and long-term outcomes. Ten OHT patients and 7 patients with VAD developed extrapulmonary M. abscessus subspecies abscessus infection. The median time from presumed inoculation during cardiac surgery to the first positive culture was 106 days in OHT and 29 days in VAD recipients. The most common sites of positive cultures were blood (n = 12), sternum/mediastinum (n = 8), and the VAD driveline exit site (n = 7). The 14 patients diagnosed when alive received combination antimicrobial therapy for a median of 21 weeks, developed 28 antibiotic-related adverse events, and underwent 27 surgeries. Only 8 (47%) patients survived longer than 12 weeks after diagnosis, including 2 patients with VAD who experienced long-term survival after an explantation of infected VADs and OHT. Despite aggressive medical and surgical management, OHT and VAD patients with MABC infection experienced substantial morbidity and mortality.

Selective sonographic screening for developmental dysplasia of the hip – increasing trends in late diagnosis

There are concerns that selective sonographic screening for developmental dysplasia of the hip (DDH) may be suboptimal. Our aim was to test this hypothesis by identifying trends in presentation and surgical treatment in patients with DDH. This is a retrospective review of children born between 1997-2018 who were treated surgically for DDH at our sub- regional paediatric orthopaedic unit. Demographic data, risk factors, age of diagnosis and surgical treatments were analysed. Late diagnosis was defined as greater than 4 months. 103 children (14 male, 89 female) underwent surgery. 93 hips were operated for dislocation and 21 for dysplasia. 13 patients presented with bilateral hip dislocations. The median age at diagnosis was 10 months (95% CI: 4-15). 62/103 (60.2%) were diagnosed late (after 4 months) and the median age for diagnosis in this group was 18.5 months (95% CI: 16-20.5). Significantly more patients were referred late (p=0.0077). The presence of risk factors (breech presentation or family history) was associated with early diagnosis. Over the duration of our study the operation rate per 1000 live births gradually increased, and on Poisson regression analysis there was a statistically significant increasing trend towards late diagnosis in recent years (p=0.0237), which necessitated more aggressive surgical management. In the UK, the current selective sonographic screening programme for DDH has shown a deterioration over the years of this study and this questions its current effectiveness. It appears that the majority of irreducible hip dislocations are diagnosed late, with an increased need for surgical management.

Palatal Mucoperiosteum-A Redeemable Barrier in Surgical Management of Rhinocerebral Mucormycosis.

Ever since the commencement of the COVID-19 pandemic, post-recovery complications have been in the highlights, out of which rhinocerebral mucormycosis tops the chart. The surgical management of this disease was always aggressive debridement, resection, and antifungal treatment. Oronasal communication after aggressive surgical management always leads to breathing, feeding, and cosmetic impairments. In this study, cases were managed by leaving the palatal mucosa intact after the removal of all the affected hard and soft tissues including the periosteum attached to the mucosa, to preserve the palatal mucoperiosteum and to improve the postoperative complications in patients by maintaining the oronasal separation. Prospective review of the operated case of rhinocerebral mucormycosis in 20 patients was presented. An intraoral approach for hard and soft tissue resection was employed. The palatal mucoperiosteum was salvaged in all patients. Postoperative complications like oronasal communication, ill-fitting prosthesis, and healing were evaluated. A total of 20 patients underwent maxillectomy for mucormycosis involving the maxilla and palate along with other facial bones. The palatal mucosa was preserved and used for oronasal separation. The expected complications of nasal regurgitation, crustation of the maxillary cavity, ill-fitting prosthesis, etc. were evaded due to this. The administration of Inj. liposomal amphotericin B (LiAB) postsurgically has been the backbone of this procedure.