scholarly journals Triple Atresia, Triple Threat? An Unusual Constellation of Primary Surgical Abnormalities

2021 ◽  
Vol 13 (2) ◽  
pp. 189-196
Author(s):  
Raef Jackson ◽  
Carmen Francis ◽  
Karim Awad ◽  
Semiu E. Folaranmi

We present a case series of two patients with tracheo-oesophageal fistula with oesophageal atresia (TOF/OA), duodenal atresia (DA) and ano-rectal malformation (ARM). This constellation of abnormalities, dubbed triple atresia (TA), is a rare combination with few described cases in the literature. Here we describe our management of these cases, as well as the results of our literature review. Both of our cases had staged surgical procedures and were initially managed with thoracotomy for repair of TOF/OA on day two of life. They subsequently underwent laparotomy for management of their abdominal pathology at day five and seven of life. Both have survived the neonatal period and are awaiting definitive surgery for ARM. Literature review yielded seven cases of TA involving a TOF, DA, and ARM. Four patients underwent staged repair, while three patients underwent repair of TOF/OA, DA and colostomy for ARM at the same time. Of these three patients, two died, representing 22% of the overall cohort. Triple atresia remains a rare subset of patients suspected to have VACTERL association, however mortality may be significantly higher. Our data would suggest a staged approach to be optimal for long term survival.

Author(s):  
Maria Enrica Miscia ◽  
Giuseppe Lauriti ◽  
Dacia Di Renzo ◽  
Angela Riccio ◽  
Gabriele Lisi ◽  
...  

Abstract Introduction Esophageal atresia (EA) is associated with duodenal atresia (DA) in 3 to 6% of cases. The management of this association is controversial and literature is scarce on the topic. Materials and Methods We aimed to (1) review the patients with EA + DA treated at our institution and (2) systematically review the English literature, including case series of three or more patients. Results Cohort study: Five of seventy-four patients with EA had an associated DA (6.8%). Four of five cases (80%) underwent primary repair of both atresia, one of them with gastrostomy placement (25%). One of five cases (20%) had a delayed diagnosis of DA. No mortality has occurred. Systematic Review: Six of six-hundred forty-five abstract screened were included (78 patients). Twenty-four of sixty-eight (35.3%) underwent primary correction of EA + DA, and 36/68 (52.9%) underwent staged correction. Nine of thirty-six (25%) had a missed diagnosis of DA. Thirty-six of sixty-eight underwent gastrostomy placement. Complications were observed in 14/36 patients (38.9 ± 8.2%). Overall mortality reported was 41.0 ± 30.1% (32/78 patients), in particular its incidence was 41.7 ± 27.0% after a primary treatment and 37.0 ± 44.1% following a staged approach. Conclusion The management of associated EA and DA remains controversial. It seems that the staged or primary correction does not affect the mortality. Surgeons should not overlook DA when correcting an EA.


2021 ◽  
Vol 9 (3) ◽  
pp. 47
Author(s):  
Marcus Fernando Kodama Pertille Ramos ◽  
Marina Alessandra Pereira ◽  
Arthur Youssif Mota Arabi ◽  
Melissa Mello Mazepa ◽  
Andre Roncon Dias ◽  
...  

Background: Mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) represent a rare tumor composed of adenocarcinoma and neuroendocrine carcinoma components. This study reports a case series of gastric MiNEN and discusses issues related to its diagnosis, management, and outcomes. Methods: We retrospectively analyzed data from patients with gastric MiNEN who underwent surgical resection at our service from 2009 to 2020. Patients with gastric adenocarcinoma served as a comparison group. Clinical, pathologic, and surgical characteristics were compared. Results: During the selected period, 5 gastric MiNEN patients and 597 patients with gastric adenocarcinoma were included. Among the clinical variables, age, sex, BMI, and laboratory exams were similar between the two groups. Only ASA classification was different (p = 0.015). Pathological variables such as tumor size, lymphovascular invasion, number of retrieved lymph nodes, and pTNM staging were also similar between both groups. Lastly, early surgical outcomes and long-term survival did not differ between gastric MiNEN and adenocarcinoma patients. Conclusion: A MiNEN is a rare tumor that represents less than 1% of GC patients undergoing curative treatment, and demonstrated clinicopathological characteristics and outcomes similar to gastric adenocarcinoma.


Author(s):  
Kevin C. Miller ◽  
John P. Marinelli ◽  
Jeffrey R. Janus ◽  
Ashish V. Chintakuntlawar ◽  
Robert L. Foote ◽  
...  

AbstractEsthesioneuroblastoma (ENB) is a rare olfactory malignancy that can present with locally advanced disease. At our institution, patients with ENB in whom the treating surgeon believes that a margin-negative resection is initially not achievable are selected to undergo induction with chemotherapy with or without radiotherapy prior to surgery. In a retrospective review of 61 patient records, we identified six patients (10%) treated with this approach. Five of six patients (83%) went on to definitive surgery. Prior to surgery, three of five patients (60%) had a partial response after induction therapy, whereas two of five (40%) had stable disease. Microscopically margin-negative resection was achieved in four of five (80%) of the patients who went on to surgery, while one patient had negative margins on frozen section but microscopically positive margins on permanent section. Three of five patients (60%) recurred after surgery; two of these patients died with recurrent/metastatic ENB. In summary, induction therapy may facilitate margin-negative resection in locally advanced ENB. Given the apparent sensitivity of ENB to chemotherapy and radiotherapy, future prospective studies should investigate the optimal multidisciplinary approach to improve long-term survival in this rare disease.


2020 ◽  
Vol 2020 ◽  
Author(s):  
Sara Ricciardi ◽  
Federico Davini ◽  
Carmelina C. Zirafa ◽  
Gaetano Romano ◽  
Franca M. A. Melfi

HPB Surgery ◽  
2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
K. S. Norman ◽  
S. R. Domingo ◽  
L. L. Wong

Background. Chronic kidney disease affects 20 million US patients, with nearly 600,000 on dialysis. Long-term survival is limited and the risk of complex pancreatic surgery in this group is questionable. Previous studies are limited to case reports and small case series and a large database may help determine the true risk of pancreatic surgery in this population. Methods. The American College of Surgeons National Surgical Quality Improvement Program database was queried (2005–2011) for patients who underwent pancreatic resection. Renal failure was defined as the clinical condition associated with rapid, steadily increasing azotemia (rise in BUN) and increasing creatinine above 3 mg/dL. Operative trends and short-term outcomes were reviewed for those with and without renal failure (RF). Results. In 18,533 patients, 28 had RF. There was no difference in wound infections, neurologic or cardiovascular complications. Compared to non-RF patients, those with RF had more unplanned intubation (OR 4.89, 95% CI 1.85–12.89), bleeding requiring transfusion (OR 3.12, 95% CI 1.37–14.21), septic shock (OR 8.86, 95% CI 3.75–20.91), higher 30-day mortality (21.4% versus 2.3%, P<0.001) and longer hospital stay (23 versus 12 days, P<0.001). Conclusions. RF patients have much higher morbidity and mortality after pancreatic resections and surgeons should consider this before proceeding.


2019 ◽  
Vol 7 (11) ◽  
pp. 2092-2101 ◽  
Author(s):  
Abdelkader Taibi ◽  
Jeremie Jacques ◽  
Sylvaine Durand Fontanier ◽  
Aurelie Charissoux ◽  
Sylvia M. Bardet ◽  
...  

2017 ◽  
Vol 4 (4) ◽  
pp. 255-262
Author(s):  
Ryan Rhome ◽  
Isabelle M Germano ◽  
Ren-Dih Sheu ◽  
Sheryl Green

Abstract Background Growth hormone (GH)-secreting pituitary adenomas represent an uncommon subset of pituitary neoplasms. Stereotactic radiosurgery (SRS) and fractionated stereotactic radiotherapy (FSRT) have been used as primary or adjuvant treatment. The purpose of this study is to report the long-term tumor control and toxicity from our institution and to perform a systematic literature review of acromegaly patients treated with FSRT. Methods We retrospectively reviewed all patients treated with FSRT (median dose 50.4 Gray [Gy], range 50.4–54 Gy) between 2005 and 2012 who had: 1) GH-secreting adenoma with persistently elevated insulin growth factor-1 (IGF-1) despite medical therapy and 2) clinical follow up >3 years after FSRT. Patients were treated with modern FSRT planning techniques. Biochemical control was defined as IGF-1 normalization. Systematic review of the literature was performed for FSRT in acromegaly. Results With a median follow-up of 80 months, radiographic control was achieved in all 11 patients and overall survival was 100%. Long-term biochemical control was achieved in 10 patients (90.9%) with either FSRT alone (36.4%) or FSRT with continued medical management (45.5%). No patient experienced new hypopituitarism, cranial nerve dysfunctions, or visual deficits. Our systematic review found published rates of biochemical control and hypopituitarism vary, with uniformly good radiographic control and low incidence of visual changes. Conclusions Adjuvant FSRT offered effective long-term biochemical control and radiographic control, and there was a lower rate of complications in this current series. Review of the literature shows variations in published rates of biochemical control after FSRT for acromegaly, but low incidence of serious toxicities.


2017 ◽  
Vol 14 ◽  
pp. 36-39 ◽  
Author(s):  
Georgios K. Georgiou ◽  
Athina Tsili ◽  
Anna Batistatou ◽  
Alexandra Papoudou-Bai ◽  
Georgios Papadopoulos ◽  
...  

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