LIMB BODY WALL COMPLEX IN TWO HETEROZYGOTIC TWINS: A CASE REPORT

Introdution. Limb-Body Wall Complex (LBWC) is a congenital defect which includes at least two of the following characteristics: abdominal and/or thoracic body wall defects, exencephaly or encephalocoele with or without craniofacial defects (56%) and spinal defects associated with marked vertebral or sacral defects (95%). Case Report. We present a case report of an infant with LBWC, borned by diamniotic twin pregnancy. A prenatal ultrasound reported an healthy fetus and a fetus with multiple malformations. At birth we found a big abdominal wall defect, absence of scrotal sac and testicles, asymmetric chest and no major deformities in craniofacial region. At 2 hours of life, we removed the amniotic sac, we put the stomach and the spleen in the abdominal cavity, which is virtual, and positioned a spring-loaded silo. At 15th day of life we had complete reduction of the intestinal loops and liver and we closed the wall defect with a prosthesis and a cryopreserved skin. The general conditions of the patient, very severe since birth, became progressively worse and he died in the 21st day of life. Discussion. Once the diagnosis is done the physician should offer the parents a therapeutic abortion, and above all, in case they want to carry the pregnancy to term, we need to prepare them to the severity of the malformation and the high probability of death

Migration of surgical clips through a right lobectomy stump mimicking an asthmatic syndrome

The mechanical stapler is routinely used in thoracic surgery practice to attend resection of bronchus and vessels. Herein, we reported a very rare complication as the migration of a titanium surgical clip through a right lobectomy stump. One year after the procedure, the patient complained of persistent cough. A misdiagnosis of asthma was made and she treated for 6 months with bronchodilators, corticosteroid and antihistaminic without success. Thus, patient re-referred of our unit. No clinical signs of infection as fewer, productive cough, dyspnea were present. The laboratory exams were within normal value including white cells. CT scan revealed no abnormalities. Bronchoscopy demonstrated a healed upper bronchus stump without evidence of an actual, open bronchopleural fistula but with clips apparently working their way into the airway, with approximately half of the clip visible within the lumen. The side of the clips that would be open before closure by the surgeon formed the leading edge of the clips visible in the lumen. The clips were successfully removed during flexible bronchoscopy with a forceps usually used for biopsy. After the procedure, the cough disappeared. The endoscopy check after 3 months showed a normal bronchial stump without evidence of fistula

Total mesorectal excision for mid and low rectal cancer: Laparoscopic vs robotic surgery

AIM: To compare the short- and long-term outcomes of laparoscopic and robotic surgery for middle and low rectal cancer. METHODS: This is a retrospective study on a prospectively collected database containing 111 patients who underwent minimally invasive rectal resection with total mesorectal excision (TME) with curative intent between January 2008 and December 2014 (robot, n = 53; laparoscopy, n = 58). The patients all had a diagnosis of middle and low rectal adenocarcinoma with stage I-III disease. The median follow-up period was 37.4 mo. Perioperative results, morbidity a pathological data were evaluated and compared. The 3-year overall survival and disease-free survival rates were calculated and compared. RESULTS: Patients were comparable in terms of preoperative and demographic parameters. The median surgery time was 192 min for laparoscopic TME (L-TME) and 342 min for robotic TME (R-TME) (P < 0.001). There were no differences found in the rates of conversion to open surgery and morbidity. The patients who underwent laparoscopic surgery stayed in the hospital two days longer than the robotic group patients (8 d for L-TME and 6 d for R-TME, P < 0.001). The pathologic evaluation showed a higher number of harvested lymph nodes in the robotic group (18 for R-TME, 11 for L-TME, P < 0.001) and a shorter distal resection margin for laparoscopic patients (1.5 cm for L-TME, 2.5 cm for R-TME, P < 0.001). The three-year overall survival and disease-free survival rates were similar between groups. CONCLUSION: Both L-TME and R-TME achieved acceptable clinical and oncologic outcomes. The robotic technique showed some advantages in rectal surgery that should be validated by further studies

FEMALE EPISPADIAS: A CASE REPORT AND REVIEW OF THE LITERATURE

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Safety of mRNA COVID-19 Vaccines in Patients with Inborn Errors of Immunity: an Italian Multicentric Study

Purpose: Little is known about vaccine safety in inborn errors of immunity (IEI) patients during the current vaccination campaign for COVID-19. To better investigate the reactogenicity and adverse event profile after two, three, and four doses of mRNA vaccines, we conducted an observational, multicentric study on 342 PID patients from four Italian Referral Centres. Methods: We conducted a survey on self-reported adverse reactions in IEI patients who received mRNA vaccine by administering a questionnaire after each dose. Results: Over the whole study period, none of the patients needed hospitalization or had hypersensitivity reactions, including anaphylaxis and delayed injection site reaction. After two vaccination doses, 35.4% of patients showed only local reactogenicity-related symptoms (RrS), 44.4% reported both systemic and local RrS, and 5% reported only systemic RrS. In more than 60% of cases, local or systemic RrS were mild. After the first and second booster doses, patients showed fewer adverse events (AEs) than after the first vaccination course. Patients aged 50 years and older reported adverse events and RrS less frequently. Among AEs requiring treatment, one common variable immune deficiency patient affected by T cell large granular lymphocytic leukemia developed neutropenia and one patient had Bell’s paralysis perhaps during herpes zoster reactivation. Conclusion: Although our follow-up period is relatively short, the safety data we reported are reassuring. This data would help to contrast the vaccine hesitancy often manifested by patients with IEI and to better inform their healthcare providers