A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

Clinical-pathological features of gastrointestinal stromal tumors presenting associated with other neoplasms: a brief report.

AIM: Over the past 10 years, few authors reported the synchronous detection of gastrointestinal stromal tumors (GISTs) and other neoplasms in the 9-33% of GISTs series. The primary aim of the present study was of investigating the features of GISTs detected in patients with other malignancies. METHODS: From 1999 to 2010 the GISTs detected at surgical exploration or preoperative assessment for other malignancies plus primary-GISTs, were recorded and reviewed. RESULTS: All synchronous GISTs were positive for kit/CD34, resulting smaller in size, with a lower mitotic index and occurring in elderly patients, comparing with primary-GISTs (P<0.05). Moreover a prevalence of males and of lower-risk classifications were noted, not reaching, however, a statistical value. CONCLUSION: According with our findings, the synchronous GISTs are mainly asymptomatic/incidentally detected and display some of the low malignant features; we recommend, however, the surgical excision of GISTs occurring in patients with other malignancies in order to define the histology and risk features and since it might result in an incorrect management if misdiagnosed as a metastases.

[Solitary small bowel metastasis after resection of bile duct carcinoma]. / Metastasi ileale solitaria da neoplasia biliare extraepatica precedentemente resecata.

Small bowel solitary metastases are a very rare occurrence and are more frequently recognized only in the presence of a severe complication, such as intestinal hemorrhage or occlusion. We report the case of a 75 year-old man who was admitted with a recent history of mechanical ileus developed one year after the surgical removal of an endoscopically intubated carcinoma of the extrahepatic biliary tree (pT3 pN0 Mx). A solitary metastasis of the small bowel, 30 cm from the ileo-cecal valve, was excised during the emergency laparotomy and a side-to-side anastomosis was performed to reconstruct the intestinal continuity. Patient was, thereafter, discharged in the 9th postoperative day. Local recurrence and intrabdominal dissemination are often observed in patients treated for bilio-pancreatic carcinoma. Preoperative invasive (ERCP, FNA, PTBD, etc.) diagnostic procedures and surgical tumor manipulation are associated with a greater risk of metastasis implantation and intraabdominal dissemination. In accordance to the literature, the authors propose, in cases with resectable bilio-pancreatic neoplasms, the use of standard external low dose radiotherapy prior to any invasive diagnostic procedure and/or surgical removal.

[Multidisciplinary treatment in Budd-Chiari syndrome: clinical case]. / Trattamento multidisciplinare nella sindrome di Budd-Chiari: caso clinico.

Budd-Chiari Syndrome (BCS) is characterized by obstruction of hepatic venous outflow. When obstruction is limited to the suprahepatic veins, portocaval shunting provides an immediate relief of symptoms. If the obstacle results also from narrowing of the inferior vena cava (IVC), multimodality treatments seem to offer safer and easier alternative. In the patient herein reported, combination of side-to-side portocaval anastomosis with a cavo-atrial shunt through an expandible metallic stent provided immediate relief of symptoms. The patient is doing well after 85 months from combined treatment. In conclusion infracaval stenting combined to side-to-side portocaval shunting should represent the treatment of choice in acute or subacute forms of BCS.

[Intestinal endometriosis. Three new cases and review of the literature]. / Endometriosi intestinale. Tre nuovi casi e revisione della Letteratura.

OBJECTIVE: The study was undertaken to identify some features of the intestinal endometriosis such as symptoms, helpful investigations, pattern of distribution and surgical management. PATIENTS: Three consecutive cases, observed during a sixteen month period, are reported. The most frequent symptoms were chronic pelvic and abdominal pain, dysmenorrhea, alterated bowel habit and menorrhagia. The diagnosis of intestinal endometriosis was incidental in all but one case admitted for an intestinal subocclusive syndrome in patient with a past history of pelvic endometriosis previously documented by laparoscopy. RESULTS: All patients presented a sigmoid localization of endometriosis with different degree of stenosis and underwent sigmoid resection, followed by a resolution of abdominal symptoms. DISCUSSION: Although the exact frequency of intestinal endometriosis is difficult to know because of the lack of specific symptoms and reliable investigations, it has been estimated that implants to the bowel may occur in 3%-37% of women affected by endometriosis. The sigmoid colon is the most common site of localization. The main symptoms are pelvic pain, dysmenorrhea, infertility and diarrhoea or constipation; rarely patients present bowel occlusion due to stenosis (less than 15% of the cases) or cyclic rectal bleeding. CONCLUSION: Generally, intestinal endometriosis is not suspected preoperatively in those patients without a past history of this condition; however an accurate diagnosis can be provided throughout laparoscopy, before open surgery. The hormonal therapy is not successful in alleviating moderate to severe obstructive symptoms. Thus surgery still remains the most effective treatment for advanced intestinal endometriosis.

[Rectorrhagia caused by lipoma of the hepatic flexure of the colon. A case report and review of the literature]. / Rettorragia da lipoma della flessura epatica del colon. Descrizione di un caso clinico e revisione della letteratura.

The Authors report a case of intestinal bleeding due to lipoma of the epatic flexure. Lipomas are the commonest mesenchymal benign tumors that can be found in the colon and are second as frequence only to the adenomatous polyps. They are most frequent in the right colon and shown an opposite distribution in comparison with adenocarcinomas and adenomatous polyps. When tumor size is greater than 3 cm, lipomas become symptomatic. Radiological and endoscopic investigations provide orientative elements for diagnosis. In lipomas less than 2 cm in diameter an endoscopic treatment is feasible.

Revascularization of the ischemic diabetic foot by popliteal-to-distal bypass.

BACKGROUND: Diabetic foot revascularization by popliteal-to-distal bypass is a procedure for limb salvage spread in the last decade. METHODS: The authors report their experience with a consecutive series of 15 ischemic feet (mean transcutaneous oxygen 5.3 +/- 4.1 mmHg) with gangrenous lesions due to extensive tibial arteries occlusive disease beginning at the popliteal artery trifurcation (9 cases) or involving the distal popliteal artery (6 cases). Limb salvage was achieved by popliteal-to-distal bypass with autogenous inverted saphenous vein. RESULTS: No operative death was observed. At a mean follow-up of 35 +/- 23 months (range 3-84 months) 4 bypasses were occluded and two were surgically revised after 4 and 50 months from surgery and subsequently remained patent. One patient was submitted to a major amputation. By life table analysis the cumulative primary and secondary patency and limb salvage rates for this group of diabetic patients were at 2 years 79.3%, 86.2% and 93.1% respectively (SE < 10%). CONCLUSIONS: This small experience and a review of the literature confirm the validity of the popliteal-to-distal bypass and the need for a more aggressive vascular surgical attitude to treat the ischemic diabetic foot.

Obstructive biliary symptomatology as the first sign of HIV-infection.

A 41-year-old white homosexual man presented with epigastric pain and jaundice. Physical examination showed enlargement of bilateral axillar and left inguinal lymph node, while ERCP and a CT scan suggested interruption of bile flow in the intrapancreatic tract of the common bile duct. An endoprosthesis was positioned in the common bile duct during the ERCP. Blood tests (both ELISA and Western blot techniques) showed positivity for anti-HIV antibodies and a CD4 count of 780/mmc (normal: 900-1,200/mmc). A few days later, a dramatic increase of the size of a lymph node in this right axilla occurred, rapidly reaching 5 cm of diameter. A biopsy was performed at this level, and histological examination revealed a high grade B-cell Burkitt type lymphoma. Bone marrow biopsy was negative, as well as lumbar puncture. Aggressive chemotherapy with adriamycin, cyclophosphamide, bleomycine, eldesine and prednisone, together with intratechal administration of methotrexate, was attempted. However, after a marginal and transient regression, the NHL rapidly progressed and the patient eventually died seven months after the diagnosis of NHL. A post mortem examination confirmed the diagnosis of Burkitt lymphoma of the peripancreatic and axillar lymph nodes, with diffusion to the leptomeninges, subaracnoideal spaces and encephalus. No signs of lymphoma were detected in other nodal or extra nodal areas.

[Acute abdominal pain in emergency surgery. Clinical epidemiologic study of 450 patients]. / Il dolore addominale acuto in chirurgia d'urgenza. Studio clinico-epidemiologico su 450 pazienti.

A retrospective study on 450 patients admitted in emergency with acute abdominal pain in a general surgical unit of the Ist Institute of Surgery of the University of Rome "La Sapienza" was carried out during a 7 years period. The aim of the study was to identify the most frequent causes of acute abdominal pain correlated with age, sex, onset of pain and other conditions in order to improve diagnosis and reduce mobility for mortality. Appendicitis was the most frequent diagnosis (75 pts = 16.4%). Non-specific abdominal pain (NSAP), found in 71 pts (15.5%), cholelithiasis in 57 pts (12.5%), abdominal malignancy in 47 pts (10.3%) were other frequent conditions. The largest number of admissions occurred in the age groups 60-70 years (16.6%) and 20-30 years (14.2%). Surgical operations were performed in 206 patients (45.7%). The overall mortality was 4.2% (19/450 pts) and the rate increased significantly in patients aged > 60 years. Postoperative mortality was 5.8% (12/206 pts) while mortality rate in non-operated patients was 2.8%. The causes of perioperative death included perforated peptic ulcer, abdominal malignancies (15.4%) and urgent colonic resections (9.4%). The duration of inpatients stay increased significantly with the age of the patients, including those with nsap. The results of the study indicate a need to review the methods of diagnosis of appendicitis and to obtain a better clinical performance in patients with nsap.

[Distal popliteal bypass in the diabetic patient. Clinical case]. / Il bypass popliteo distale nel paziente diabetico. Caso clinico.

Foot revascularization by popliteal-to-distal bypass is a well documented procedure for limb salvage in diabetic patients. The authors report their experience with a diabetic patient admitted with an ischemic left foot [transcutaneous oxygen (PtcO2): 3 mmHg] due to extensive tibial artery occlusive disease beginning at the popliteal artery trifurcation. Limb salvage was achieved by anatomical popliteal-anterior tibial bypass with inverted saphenous vein. Nine months later the patient was readmitted with an ischemic right foot (PtcO2: 16 mmHg) and a similar pattern of peripheral arterial occlusive disease. Limb salvage was achieved by extranatomical popliteal-dorsalis pedis bypass with a reversed saphenous vein. At six months from the latest surgery both feet are healed, PtcO2 is 60 mmHg and 37 mmHg for the left and right foot respectively and the patient has resumed a normal walking distance. This experience, and a review of the literature, confirm the validity of the popliteal-to-distal bypass and the need for more aggressive vascular surgical attitude to treat the ischemic diabetic foot.

[Efficacy of the popliteal to distal bypass in salvage of the ischemic diabetic foot]. / Efficacia del by-pass popliteo distale per il salvataggio del piede diabetico ischemico.

Foot revascularization by popliteal-to-distal by-pass is a well documented procedure for limb salvage in diabetic patients. The authors report their experience with a consecutive series of 5 ischemic feet [transcutaneous oxygen (PtO2): 6 +/- 5.6 mmHg] due to extensive tibial artery occlusive disease beginning at the popliteal artery trifurcation. Limb salvage was achieved by popliteal-to-anterior tibial bypass (1 case) and popliteal-to-dorsalis pedis artery by-pass with autogenous inverted saphenous vein. No operative death was observed. At follow-up (range 3-34 months) one patient died at 3 months after surgery because of an intestinal bleeding. All 4 remaining by-passes are well functioning [PtcO2 46 +/- 11.2 mmHg) and all patients have resumed their normal walking distance. This preliminary experience, and a review of the literature, confirm the validity of the popliteal-to-distal by-pass and the need for more aggressive vascular surgical attitude to treat the ischemic diabetic foot.

[Role of ultrasonography in emergency surgery]. / Ruolo dell'ecografia in chirurgia d'urgenza.

A prospective study was carried out to compare the value of ultrasound examination with conventional management in patients presenting with emergency surgical problems. During an 4 years period the authors followed 301 patients referred to the Ist Institute of Surgery of the University of Rome "La Sapienza". After immediate clinical evaluation one or more US examinations were performed in each patient following a standard protocol of study. Abdominal emergencies were the most frequent conditions, found in 75% of the cases and the acute abdomen due to trauma in 17.6% of the cases. The overall sensibility, specificity and accuracy of clinical examinations and US examinations were respectively of 88%, 33%, 70% and 95%, 94%, 95%. In 38 patients (12.7%), a diagnosis was made up by sonographic examination which had not been clinically expected (group A). The principal condition in this group was gynaecological pathology, found in 17 patients. In 161 patients (53.3%) sonography confirmed the first diagnosis clinically suspected (group B) and in 23 cases the second or third differential diagnosis (group D). Sonography made no contribution to the diagnosis in 77 cases (25,5%) and only in 2 cases was considered misleading. The result of this study demonstrate the usefulness of emergency ultrasonography in gynecological, gall bladder, pancreatic and vascular diseases. Moreover sonography must be considered the principal diagnostic tool in patients with trauma. In order to recognize gynecological diseases which can simulate appendicitis US examination must be considered necessary in young female patients. When appendicitis is suspected US is useful in children and in older patients too.

[The surgical treatment of thyroglossal duct cysts and fistulae]. / Il trattamento chirurgico delle cisti e fistole del dotto tireoglosso.

The authors report the results of treatment in 20 patients with thyroglossal duct cysts and/or fistula. Three patients underwent the standard Sistrunk's operation (excision of the cyst with resection of the duct above the hyoid bone with the portion of the muscle surrounding to the foramen caecum), 13 patients were submitted to a modified Sistrunk's operation (removal of the cyst and/or fistula and resection of the middle portion of the hyoid bone with dissection above it only with macroscopic evidence of duct epithelium) and 4 patients underwent less radical procedures. There were no recurrence in those patients operated upon with the standard or modified Sistrunk's operation. On the contrary two recurrence were observed in patients operated in elsewhere by simple excising the cyst without hyoid bone resection. In conclusion the authors strongly support the modified Sistrunk's operation as treatment of choice in patients with thyroglossal duct cysts and fistula; This operative procedure can avoid long-term recurrence even though many authors suggest that only the standard Sistrunk's operation can guarantee the lowest rate of failures.

Impact of primary surgical approach in the management of the impalpable testis.

There is no univoque opinion about the place of preoperative studies in non-palpable testes. During a 6.5-year period, we operated on 296 impalpable testes in prepubertal boys. A combined inguinal-abdominal approach was used in all cases verifying the eventual abdominal testis and its exact vascular anatomy before any manipulation of the cord was undertaken. Forty-five testes (15.2%) were canalicular, 142 (48%) were abdominal, 5 (1.7%) dysgenetic and 104 (34.1%) absent (agenesis or vanishing testis). Of the abdominal testes, 122 underwent a standard orchidopexy in dartos pouch, 11 a staged repair, 8 a Fowler-Stephens operation and 1 orchiectomy. All means of investigation for impalpable testes are either unreliable, too expensive or too invasive for routine use, and in most cases, a surgical exploration has to be performed anyway. The primary surgical approach has the most favorable cost/benefit ratio, being diagnostic and therapeutic at one time. Provided the exploration is performed correctly, all the advantages of previous laparoscopy can be achieved with surgery alone.

Accessory perineal scrotum associated with anorectal malformation. A report of 2 cases.

Two new cases of accessory perineal scrotum in association with anorectal malformation are reported. The development of accessory scrota--as distinct from ectopic scrota--can be attributed to abnormal migration of the labioscrotal swellings.

Adult polycystic liver disease in childhood. A case report.

A case of adult type polycystic liver disease in a child is reported. The entire right hepatic lobe was involved whereas the left lobe was normal. Spleen, kidneys and pancreas did not show cystic alterations. Diagnostic examinations and histologic findings are illustrated. The patient underwent right hepatectomy. The adult and childhood types of congenital polycystic liver disease are discussed.

Role of delayed cutaneous hypersensitivity reaction in classifying patients with bronchial carcinoma.

Cell-mediated immune response was evaluated in 150 patients with histologically confirmed bronchopulmonary carcinoma using bacterial and fungal recall antigens injected intradermally (PPD, candida, trichophyton). In the study group negative skin test reaction was found in 51 of 150 patients (34.0%), whereas in the control population it was found in 5 of 33 cases (15.1%) (p less than 0.05). Histologic cell type and stage of disease were defined for each patient. It was possible to calculate the growth rate of the primary tumor only in 68 of 150 patients, and it was recorded as doubling time. Evaluation of the skin test reaction in each prognostic subgroup showed no statistically significant differences. The only statistically significant differences were found when each prognostic subgroup was compared with the control population according to the frequency of a negative response to the skin test, particularly in stage III M1 (p less than 0.05) and stage III M0 (p less than 0.02). The delayed cutaneous hypersensitivity studied with recall antigen stimulation was mainly correlated with the stage of disease, and it should not be considered as an independent prognostic factor.