Insulinoma with peripheral neuropathy: a case report.

BACKGROUND: Insulinomas are rare neuroendocrine tumors that typically present with hypoglycemic crises. Peripheral neuropathy is an uncommon complication of insulinoma. Most clinicians expect peripheral neuropathy symptoms to reverse completely after the insulin-secreting tumor is resected, but this may be a misassumption. CASE REPORT: We report a case of a 16-year-old Brazilian boy with clonic muscle spasms of the lower limbs for almost one year. Disabling paraparesis and confusional episodes had progressively set in as well. There were no sensorial abnormalities in the lower limbs, upper limbs or cranial nerves. An electromyography revealed a motor neuropathy of the lower limbs. The diagnosis of insulinoma was established as serum insulin and C-peptide concentrations were inappropriately normal during spontaneous episodes of hypoglycemia. Following a normal abdominal magnetic resonance scan, the imaging work-up continued with an endoscopic ultrasound, which localized the tumor at the pancreatic body-tail transition. Once localized, a prompt surgical removal (enucleation) of the tumor was undertaken, leading to an immediate and complete resolution of hypoglycemia. The time length between the onset of symptoms and tumor resection was 15 months. After surgery, the symptoms of peripheral neuropathy of the lower limbs showed a slow and only partial improvement. At a two-year follow-up after surgery, although being able to lead a normal and productive life, the patient still reported symptoms of reduced muscular strength in the lower limbs and a new electroneuromyography analysis showed chronic denervation and reinnervation in the legs' muscles-indicating chronic neuropathic injury. CONCLUSION: The events of this case reinforce the importance of an agile diagnostic work-up and spry definitive treatment for patients with this uncommon disease, enabling the cure of neuroglycopenia before permanent bothersome complications ensue.

Mirizzi syndrome grades III and IV: surgical treatment.

OBJECTIVE: : to evaluate the epidemiology and outcomes of surgical treatment of patients with Mirizzi Syndrome (MS) grades III and IV, the most advanced according to Csendes classification. METHODS: : we conducted a retrospective, cross-sectional study by reviewing records of thirteen patients with grades III and IV MS operated from December 2001 to September 2013, among the 3,691 cholecystectomies performed in the period. RESULTS: : the incidence of MS was 0.6% (23 cases) and grades III and IV amounted to 0.35% of this number. There was a predominance of type IV (12 cases). The preoperative diagnosis was possible in 53.8% of cases. The preferred approach was biliary-digestive derivation (10 cases), and "T" tube drainage with suture of the bile duct was the choice in three special occasions. Three patients had biliary fistula resolved with clinical management, and one coliperitoneum case required reoperation. In the outpatient follow-up of patients who underwent biliodigestive anastomosis (eight), 50% are asymptomatic, 25% had anastomotic stricture and 25% lost follow-up. The mean follow-up was 41.8 months. CONCLUSION: : MS in advanced degrees has low incidence, preoperative diagnosis in only half of cases, and has the biliodigestive anastomosis as the best conduct, but not without morbidity. OBJETIVO: avaliar a epidemiologia e os resultados do tratamento cirúrgico de doentes portadores de graus III e IV, mais avançados, da Síndrome de Mirizzi (SM) de acordo com a classificação de Csendes. MÉTODOS: estudo retrospectivo, de corte transversal através da revisão de prontuários de 13 pacientes portadores de graus III e IV da SM operados de dezembro de 2001 a setembro de 2013, entre 3691 colecistectomias realizadas neste período. RESULTADOS: a incidência da SM foi 0,6% (23 casos) e os graus III e IV perfizeram 0,35% deste número. Houve um predomínio de tipo IV (12 casos). O diagnóstico pré-operatório foi possível em 53,8% dos casos. A conduta preferencial foi derivação biliodigestiva (10 casos) e foi optado por drenagem com tubo "T" e sutura da via biliar em três ocasiões especiais. Três pacientes apresentaram fístula biliar resolvida com conduta expectante e um caso de coleperitônio necessitou reoperação. No seguimento ambulatorial dos pacientes que realizaram a anastomose biliodigestiva (oito), 50% estão assintomáticos, 25% apresentaram estenose da anastomose e 25% perderam seguimento. O tempo médio de acompanhamento foi 41,8 meses. CONCLUSÃO: de incidência baixa e de diagnóstico pré-operatório em apenas metade dos casos, a SM em graus avançados tem na anastomose biliodigestiva sua melhor conduta, porém não isenta de morbimortalidade.

Mirizzi syndrome grades III and IV: surgical treatment / Síndrome de Mirizzi graus III e IV: tratamento cirúrgico

ABSTRACT Objective : to evaluate the epidemiology and outcomes of surgical treatment of patients with Mirizzi Syndrome (MS) grades III and IV, the most advanced according to Csendes classification. Methods : we conducted a retrospective, cross-sectional study by reviewing records of thirteen patients with grades III and IV MS operated from December 2001 to September 2013, among the 3,691 cholecystectomies performed in the period. Results : the incidence of MS was 0.6% (23 cases) and grades III and IV amounted to 0.35% of this number. There was a predominance of type IV (12 cases). The preoperative diagnosis was possible in 53.8% of cases. The preferred approach was biliary-digestive derivation (10 cases), and "T" tube drainage with suture of the bile duct was the choice in three special occasions. Three patients had biliary fistula resolved with clinical management, and one coliperitoneum case required reoperation. In the outpatient follow-up of patients who underwent biliodigestive anastomosis (eight), 50% are asymptomatic, 25% had anastomotic stricture and 25% lost follow-up. The mean follow-up was 41.8 months. Conclusion : MS in advanced degrees has low incidence, preoperative diagnosis in only half of cases, and has the biliodigestive anastomosis as the best conduct, but not without morbidity.

RESUMO Objetivo: avaliar a epidemiologia e os resultados do tratamento cirúrgico de doentes portadores de graus III e IV, mais avançados, da Síndrome de Mirizzi (SM) de acordo com a classificação de Csendes. Métodos: estudo retrospectivo, de corte transversal através da revisão de prontuários de 13 pacientes portadores de graus III e IV da SM operados de dezembro de 2001 a setembro de 2013, entre 3691 colecistectomias realizadas neste período. Resultados: a incidência da SM foi 0,6% (23 casos) e os graus III e IV perfizeram 0,35% deste número. Houve um predomínio de tipo IV (12 casos). O diagnóstico pré-operatório foi possível em 53,8% dos casos. A conduta preferencial foi derivação biliodigestiva (10 casos) e foi optado por drenagem com tubo "T" e sutura da via biliar em três ocasiões especiais. Três pacientes apresentaram fístula biliar resolvida com conduta expectante e um caso de coleperitônio necessitou reoperação. No seguimento ambulatorial dos pacientes que realizaram a anastomose biliodigestiva (oito), 50% estão assintomáticos, 25% apresentaram estenose da anastomose e 25% perderam seguimento. O tempo médio de acompanhamento foi 41,8 meses. Conclusão: de incidência baixa e de diagnóstico pré-operatório em apenas metade dos casos, a SM em graus avançados tem na anastomose biliodigestiva sua melhor conduta, porém não isenta de morbimortalidade.