Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment.

Polycystic liver disease (PLD) is a clinical condition characterized by the presence of more than 10 cysts in the liver. It is a rare disease Of genetic etiology that presents as an isolated disease or assoc\iated with polycystic kidney disease. Ductal plate malformation, ciliary dysfunction, and changes in cell signaling are the main factors involved in its pathogenesis. Most patients with PLD are asymptomatic, but in 2-5% of cases the disease has disabling symptoms and a significant reduction in quality of life. The diagnosis is based on family history of hepatic and/or renal polycystic disease, clinical manifestations, patient age, and polycystic liver phenotype shown on imaging examinations. PLD treatment has evolved considerably in the last decades. Somatostatin analogues hold promise in controlling disease progression, but liver transplantation remains a unique curative treatment modality.

Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease.

Autosomal dominant polycystic liver disease (ADPLD) is a rare disease with variable clinical presentations, characterized by cystic enlargement of the liver. The diagnosis is made based on family history, patient's age, and liver phenotype and is confirmed by imaging tests. The treatment aims to reduce symptoms caused by the increased liver volume and can be performed by aspiration with sclerotherapy, fenestration, and liver resection. Although ADPLD is a rare disease, it is an important differential diagnosis of cystic diseases such as polycystic kidney disease; therefore, the aim of this article was to present the diagnostic and therapeutic approach of a case of ADPLD and conducting a literature review. This is the case of a 32-year-old male patient, who was hospitalized due to abdominal pain, hepatomegaly, lack of appetite, and weight loss. Imaging propaedeutics showed a significant increase in the liver volume due to hepatic cysts. After a multidisciplinary evaluation, given the clinical changes and the location of the hepatic cysts, fenestration was performed by laparotomy. The postoperative period was uneventful. The treatment was efficient in promoting symptomatic relief and improving the quality of life in this patient. Case reports on this disease are quite limited in the currently available literature, and there are gaps in knowledge with regard to the diagnosis and management of ADPLD. The importance of this article is that it will highlight the limitations in treatment options and allow physicians to make a more informed decision when diagnosing and treating a patient with ADPLD in the future.

Model for establishing a new liver transplantation center through mentorship from a university with transplantation expertise.

BACKGROUND: Setting up new liver transplant (LT) centers is essential for countries with organ shortages. However, good outcomes require experience, because LT learning depends on a high number of surgeries. This study aims to describe how a new center was set up from a partnership between the new center and an experienced one. The step-by-step preparation process, the time needed and the results of the new center are depicted. MATERIAL AND METHODS: The mentoring process lasted 40 months, in which half of the 52 patients included on the transplant list received LT. After the mentorship, a 22-month period was also analyzed, in which 46 new patients were added to the waiting list and nine were operated on. RESULTS: The 30-day survival rates during (92.3%) and after (66.7%) the partnership were similar to the other LT centers in the same region, as well as the rates of longer periods. The waiting time on the LT list, the characteristics of the donors and the ischemia times did not differ during or after the mentorship. CONCLUSION: The partnership between universities is a suitable way to set up LT centers, achieving good results for the institutions and the patients involved.

Alterations of the gastric stump and not resected stomach mucosa after truncal vagotomy in rats.

PURPOSE: To evaluate morphological changes of the gastric stump and not resected stomach mucosa after the completion of truncal vagotomy. METHODS: Eighty male Wistar rats were divided into four groups: CT, TV, RY and RYTV. In CT group, abdominal viscera were manipulated and the abdominal cavity was closed, in TV vagal trunks were isolated and sectioned, in RY a partial Roux-en-Y gastrectomy was performed and in RYTV the vagal trunks were sectioned and a partial Roux-en-Y gastrectomy was performed. At the 54th week after surgery, the rats were euthanized. The findings were submitted to histological analyses. RESULTS: None macroscopic or histological alterations in groups TV and CT was observed. Specimens from RY and RYTV groups did not show alterations in the gastric stump mucosa. At the jejunal side of the gastroenterostomy we found shallow ulcerative lesions always single, well-defined and with variable diameter 3 to 6 mm, six times in the RY group and none in the RYTV group (RY>RYTV, p=0.008). Neoplastic or preneoplastic lesions were not diagnosed in all groups. CONCLUSION: Truncal vagotomy is a safe and non-carcinogenic method in not resected and partially resected stomach.

Alloxan diabetes alters the tensile strength, morphological and morphometric parameters of abdominal wall healing in rats.

PURPOSE: To investigate the effects of alloxan diabetes on the abdominal wall healing of rats undergoing laparotomy. METHODS: Ninety-six male Wistar rats weighing between 200 and 300 grams, divided into two groups: non-diabetic group (G1) and another with untreated diabetes (G2). Three months after diabetes induction, the animals underwent a 5cm-long- laparotomy and 5.0 nylon monofilament suture. After the surgery, 12 animals from each group were euthanized on days 4, 14, 21 and 30 corresponding to the moments M1, M2, M3 and M4. In each moment a fragment of the abdominal wall containing the scar was removed for tensile strength measurement, histological and morphometric study. Clinical and biochemical parameters were also analyzed. RESULTS: G2 animals showed parameters compatible with severe diabetes and decreased plasma levels of insulin. The tensile strength in G2 was significantly smaller in M2 and M4, with a tendency to fall in the other two. Through light microscope, diabetic animals showed more difficulty to increase collagen density and contraction. G2 animals showed high cellularity of fibroblasts in later healing moments, with collagen thinning in M2 and M4. CONCLUSION: The abdominal wound healing in untreated diabetic animals was altered and led to a higher incidence of dehiscence and infections.

Alterations of the gastric stump and not resected stomach mucosa after truncal vagotomy in rats

To evaluate morphological changes of the gastric stump and not resected stomach mucosa after the completion of truncal vagotomy. METHODS: Eighty male Wistar rats were divided into four groups: CT, TV, RY and RYTV. In CT group, abdominal viscera were manipulated and the abdominal cavity was closed, in TV vagal trunks were isolated and sectioned, in RY a partial Roux-en-Y gastrectomy was performed and in RYTV the vagal trunks were sectioned and a partial Roux-en-Y gastrectomy was performed. At the 54th week after surgery, the rats were euthanized. The findings were submitted to histological analyses. RESULTS: None macroscopic or histological alterations in groups TV and CT was observed. Specimens from RY and RYTV groups did not show alterations in the gastric stump mucosa. At the jejunal side of the gastroenterostomy we found shallow ulcerative lesions always single, well-defined and with variable diameter 3 to 6 mm, six times in the RY group and none in the RYTV group (RY>RYTV, p=0.008). Neoplastic or preneoplastic lesions were not diagnosed in all groups. CONCLUSION: Truncal vagotomy is a safe and non-carcinogenic method in not resected and partially resected stomach.

Alloxan diabetes alters the tensile strength, morphological and morphometric parameters of abdominal wall healing in rats

To investigate the effects of alloxan diabetes on the abdominal wall healing of rats undergoing laparotomy. METHODS: Ninety-six male Wistar rats weighing between 200 and 300 grams, divided into two groups: non-diabetic group (G1) and another with untreated diabetes (G2). Three months after diabetes induction, the animals underwent a 5cm-long- laparotomy and 5.0 nylon monofilament suture. After the surgery, 12 animals from each group were euthanized on days 4, 14, 21 and 30 corresponding to the moments M1, M2, M3 and M4. In each moment a fragment of the abdominal wall containing the scar was removed for tensile strength measurement, histological and morphometric study. Clinical and biochemical parameters were also analyzed. RESULTS: G2 animals showed parameters compatible with severe diabetes and decreased plasma levels of insulin. The tensile strength in G2 was significantly smaller in M2 and M4, with a tendency to fall in the other two. Through light microscope, diabetic animals showed more difficulty to increase collagen density and contraction. G2 animals showed high cellularity of fibroblasts in later healing moments, with collagen thinning in M2 and M4. CONCLUSION: The abdominal wound healing in untreated diabetic animals was altered and led to a higher incidence of dehiscence and infections.

Efficacy of surgical versus conservative treatment in esophageal perforation: a systematic review of case series studies.

PURPOSE: To evaluate the efficacy of surgical treatment for esophageal perforation. METHODS: A systematic review of the literature was performed. We conducted a search strategy in the main electronic databases such as PubMed, Embase and Lilacs to identify all case series. RESULTS: Thirty three case series met the inclusion criteria with a total of 1417 participants. The predominant etiology was iatrogenic (54.2%) followed by spontaneous cause (20.4%) and in 66.1% the localization was thoracic. In 65.4% and 33.4% surgical and conservative therapy, respectively, was considered the first choice. There was a statistically significance different with regards mortality rate favoring the surgical group (16.3%) versus conservative treatment (21.2%) (p<0.05). CONCLUSION: Surgical treatment was more effective and safe than conservative treatment concerning mortality rates, although the possibility of bias due to clinical and methodological heterogeneity among the included studies and the level of evidence that cannot be ruled out.

Efficacy of surgical versus conservative treatment in esophageal perforation: a systematic review of case series studies

PURPOSE: To evaluate the efficacy of surgical treatment for esophageal perforation. METHODS: A systematic review of the literature was performed. We conducted a search strategy in the main electronic databases such as PubMed, Embase and Lilacs to identify all case series. RESULTS: Thirty three case series met the inclusion criteria with a total of 1417 participants. The predominant etiology was iatrogenic (54.2%) followed by spontaneous cause (20.4%) and in 66.1% the localization was thoracic. In 65.4% and 33.4% surgical and conservative therapy, respectively, was considered the first choice. There was a statistically significance different with regards mortality rate favoring the surgical group (16.3%) versus conservative treatment (21.2%) (p<0.05). CONCLUSION: Surgical treatment was more effective and safe than conservative treatment concerning mortality rates, although the possibility of bias due to clinical and methodological heterogeneity among the included studies and the level of evidence that cannot be ruled out.

Intestinal healing in rats submitted to ethanol ingestion.

PURPOSE: To study the effect of alcoholism on intestinal healing and postoperative complications in rats METHODS: One hundred and sixty rats were divided into two groups: control and treated. The control group received water and the treated group 30% ethanol. After 180 days, colotomy with anastomosis were performed. After, the groups were divided into four subgroups: 20 rats for study at the following moments: 4(th), 7(th), 14(th) and 21(st) postoperative. The analyzed parameters were: weight gain, breaking strength, tissue hydroxyproline, postoperative complications and histopathological study RESULTS: Weight gain was greater in the control group (p<0.05). When all the subgroups were clustered, breaking strength was significantly greater in the control (p<0.05). Histopathology and hydroxyproline dosage did not show differences. There were five surgical site infections in the treated group while the control group showed two (p>0.05). Nine fistulas occurred in the treated group whereas the control group two (p<0.05). There were three deaths in the control group and seven in the treated group (p>0.05). CONCLUSIONS: Treated group undergo a malnutrition process that is revealed by lower weight gain. Impaired intestinal healing as indicated by smaller breaking strength. There were a larger number of postoperative complications in the treated animals.

Intestinal healing in rats submitted to ethanol ingestion / Cicatrização intestinal em ratos submetidos à ingestão de etanol

PURPOSE: To study the effect of alcoholism on intestinal healing and postoperative complications in rats METHODS: One hundred and sixty rats were divided into two groups: control and treated. The control group received water and the treated group 30 percent ethanol. After 180 days, colotomy with anastomosis were performed. After, the groups were divided into four subgroups: 20 rats for study at the following moments: 4th, 7th, 14th and 21st postoperative. The analyzed parameters were: weight gain, breaking strength, tissue hydroxyproline, postoperative complications and histopathological study RESULTS: Weight gain was greater in the control group (p<0.05). When all the subgroups were clustered, breaking strength was significantly greater in the control (p<0.05). Histopathology and hydroxyproline dosage did not show differences. There were five surgical site infections in the treated group while the control group showed two (p>0.05). Nine fistulas occurred in the treated group whereas the control group two (p<0.05). There were three deaths in the control group and seven in the treated group (p>0.05). CONCLUSIONS: Treated group undergo a malnutrition process that is revealed by lower weight gain. Impaired intestinal healing as indicated by smaller breaking strength. There were a larger number of postoperative complications in the treated animals.

OBJETIVO: Estudar o efeito do alcoolismo no processo de cicatrização intestinal e suas complicações pós-operatórias em ratos. MÉTODOS: Cento e sessenta ratos foram divididos em dois grupos: tratado e controle. O controle recebeu água, enquanto o tratado etanol a 30 por cento. Após 180 dias foram realizadas colotomia, seguida de anastomose. Após os animais foram divididos em quatro subgrupos de 20 ratos para estudo nos seguintes momentos: 4º, 7º, 14º e 21º pós-operatório. Os parâmetros analisados foram: ganho de peso, força de ruptura, hidroxiprolina tecidual, complicações pós-operatórias e estudo histopatológico. RESULTADOS: O ganho de peso foi superior no grupo controle (p<0,05). Após agrupamento dos momentos a força de ruptura foi superior no controle (p<0,05). Não houve diferença quanto à histopatologia e hidroxiprolina. Houve cinco infecções de incisão no grupo tratado, enquanto no controle ocorreram duas (p>0,05). Houve nove fístulas no grupo tratado, enquanto no controle duas (p<0,05). Ocorreram sete mortes no grupo tratado e apenas três no controle (p>0,05). CONCLUSÕES: No grupo tratado ocorreu um processo de subnutrição evidenciado pelo menor ganho de peso. Piora na cicatrização intestinal, indicada pela menor força de ruptura. Ocorreu um maior número de complicações pós-operatórias no grupo tratado.

[Preoperative progressive pneumoperitoneum in voluminous abdominal wall hernias]. / O uso do pneumoperitônio progressivo no pré-operatório das hérnias volumosas da parede abdominal.

CONTEXT: Correction of voluminous hernias and large abdominal wall defects is a big challenge in surgical practice due to technical difficulties and the high incidence of respiratory and cardiovascular complications. OBJECTIVES: To present the authors experience with inducing progressive pneumoperitoneum preoperative to surgical treatment of voluminous hernias of the abdominal wall. METHODS: Retrospective study of six patients who presented voluminous hernias of the abdominal wall and were operated after installation of a pneumoperitoneum. The procedure was performed by placing a catheter in the abdominal cavity at the level of the left hypochondrium with ambient air insufflation for 10 to 15 days. RESULTS: Four of the six patients were female and two male. Ages ranged from 42 to 62 years. Hernia duration varied from 5 to 40 years. Four patients had incisional, one umbilical, and one inguinal hernias. Mean pneumoperitoneum time was 11.6 days. There were no complications related to pneumoperitoneum installation and maintenance. All hernias were corrected without technical difficulties. The Lichtenstein technique was used to correct the inguinal hernia, peritoneal aponeurotic transposition for one of the incisional hernias, with the rest corrected using polypropylene mesh. One death and one wall infection were observed post operatively. No recurrences were reported until now, in 4 to 36 months of follow-up. CONCLUSION: Preoperative progressive pneumoperitoneum is a safe and easy executed procedure, which simplifies surgery and reduces post-operative respiratory and cardiovascular complications. It is indicated for patients with hernias that have lost the right of domain in the abdominal cavity.

O uso do pneumoperitônio progressivo no pré-operatório das hérnias volumosas da parede abdominal / Preoperative progressive pneumoperitoneum in voluminous abdominal wall hernias

CONTEXTO: A correção das hérnias volumosas e dos grandes defeitos da parede abdominal constitui grande desafio da prática cirúrgica, em virtude das dificuldades técnicas e do alto índice de complicações respiratórias e cardiovasculares. OBJETIVOS: Apresentar experiência com a indução do pneumoperitônio progressivo no pré-operatório do tratamento cirúrgico das hérnias volumosas da parede abdominal. MÉTODOS: Estudo retrospectivo de seis pacientes que apresentavam hérnias volumosas da parede abdominal, e que foram operados após a instalação de um pneumoperitônio. O procedimento foi realizado através da colocação de um cateter na cavidade abdominal, na altura do hipocôndrio esquerdo, com insuflação de ar ambiente por período de 10 a 15 dias. RESULTADOS: Dos seis pacientes operados, quatro eram do sexo feminino e dois do masculino. A idade mínima era de 40 e a máxima de 62 anos. A duração da hérnia variou de 5 a 40 anos. Quatro pacientes tinham hérnia incisional, um umbilical e outro inguinal. O tempo médio de pneumoperitônio foi de 11,6 dias. Não houve complicações relacionadas à instalação e manutenção do pneumoperitônio. Todas as hérnias foram corrigidas sem dificuldades técnicas. Utilizou-se a técnica de Lichtenstein para a correção da hérnia inguinal, a transposição peritônio-aponeurótica para uma das hérnias incisionais, sendo as demais corrigidas com uso de tela de polipropileno. Um óbito e uma infecção de parede foram observados no pós-operatório dessas cirurgias. Não houve recidivas registradas até o momento, num período de seguimento de 4 a 36 meses. CONCLUSÃO: O pneumoperitônio progressivo pré-operatório é um procedimento seguro e de fácil execução, pois facilita o procedimento cirúrgico e diminui as complicações respiratórias e cardiovasculares no pós-operatório. É indicado para doentes com hérnias que perderam domicílio na cavidade abdominal.

CONTEXT: Correction of voluminous hernias and large abdominal wall defects is a big challenge in surgical practice due to technical difficulties and the high incidence of respiratory and cardiovascular complications. OBJECTIVES: To present the authors experience with inducing progressive pneumoperitoneum preoperative to surgical treatment of voluminous hernias of the abdominal wall. METHODS: Retrospective study of six patients who presented voluminous hernias of the abdominal wall and were operated after installation of a pneumoperitoneum. The procedure was performed by placing a catheter in the abdominal cavity at the level of the left hypochondrium with ambient air insufflation for 10 to 15 days. RESULTS: Four of the six patients were female and two male. Ages ranged from 42 to 62 years. Hernia duration varied from 5 to 40 years. Four patients had incisional, one umbilical, and one inguinal hernias. Mean pneumoperitoneum time was 11.6 days. There were no complications related to pneumoperitoneum installation and maintenance. All hernias were corrected without technical difficulties. The Lichtenstein technique was used to correct the inguinal hernia, peritoneal aponeurotic transposition for one of the incisional hernias, with the rest corrected using polypropylene mesh. One death and one wall infection were observed post operatively. No recurrences were reported until now, in 4 to 36 months of follow-up. CONCLUSION: Preoperative progressive pneumoperitoneum is a safe and easy executed procedure, which simplifies surgery and reduces post-operative respiratory and cardiovascular complications. It is indicated for patients with hernias that have lost the right of domain in the abdominal cavity.