The Journal of Bucharest College of Physicians and the Romanian Academy of Medical Sciences

Dragos Ene

Dragos Ene

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome Berdon’s syndrome - First Report in Romania

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare motility disorder with high mortality rate described by Berdon in 1976. We present the first case of Berdon’s syndrome (heterozygous pathogenic variant in the ACTG2 gene) reported in Romania, a female newborn admitted in NICU “Marie S. Curie” Emergency Children’s Hospital Bucharest for intestinal obstruction after birth. Total parenteral nutrition, ileostomy, gastrostomy, clean intermittent bladder catheterisation, evaluation for multivisceral transplantation were performed. She was discharged from our NICU ward at the age of 4 years and 2 months with home total parenteral nutrition administered by her mother in sterile condition, clean intermittent catheterisation for bladder evacuation performed by her mother, monitored monthly for about three years, with normal cardio-respiratory function, no signs of thrombosis, she maintained relatively low platelet count without positive blood culture, good liver and renal function test. Normal neurological and psychomotor development according to age. Her course was complicated by multiorgan failure with death ensuing at the age of 7 years and 10 months.

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Treating Congenital Diaphragmatic Hernia with ECMO: First Successful Case in Romania

We present the first successful case of extracorporeal membrane oxygenation (ECMO) at „M.S. Curie” Emergency Clinical Hospital for Children, Newborn Intensive Care Unit: a term neonate with cardiorespiratory failure secondary to left sided congenital diaphragmatic hernia. The patient was placed on veno-venous ECMO at three days of life;
however, on the fourth day, due to unstable right ventricular function, conversion to veno-arterial ECMO was mandatory. At one week of life, the patient was operated on-site for diaphragmatic hernia without ECMO support and then reintroduced on ECMO immediately after the surgical procedure, being on ECMO support for a total of 8 days. Antithrombotic treatment was administered for significant occlusion of the right common carotid artery and right internal jugular vein (complication of the cannulation for ECMO) and also long term treatment for Persistent Pulmonary Hypertension was needed. The patient was discharged at the age of four months with moderate neurodevelopmental delay. The literature review indicates that neonatal ECMO procedure in Romania is still in its early stages. Despite this state, our current case proves that ECMO can be successfully performed with increased chances of survival for neonates with severe prognosis after failure of conventional therapy.

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Upper GI Bleeding with Hemorrhagic Shock Caused by Infectious Esophagitis

CMV infection in healthy hosts is generally asymptomatic, producing a latent infection with antibodies persisting for months or even years after the recovery. In the population at risk, CMV infection is one of the most frequent opportunistic infection. The most frequent GI manifestation of CMV infection is colitis followed by esophagitis although it can affect all organs. CMV esophagitis has been reported in immunocompromised hosts by conditions like organ transplantation, bone marrow transplantation, in patients with HIV infection and AIDS or other debilitating diseases. [...]

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Intrathoracic Anastomotic Leak after Ivor Lewis Esophagectomy: Non-Surgical Management

Esophagectomy is the best therapy for the patients with locoregionally advanced esophageal cancer, but carries serious risks of associated morbidity and mortality. Esophageal anastomotic leak is a severe post-operative complication with a rate of mortality that can reach 60%. Clinical presentations of esophageal anastomotic leaks varies from asymptomatic to severe sepsis and death. The prognosis depends on the duration to diagnosis and the severity of contamination. An anastomotic esophageal leak has a great impact on the length of hospitalization, morbidity, stricture formation and dysphagia. The management of intrathoracic anastomotic leaks include conservative, surgical or endoscopic treatment, but the indication between these options of treatment remains controversial. [...]

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