Duodenal duplication with accessory papilla causing recurrent pancreatitis in a child: a case report

We discuss a 16-year-old male patient who presented with three episodes of recurrent pancreatitis within the last 6 months. Preoperative imaging studies suggested a choledochal cyst within the second portion of the duodenum. Patient was taken to surgery and the lesion was removed. Pathology examination of the cyst revealed a duodenal duplication. The accessory pancreatic papilla entering the closed duplication cyst was the main cause of the pancreatitis in this child.

Infection-associated hemophagocytic syndrome: a rare potentially fatal complication of systemic infection: report of three cases

Infection-associated hemophagocytic syndrome is a rare, potentially fatal complication of systemic infection. It occurs most often in immunocompromised patients associated with a viral infection but the spectrum of conditions have been broadened to include virtually every type of infectious pathogen, malignancy and immunosuppressive therapy. We present three pediatric patients with a similar clinical history of pancytopenia, hepatosplenomegaly, and acute liver failure, and discuss the autopsy findings.

Renohepaticopancreatic dysplasia: diagnostic dilemma

We present the case of an eighteen day old baby boy hospitalized with an abdominal mass, renal insufficiency and jaundice. Multiple radiographic, radionuclear and surgical interventions were required to diagnose renohepaticopancreatic dysplasia, also known as Ivemark II syndrome. In spite of aggressive intensive care support, the patient developed multisystemic organ failure and died. Clinical presentation and autopsy findings are presented.

Late presentation of congenital cystic adenomatoid malformation

We present the case of a 9 year old girl with history of progressive pneumatoceles and infection since she was 3 years old. A chest computerized tomography revealed a cystic lung mass. The patient was taken to surgery and a left lower lobe lobectomy was performed. The pathologic diagnosis was that of a congenital cystic adenomatoid malformation. We discuss the clinical presentation, and pathology of this entity with a brief review of the literature.

Pulmonary hypoplasia in Jarcho-Levin syndrome

Jarcho-Levin syndrome, also known as spondylothoracic dysplasia and characterized by short trunk dwarfism, "crab-like" rib cage, with ribs and vertebral defects; it is not uncommon in Puerto Ricans. Many patients die in early infancy due to respiratory compromise associated to lung restriction and the reported cases emphasize mostly the skeletal malformations associated to the syndrome. We report the autopsy findings in a newborn with isolated Jarcho-Levin syndrome emphasizing pulmonary pathology. He was a pre-term male who died of respiratory failure at three hours old and, autopsy findings confirmed the clinical diagnosis. Internal examination showed hypoplastic lungs with normal lobation. The histological structure appeared normal and relatively mature; the diaphragm showed eventration and unilateral absence of musculature. This case shows the worst spectum of the Jarcho-Levin syndrome: pulmonary hypoplasia not compatible with extrauterine life. Since thoracic restriction is present during the fetal period, the degree of pulmonary hypoplasia probably defines survival beyond the neonatal period.

Rhabdoid tumor: an unusual pediatric brain tumor

Rhabdoid tumors of the brain are rare with an invariable dismal prognosis despite treatment. This is the case of a 3 year old boy who presented lethargy, somnolence, nausea, vomiting, and headaches one week prior to hospitalization. A posterior fossa tumor with hydrocephalus was noted on a head computed tomography (CT) scan. A ventriculoperitoneal shunt was placed with subsequent gross total tumor resection. Pathology findings were those of a rhabdoid tumor. The histopathology, immunohistochemistry and ultrastructure of this unusual pediatric cerebral neoplasia is discussed.

Cavernous hemangioma presenting as a right adnexal mass in a child

This is the case of an 11-year-old girl who presented with a right adnexal mass and vague abdominal symptoms since seven months prior to her hospital admission for surgery. CT-scan and sonographic images were those of a benign lesion, probably ovarian torsion or infarction. Serum tumoral markers were normal. A right salpingo-oophorectomy and appendectomy were performed. Pathology examination revealed a cavernous hemangioma of the ovary. The clinicopathologic presentation of this unusual benign ovarian tumor is discussed.

Cerebral hemiatrophy: an associated finding in an epileptic child

Cerebral hemiatrophy is a rarely occurring condition of different etiologies that can be regarded as the final stage of a number of different disease processes. It is characterized by a marked asymmetry of the cerebral hemispheres. A 12 year old girl with history of epilepsy since infancy and psychomotor delay presented in status epilepticus, developed marked cerebral edema, bilateral uncal herniation and bilateral infarcts of the posterior cerebral artery territories. Autopsy findings revealed left cerebral hemiatrophy as an incidental findings. The clinicopathologic features and classification of this entity are discussed.

Pulmonary sequestration presenting as fetal hydrops

Pulmonary sequestration is a rare developmental anomaly of the lung characterized by nonfunctional pulmonary tissue without communication with the tracheobronchial tree and receiving an aberrant systemic arterial blood supply. Few cases of non-immune hydrops fetalis associated with this entity have been reported. A 2 day old male baby born by cesarean section at 31 weeks gestational age due to fetal hydrops is presented. Autopsy revealed a hydropic baby with extralobar pulmonary sequestration and bilateral pulmonary hypoplasia. The clinicopathologic presentation of this unusual pulmonary developmental anomaly is discussed.