Abdominal Problems in Children with Congenital Cardiovascular Abnormalities.

BACKGROUND: Congenital cardiovascular abnormality is an important cause of morbidity and mortality in childhood. Both the type of congenital cardiovascular abnormality and cardiopulmonary bypass are responsible for gastrointestinal system problems. AIMS: Intra-abdominal problems, such as paralytic ileus, necrotizing enterocolitis, and intestinal perforation, are common in patients who have been operated or who are being followed for congenital cardiovascular abnormalities. Besides the primary congenital cardiovascular abnormalities, ischemia secondary to cardiac catheterization or surgery contributes to the incidence of these problems. STUDY DESIGN: Cross-sectional study. METHODS: In this study, we aimed to screen the intra-abdominal problems seen in patients with congenital cardiovascular abnormalities who had undergone surgical or angiographical intervention(s). Patients with congenital cardiovascular abnormalities who had been treated medically or surgically between 2000 and 2014 were analyzed retrospectively in terms of intra-abdominal problems. The patients' demographic data, type of congenital cardiovascular abnormalities, the intervention applied (surgical, angiographic), the incidence of intra-abdominal problem(s), the interventions applied for the intra-abdominal problems, and the results were evaluated. RESULTS: Fourteen (Group I) of the 76 patients with congenital cardiovascular abnormalities diagnosis were operated due to intra-abdominal problems, and 62 (Group II) were followed-up clinically for intra-abdominal problems. In Group I (10 boys and 4 girls), 11 patients were aged between 0 and 12 months, and three patients were older than 12 months. Group II included 52 patients aged between 0 and 12 months and 10 patients older than 12 months. Cardiovascular surgical interventions had been applied to six patients in Group I and 40 patients in Group II. The most frequent intra-abdominal problems were necrotizing enterocolitis and intestinal perforation in Group I, and paralytic ileus in Group II. Seven of the Group I patients and 22 of the Group II patients died. The patients who died in both groups had more than three congenital cardiovascular abnormalities in the same patient, and 80% of these patients had been operated for congenital cardiovascular abnormalities. CONCLUSION: The gastrointestinal system is involved in important complications experienced by patients with congenital cardiovascular abnormalities. The mortality rate was higher in operated patients due to gastrointestinal complications. Gastrointestinal complications are more frequent in patients with cyanotic anomalies. The presence of more than one congenital cardiovascular abnormality in a patient increased the mortality rate.

A case of Hirschsprung disease: does thyroid hormone have any effect?

Hirschsprung disease, the colonization defect of neural crest cells through the colon, is one of the reasons for functional obstruction in neonates. Furthermore, hypothyroidism has been known to be one of the causes of bowel hypomotility and pseudoobstruction. These two diseases are generally considered in the differential diagnosis. Although defective thyroid function has been found to be responsible for inappropriate neuronal migration in the brain, the effect of thyroid hormone on neural crest cell migration to the bowel has not yet been evaluated. Here, we report a case with Hirschsprung disease and congenital hypothyroidism, which may point to the need for future studies evaluating the interaction of colonic neural crest cell colonization and thyroid hormone.

Congenital mesoblastic nephroma rich from mitosis after in vitro fertilization: a case report.

Recently, various childhood tumors such as leukemia, neuroblastoma, hepatoblastoma, retinoblastoma, and central nervous system tumors in patients born after assisted conception have been reported. Although involvement of in vitro fertilization in the tumor pathogenesis was not established, the likely effect of assisted reproductive technology has been increasingly considered in these tumors in the last decade. Congenital mesoblastic nephroma is the most common renal tumor of infancy younger than 6 months associated with an overall good prognosis. The cellular variant of congenital mesoblastic nephroma, which occurs primarily in infants older than 3 months, confers a less favorable prognosis. We present a case of an atypical congenital mesoblastic nephroma with cellular elements in a 2-month-old infant who was born after in vitro fertilization. To our knowledge, this is the second congenital mesoblastic nephroma case and the first one with cellular variant reported to date in the English literature after a pregnancy induced by an assisted reproductive technology.

Hepatic laceration because of malpositioning of the umbilical vein catheter: case report and literature review.

Umbilical vein catheterization that is a common bedside procedure in the neonatal intensive care units is not without complication. The most common complications are thrombus formation, embolism, vessel perforation, hemorrhage, and infection. Complications related to the liver carry a high risk for mortality. Laceration is an ominous complication of umbilical vein catheter that is generally a result of direct injury through the liver parenchyma. Abdominal distension that develops gradually should alert the physician for a likely development of intrahepatic bleeding. Surgery is mandatory in patients with ongoing bleeding after the withdrawal of the catheter. Early diagnosis and treatment are lifesaving in these patients.

Angiokeratoma of clitoris: a rare lesion in an adolescent girl.

Angiokeratoma is a benign vascular lesion. It is the result of dilation of ectatic subdermal vessels and congested capillaries. Weakness of the vessel walls, either from acquired or congenital reasons, can cause formation of the lesion. Angiokeratoma is more common in males, in whom it forms on the scrotal wall. An equivalent form can occur in females, generally on the vulva. The clitoris is an extremely rare location. We present the case of a 14-year-old girl with a clitoral angiokeratoma. To our knowledge, this is the first presented case of clitoral angiokeratoma in a child in English published reports.

An unusual cause of paraphimosis: hemangioma of the glans penis.

Paraphimosis is a urologic emergency that occurs in uncircumcised boys. In untreated paraphimosis, increasing edema caused by vascular and lymphatic obstruction results in ominous sequelae such as penile gangrene, which leads to autoamputation in days or weeks. Most cases of paraphimosis are iatrogenic, but that condition has unusual causes as well. We present a very rare case of paraphimosis that occurred in a patient with a hemangioma of the glans penis. We suggest that the swelling of the hemangioma pushed the penile skin backward and caused paraphimosis. To our knowledge, such a presentation has not been previously reported in the English literature.

Renal parenchymal malacoplakia: a different stage of xanthogranulomatous pyelonephritis?

Malacoplakia is a rare inflammatory condition characterized by demonstrative Michaelis-Gutmann bodies, which are foamy histiocytes with distinctive basophilic inclusions. Malacoplakia is caused by the inadequate elimination of bacteria by macrophages or monocytes as a result of defective phagocytic activity. Xanthogranulomatous pyelonephritis is characterized by the destruction of renal parenchyma and its replacement by solid sheets of foamy lipid-laden macrophages. Prolonged infection of the kidney, which is frequently caused by an obstruction of the urinary tract, is the pathologic mechanism of that condition. We present a 6-year-old patient with a poorly functioning kidney who had a prolonged recurrent urinary tract infection. The results of histologic analysis revealed an inflammatory infiltration consisting predominantly of foamy and epithelioid histiocytes that contained round intracytoplasmic concretions characteristic of Michaelis-Gutmann bodies. We suggest that malacoplakia might be a stage of xanthogranulomatous pyelonephritis.

Actinomycosis: a differential diagnosis for appendicitis. A case report and review of the literature.

Actinomyces is a genus of gram-positive anaerobic or microaerophilic bacteria that colonize the upper respiratory and gastrointestinal tracts and the female genital tract. These organisms cause disseminated disease in the mouth, the respiratory system, and rarely in the gastrointestinal tract. The diseases produced by Actinomyces species result from the disruption of the barriers that allow the dissemination of the bacteria through the surrounding tissues. The appendix is often a nidus of Actinomyces infection, but a prompt diagnosis cannot be made without the results of histologic examination of the appendix. The treatment of choice for actinomycosis of the appendix is the high-dose parenteral administration of penicillin G for 2 weeks immediately after the diagnosis has been made and continued oral treatment with that agent for at least the next 6 months. We present the case of a 13-year-old adolescent boy with actinomycosis of the appendix that was identified by histologic examination after appendectomy.

Congenital teratoma of the tongue: a case report and review of the literature.

Teratomas of the head and neck are rare. They develop in neonates and are associated with feeding difficulty, airway obstruction, and difficulty in swallowing (which may cause polyhydramnios). Although some tumors consist of immature elements, teratomas of the head and neck are mature in nature, and excision is the most effective treatment. We present a case report of a neonate with a polypoid mass protruding from his mouth. He exhibited respiratory arrest immediately after birth and underwent intubation. The mass, which was completely excised, was histologically identified as a mature teratoma.

Renal cell carcinoma: case report and literature review.

Renal cell carcinoma arising from epithelial cells of the renal tubule is a highly aggressive and malignant tumor in all ages. Less than 2% of cases occur in childhood, relatively in older age group. Only a few pediatric series have been presented in the English literature. Tumor is presented with characteristic findings of flank pain, gross hematuria, and palpable mass. Although one half of the patients have metastasis at the time of diagnosis, most cases are currently being incidentally detected using improved imaging techniques. The overall prognosis in children appears to be similar to that in adults. Tumor stage and complete surgical resection have been reported as the most meaningful prognostic factors for the outcome. The incidence of metastatic disease is same as in adults. The effect of chemotherapy and immunotherapy, either preoperatively or postoperatively, is unclear. Cure is the most likely consequence in localized and completely resected tumors. Here, we present an 8-year-old boy with renal cell carcinoma demonstrating only hematuria without any pathological physical examination findings. The mass was described by abdominal ultrasonography and computed tomography in the left kidney. After the left nephroureterectomy, the patient was given no therapy.

Congenital sublingual teratoid cyst: a case report and literature review.

Head and neck dermoid cysts comprise less than 10% of all dermoid cysts. The sublingual location is very rare. One hypothesis for their etiology is that they arise from entrapment of epithelial debris or rests during the midline fusion of the first 2 branchial arches. They are classified as epidermoid, dermoid, and teratoid cysts. Sublingual cysts are slow-growing lesions generally seen in the second and third decades of life. They are rare in childhood. The cysts may interfere with swallowing and breathing. Rarely, they may show malignant transformation. Surgical extirpation is the treatment of choice.

Esophageal stenosis after coil embolization of an aortopulmonary collateral artery: report of a very unusual cause.

A 10-year-old boy who had previously undergone surgery for tetralogy of Fallot, pulmonary atresia, and ventricular septal defect was admitted with difficulty in swallowing and significant failure to thrive. His history included that he had 2 angiographically detected aberrant pulmonary arteries extending from the descending aorta to the right and left lungs, respectively. Both collaterals had been ligated during the corrective surgery; however, early postoperative evaluation revealed that the vessel that crossed behind the esophagus to the left lung had become recanalized. Coil embolization was performed to occlude this collateral. The patient had begun to develop swallowing difficulties 2 years after the embolization. Esophagography revealed a significant stricture in the middle of the esophagus, just anterior to the location of the coil in the vessel behind. The patient underwent a program of esophageal dilatation. This was successful, and he regained normal swallowing ability. To our knowledge, no similar case of esophageal stenosis has been reported in the English literature. We believe that inflammation surrounding the coiled aberrant artery, presumably caused by injury during the corrective surgery and resulting in hemorrhage, led to fibrosis around the vessel. This fibrosis also involved the adjacent esophageal wall, thus causing progressive stenosis.

High body mass index as a possible risk factor for pilonidal sinus disease in adolescents.

Pilonidal sinus disease (PSD) is common in adults, but it may also develop in adolescents. The intergluteal groove is a deep moist area in which broken hairs and foreign bodies can collect, often leading to infection. Only a few papers have been published considering PSD in children. For the present study, we retrospectively examined the data of operated patients with PSD. From that review, it appears that high body mass index (BMI) might be a risk factor for the development of PSD and its complications in older children. Fourteen young patients (12 males, 2 females, 12-18 years of age) underwent surgery for PSD. According to the BMI-for-age, eight of these patients (57, 1%) were overweight or obese. Five of them (35.8%) developed mild to moderate postoperative complications. Symptoms recurred in one patient (7.1%) whose BMI was considered as overweight. In patients with normal weight no early or late complications developed. Our findings suggest that high BMI in adolescents is a significant risk factor in the development of both symptoms and complications of PSD after surgical treatment.

Persistent pulmonary interstitial emphysema in an unventilated neonate.

Persistent pulmonary interstitial emphysema (PPIE) is a chronic form of pulmonary interstitial emphysema. The disease is histologically distinguished by large cysts and giant cells. Our patient was a female twin who was born at 31 weeks of gestation with a birth weight of 1,450 g. A chest X-ray at 2 hr after delivery was normal. At 12 hr, respiratory distress developed, and nasal continuous positive airway pressure (CPAP) was initiated. A chest film revealed left-sided pneumothorax. A chest tube was inserted, and the baby continued on nasal CPAP for 5 days. Her chest X-ray on postnatal day 4 showed diffuse cystic changes in the left lung. Thoracic computed tomography revealed multiple thick-walled cysts, the largest measuring 3 cm in diameter. Our case confirms that localized PIE may occur in preterm infants who have been treated with nasal CPAP only. Since this method is being used increasingly to avoid mechanical ventilation and in the postextubation period, it is very important that clinicians be aware of its complications.

Cerebral hydatid disease CT and MR findings.

In this study, we reviewed the computed tomography (CT, n=9) and magnetic resonance (MR, n=5) imaging findings of 11 patients with pathologically confirmed cerebral hydatid disease. Among our patients, there were 10 cases of Echinococcus granulosus and 1 case of Echinococcus multilocularis. Common CT and MR imaging findings of E. granulosus lesions were well-defined, smooth thin-walled, spherical, homogeneous cystic lesions with no contrast enhancement, no calcification, and no surrounding edema. E. multilocularis lesions showed calcified round, solid pattern with definite margins, contrast enhancement, and surrounding edema.

Treatment of pneumothorax in newborns: use of venous catheter versus chest tube.

BACKGROUND: The traditional treatment for pneumothorax is tube thoracostomy. Chest tube placement often involves complications, particularly in newborns. The aim of this study is to introduce the method of air drainage with venous catheter and to compare two different methods, chest tube placement and venous catheter insertion, in the treatment of pneumothorax in newborns. METHODS: We treated 72 newborn patients with pneumothorax over a 4-year period in neonatal intensive care units at two different medical centers. We randomly divided the patients into two groups. In group I, we treated the patients with chest tube placement technique. We used 18-gauge venous catheter connected to an underwater system to drain air in group II patients. The duration of the procedure, the period of time that the chest tube or catheter was left in place, and the complications associated with the two different procedures were statistically compared. RESULTS: The time required to perform the venous catheter procedure was significantly shorter than that for chest tube placement (P < 0.05). The duration of the device in place was also significantly shorter in group II than group I (P < 0.05). Only two minor complications were recorded in patients who underwent venous catheter placement, and this group's complication rate was significantly lower than the rate in the chest tube group (P < 0.05). CONCLUSION: Insertion of a venous catheter is a safe alternative to chest tube placement as a method of draining air from newborn patients with pneumothorax. This is an easy and quick bedside procedure and is particularly useful for newborn patients that require immediate air drainage.