Cystic mesenchymal hamartoma arising in intrathoracic heterotopic liver: a case report.

Intrathoracic heterotopic liver tissue is an exceedingly rare clinical entity. In most patients, it is associated with other congenital defects, including cardiac anomalies, congenital diaphragmatic hernia, pectus excavatum, and intralobar pulmonary sequestration. Although heterotopic liver could potentially lead to the same benign liver diseases that can affect the mother liver, the association of heterotopic liver tissue with tumors in childhood is extremely rare. We describe a unique case of cystic mesenchymal hamartoma arising from an intrathoracic heterotopic liver that was found incidentally during a diaphragmatic eventration repair. Association of ectopic liver tissue with mesenchymal hamartoma has not previously been reported in the literature.

Mesenteric cystic lymphangioma associated with intestinal malrotation in a newborn.

Mesenteric cystic lymphangiomas are uncommon benign cystic tumors rarely associated with other congenital malformations. We herein describe a case of a 9-day-old female presented with a two-day history of bilious vomiting, feeding intolerance, weakness and lethargy. Preoperative studies were diagnostic of intestinal malrotation. At laparotomy a cystic mesenteric mass was identified. Histopathological examination confirmed the diagnosis of mesenteric cystic lymphangioma. The exact aetiology of mesenteric lymphangioma is unknown and this case supports the theory that lymphangioma is a primary congenital malformation.

Anastomotic strictures following esophageal atresia repair: a 20-year experience with endoscopic balloon dilatation.

OBJECTIVES: The aim of the study was to evaluate the safety, effectiveness, and long-term results of endoscopic balloon dilatation in children with anastomotic strictures following esophageal atresia (EA) repair. PATIENTS AND METHODS: From January 1988 to January 2008, 59 patients were treated with balloon dilatation for anastomotic stricture following EA repair. Indication for dilatation was dysphagia of varying degree. Outcome parameters included the number of dilatations, response to dilatation, effectiveness, and complications. Response to dilatation was considered excellent when there was no need for any additional dilatation for recurrent dysphagia, satisfactory when up to 5 dilatations were required, and fair when >5 sessions were required. The treatment was considered effective when dysphagia was grade 0 or 1 for >12 months after the last dilatation session. RESULTS: A total of 165 balloon dilatations were undertaken, with an average of 279 per patient (range 1-9). Age range at diagnosis was 1 to 36 months (mean 10.5). Response to dilatation was excellent in 21 cases (35.6%), satisfactory in 26 (44.1%), and fair in 12 (20.3%). The treatment was effective in 47 patients (79.7%) and ineffective in 12 (20.3%). The median follow-up period was 19.5 months. Four patients underwent surgery; in 1 patient a retrievable stent was placed. No perforation occurred. CONCLUSIONS: Endoscopic balloon dilatation can be accomplished safely and effectively as a first-line therapy for the management of anastomotic strictures following EA repair.

A case of maturing perineal lipoblastoma in an infant.

OBJECTIVE: To report the unusual occurrence of a perineal lipoblastoma presenting as a painless enlarging mass in an infant. CASE PRESENTATION AND INTERVENTION: A 7-month-old boy presented with a 6-month history of an asymptomatic progressively enlarging perineal mass. Imaging studies were unable to provide a diagnosis. Complete resection of the mass was achieved and there was no recurrence at 18-month follow-up. Histopathologic findings were consistent with a maturing perineal lipoblastoma. CONCLUSION: This case report serves as a reminder to physicians and especially pediatric surgeons to consider perineal lipoblastoma in the differential diagnosis of soft tissue perineal masses in infancy.

Ancient Greek terminology in pediatric surgery: about the word meaning.

It is widely accepted that the medical terminology has its roots in ancient Greek and Latin. Greek words have been used not only in the field of medicine but also in every day language for many centuries. The aim of this article is to provide an abbreviated guide to the etymology and the meaning of Greek words used in the medical literature today, emphasizing on the field of pediatric surgery. Thus, the term paediatric is constructed from the words paedion, meaning "child", and iatriki, meaning "medicine", literally, "medicine for children." Surgery, however, is not a Greek word. The corresponding Greek term is chirourgiki, derived from cheir and ergon, that is, "hand and action," meaning the action made by hands. This term is also found in the French and German medical literature as chirourgie and chirurgie, respectively. Some general terms in surgery are also of Greek origin. The word trauma has been transferred into the English literature without modification and comes from the verb diatitreno, meaning to "penetrate." Other such terms include diagnosis, from the verb diagignosko, meaning to "discriminate"; symptom, from the verb sympipto, that is, "to coinside"; and the adjectives clinical and clinic, from the noun klini, meaning "bed." Focusing on special fields of pediatric surgery, the etymology and the meaning of Greek words used in the medical literature will be shown and analyzed.

Infections in a pediatric patient cohort with acute lymphoblastic leukemia during the entire course of treatment.

GOALS: To assess the type, frequency, severity, and outcome of all infectious episodes in a pediatric patient cohort with acute lymphoblastic leukemia (ALL) from a single institution during the entire length of leukemia treatment. PATIENTS AND METHODS: Eighty-six patients were treated according to a modified ALL Berlin-Frankfurt-Munster protocol. Retrospective analysis of all types of infections according to the treatment phase and the degree of neutropenia is presented. RESULTS: A total of 610 infectious episodes were recorded. Most infections were documented during maintenance (57%), followed by the induction phase (20.3%). During maintenance, 347 episodes were encountered, with nonspecific viral upper respiratory tract infections (URIs) being the commonest diagnosis (40.0%). Additionally, 38 of 58 total specific viral infections occurred during maintenance: 16 herpes simplex, 7 varicella, 10 herpes zoster infections [varicella-zoster virus (VZV), 45%]. The majority of bacteremia and fever of unknown origin occurred during induction (20%). The number of Gram-negative bacteremia was 50% of the total (26 of 52). The majority of the infections (59.5%) occurred without neutropenia [absolute neutrophil count (ANC) >1,000 microl(-1)]. Fewer infections (9.3%) were recorded with concurrent very severe neutropenia (ANC <100 microl(-1)), although 38.5% of positive blood cultures were documented with severe neutropenia. No infection-related fatality occurred. CONCLUSIONS: Most of the severe infections occurred during induction. Gram-positive bacteremia and Gram-negative bacteremia were almost equal. URIs were the commonest infections during the entire treatment and during maintenance. Specific viral infections represented a smaller percentage of the total (VZV was the commonest pathogen). Infectious complications represented a significant morbidity factor, but notably, mortality was negligible.

Multiple failed urethroplasties: definitive repair with the Duckett island-flap technique.

BACKGROUND/PURPOSE: The correction of postoperative complications after hypospadias repair presents frequently serious problems, and the results are not always satisfactory. The aim of this study is to present our experience with the tubularized island flap technique (Duckett procedure) in patients who had been submitted to repeated unsuccessful attempts for the repair of hypospadias and its complications. METHODS: During an 8-year period (1994 through 2001), 21 patients, aged 4 to 18 years, presented with severe recurrent hypospadias (3 to 13 times). In all patients, the urethral orifice was quite proximal because of disruption of the neourethra and was associated with severe penile ventriflexion. In addition, there were diverticula with hair ingrowth in 5 boys and lack of the prepuce in 4. All patients underwent the Duckett island-flap technique. The inner layer of the prepuce was used in 17. The dorsal penile skin was used for the island flap formation in the remaining 4 patients in whom the prepuce had been resected during the previous operations. RESULTS: After repair, the meatus was located at the top of the glans in all patients. There were 5 complications (24%): distal fistula near the glans (n = 1), meatal stenosis (n = 1), diverticula at the proximal anastomosis (n = 2), and anastomotic stenosis (n = 1). All of them were treated successfully by meatotomy, fistula closure, diverticula tapering, and dilatations, respectively. CONCLUSIONS: The island-flap technique gives satisfactory results in patients with multiple failed urethroplasties; it is applicable even in boys with resected prepuce and has an acceptable complication rate.

The function of the spleen in adults after ligation of the splenic artery of the traumatized spleen in childhood.

BACKGROUND: Ligation of the splenic artery (LSA) has been successfully used as a spleen-saving procedure in rare cases of splenic trauma in children in which management with splenorrhaphy or partial splenectomy alone was not possible. There are no data regarding the long-term effects of the procedure on the functional status of the spleen. The purpose of this study is to present and discuss our clinical and laboratory findings in adults who underwent LSA in childhood. METHODS: Our first 2 patients in whom LSA was done at ages 4 and 2 years in 1977 underwent the following examinations in the year 2000: 1, imaging of the spleen; 2, immunologic studies; and 3, peripheral blood tests. Their ages at reexamination were 27 and 25 years, respectively. RESULTS: Results were as follows: triplex ultrasound revealed normal size and echomorphology; Doppler techniques revealed normal vasculature; 99mTc-Tin colloid scanning revealed normal uptake. Immunoglobulins (IgG1 to IgG4, IgA, IgM, IgE), complement fraction (C3, C4), antibodies response to vaccinations, and peripheral blood tests all had normal results. No Howell-Jolly bodies were found. CONCLUSION: Laboratory investigations in adults with LSA during childhood disclosed undisturbed function of the spleen. LSA can be used as an adjunct to splenorrhaphy in children with rare splenic injuries involving major hilar vessels.