İbrahim Karnak

Sclerotherapy for lymphangioma in children

Although surgical excision has been considered to be the treatment of choice by most of the surgeons, sclerotherapy of lymphangioma has gained popularity during recent years. A prospective clinical trial was conducted to evaluate the efficacy of bleomycin and OK-432 sclerotherapies for treating lymphangioma in children. Fifteen patients were enrolled in the study conducted between 1998 and 2002. All patients were hospitalized. Bleomycin or OK-432 was injected into the lesion. Patients were observed in the hospital for 1 day. The response was considered as excellent in cases with total disappearance of the lesion. If the lesion was regressed more than 50% of the original size, the response was considered as good. Little or no change in the size of lesion was considered to be a poor response. Ten girls and five boys with ages ranging from 4 days to 12 years were treated. Five patients had been operated previously for lymphangioma. Most of the lesions were located only in the cervical region (n=13). Other cases revealed extensions into the mediastinum (n=1) or axilla (n=1). Bleomycin (n=8, 2.87+/-2.03 inj.), OK-432 (n=5, 2+/-1 inj.) or both in order (n=2, 6 and 16 injs.) were injected. No allergic reaction, scar formation or pulmonary complication was encountered. Fever (11%), local reactions (4%) and vomiting (2%) were encountered following a total of 55 injections. After a follow-up period of 6-36 months, the responses were excellent in 53.4%, good in 26.7% and poor in 6.6%. Macrocysts disappeared in the remaining 13.3% of patients who had mixed cervicofascial lymphangioma. Lower success rates were encountered among patients who had undergone prior surgery. Sclerotherapy with bleomycin and OK-432 is effective in the treatment of lymphangioma in children. Adverse effects are minor and rarely encountered. However, the surgeon should be alert for the possible serious complications. Sclerotherapy can be also used as an adjunctive therapy in the treatment plan of widespread or mixed forms of lymphangiomas. In the light of our results, sclerotherapy should be preferred as the primary mode of treatment in childhood lymphangiomas.

Congenital Lobar Emphysema: Diagnostic and Therapeutic Considerations

BACKGROUND/PURPOSE Congenital lobar emphysema (CLE) is characterized by overinflation of a pulmonary lobe and may present as a diagnostic and therapeutic dilemma. The authors have reviewed their experience to clarify the controversies about the diagnosis and management of CLE in children. METHODS Children who had CLE and were treated at our department between 1991 and 1998, inclusive, were reviewed. RESULTS Fourteen children consisting of 8 boys and 6 girls (age range, 25 days to 2.5 years) had CLE. Eight children (57%) were symptomatic at birth. Major presenting symptoms were dyspnea (n = 8, 57%), cyanosis (n = 6, 43%) and recurrent respiratory tract infection (n = 4, 28.5%). Chest x-rays and computerized tomography scans showed hyperaeration of the affected lobes in 14 and 12 patients, respectively. Pulmonary perfusion scan was performed in 8 patients and showed loss of perfusion in the affected lobe in 7 cases. The affected sites were left upper lobe (n = 8, 57%), right middle lobe (n = 4, 28.5%) and right upper lobe (n = 2, 14.5%). Congenital cardiac anomalies were encountered in 2 patients (14.5%). Ten children underwent lobectomy, and postoperative course was uneventful. Nonoperative management was performed in 4 cases (28.5%) presenting at older age with milder symptoms. In the latter, although symptoms subsided, radiological abnormalities persisted during the follow-up period of 3 months to 4 years. No mortality was encountered in the series. CONCLUSIONS The diagnosis of CLE is established by combined radiological and scintigraphic imaging methods. Surgical excision of the affected lobe is the appropriate treatment in all infants under 2 months of age and in infants older than 2 months presenting with severe respiratory symptoms. Infants older than 2 months presenting with mild to moderate respiratory symptoms associated with normal bronchoscopic findings can be treated conservatively. In conservative management, the children should be under close follow-up, and the family should be alarmed about the disease. Further studies are required to determine the long-term outcome of conservative management.

Colorectal carcinoma in children

BACKGROUND/PURPOSE Colorecta I carcinoma is extremely rare in children and presents with a poor prognosis. Surgical management and long-term follow-up of this entity are still obscure because of lack of data. Therefore, a retrospective clinical trial was performed to evaluate the clinical characteristics of childhood colorectal carcinoma and to determine the predictors of poor outcome. METHODS Records of children who had colorectal carcinoma and were treated at our unit between 1972 and 1997, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, clinical characteristics, diagnostic procedures, extent of disease, treatment methods, histological types, and outcome. A modified Dukes staging scheme was used. RESULTS There were 12 boys and 8 girls who were treated for colorectal carcinoma (range, 7 to 16 years). Predisposing conditions were encountered in 2 patients (10%), one with Blooms syndrome and another with familial occurrence of colonic carcinomas. Predominant symptoms were abdominal pain, vomiting, and rectal bleeding. Barium enema, ultrasonography, computerized tomography, and endoscopic procedures were used for the diagnosis. Rectosigmoid region was the most common site for the primary tumor (65%). All patients presented with advanced stages of disease (stage C, 7; stage D, 13). Surgical procedures were incisional biopsy (n = 4), palliative permanent colostomy (n = 4), segmental resection (n = 5), complete resection (n = 6), and rectal biopsy (n = 1). Peritoneum was the most common site of extensive intraabdominal disease followed by omentum majus and liver. The lung also was involved in 2 patients. Predominant histological type was mucinous adenocarcinoma (80%). All patients but one received adjuvant chemotherapy, and 2 received palliative radiotherapy. Thirteen patients died of disease in a period ranging from 1 day to 1 year after initial surgery. The fate of 4 patients who were discharged in a very ill status was unknown. Three patients were alive for 2 years to 4 years postoperatively, and one of them presented with end stage disease. CONCLUSIONS Delayed diagnosis, advanced stages of disease at presentation, and, most importantly, mucinous type of histology are the major determinants of poor outcome in childhood colorectal carcinoma. We emphasize that possibility of a malignant colorectal tumor should be considered for any childhood case with signs and symptoms of intestinal obstruction, intractable abdominal pain, alteration in bowel habits and gastrointestinal bleeding. Colorectal malignancy should not be excluded only on the basis of the patients age. Because of the steadily increasing incidence of precancerous bowel diseases and poor prognosis of colorectal carcinoma, childhood cases of bowel disorders should receive the same detailed and vigorous diagnostic evaluation and appropriate treatment as given to adult cases. Contrast studies, ultrasonography, computed tomography, and endoscopy are essential procedures for both confirming the diagnosis and detecting the extent of the disease.

Transverse testicular ectopia with persistent müllerian duct syndrome

Transverse testicular ectopia is rarely associated with persistent müllerian duct syndrome. The ninth pediatric case of transverse testicular ectopia with persistent müllerian duct syndrome is reported. The clinical and operative findings and treatment are discussed. The importance of abdominal exploration in the presence of two gonads in one inguinal side and the avoidance of dissection of müllerian structures has been stressed.

Bilateral Poland anomaly

Bilateral absence of the pectoralis major muscle with accompanying abnormalities of shoulder muscles has been reported in patients without Poland anomaly (PA). However, symmetric absence of pectoralis major muscles, hypoplasia of breasts and nipples with symmetric chest wall deformity and bilateral hand anomaly has not previously been reported. A 6-year-old girl with bilateral absence of pectoralis major muscles and hand involvement and symmetric chest wall deformity is, to our knowledge, the first known case of bilateral Poland anomaly.

Colonic atresia: surgical management and outcome

Abstract. Colonic atresia (CA) is a very rare cause of intestinal obstruction, and little information has been available about the management and predictors of outcome. A retrospective clinical trial was performed to delineate the clinical characteristics of CA with special emphasis on surgical treatment and factors affecting outcome. Children with CA who were treated in our department between 1977 and 1998 were reviewed: 14 boys and 4 girls aged 1 day to 5 months. All but 2 referred patients and 1 with prenatal diagnosis presented with intestinal obstruction. Plain abdominal X-ray films showed findings of intestinal obstruction in 14 cases; a barium enema demonstrated a distal atretic segment and microcolon in 4. The types of atresia were IIIa (n=9), I (n=6), and II (n=3). Type IIIa atresias were located proximal to the splenic flexure (n=8) and in the sigmoid colon (n=1), type I atresias were encountered throughout the colon; and all type II atresias were proximal to the hepatic flexure. Associated anomalies were multiple small-intestinal atresias (MSIA) (n=4), gastroschisis (GS) (n=2), pyloric atresia (n=1), Hirschsprungs disease (n=1), and complex urologic abnormalities (n=1). The initial management was an enterostomy in 15 patients (83%), including 2 referred and 2 with GS, and primary anastomosis in the remaining 3 (17%). Secondary procedures were the Santulli operation (n=2), colostomy closure and recolostomy followed by a Swenson operation (n=1), sacroabdominoperineal pull-through (n=1), and colostomy closure (n=1). Leakage was encountered in all primarily anastomosed patients. The overall mortality was 61%. Deaths occurred in patients with associated major anomalies (GS 2, MSIA 3, pyloric atresia 1) (55%) and in 3 patients who were initially managed by primary anastomosis (27%). Two additional patients died of sudden infant death syndrome (18%). Type I atresia was more common than in previously reported series and was associated with proximal multiple atresias. The initial management of CA should be prompt decompression of the intestine by an ostomy procedure, preferably end- or double-barrel. The type of surgery (primary anastomosis without prior colostomy) and associated abnormalities are the major determinants of poor outcome.

Rare congenital abnormalities of the anterior urethra

Congenital abnormalities of the anterior urethra other than hypospadias (valve, diverticulum, and megalourethra) are rare conditions and can be difficult to diagnose. Based on five recent cases of these urethral abnormalities, we review the embryology and the English literature, and have found reports of about 100 cases of valve and diverticulum and 50 cases of megalourethra. For all these abnormalities, the clinical presentation depends on age and the diagnosis depends essentially on voiding cystourethrography, which must show the entire urethra, and careful urethroscopic examination.

Hydatid Cyst: An Unusual Etiology for a Cystic Lesion of the Posterior Mediastinum

The cystic lesions of the posterior mediastinum include bronchogenic, enteric and intramural esophageal cysts, lymphangioma, and anterior meningocele. Hydatid cysts are usually located in the liver, lung, and brain. Although many uncommon locations have been reported, hydatid cyst has not previously been encountered as an isolated posterior mediastinal lesion. A 4-year-old boy with a posterior mediastinal cystic lesion, which has been proven to be a hydatid cyst, is reported.

Testicular enlargement in patients with 11-hydroxylase deficiency

Testicular nodules or tumors have been well described in patients with congenital adrenal hyperplasia (CAH) and usually associated with 21-hydroxylase deficiency. The authors report on a 11-hydroxylase--deficient patient presenting bilateral testicular enlargement and review the literature. Testicular biopsy was not very helpful to make differential diagnosis between adrenal rest hyperplasia and Leydig cell tumor. The size of testes regressed after steroid replacement therapy, and this observation was suggestive for adrenal rest hyperplasia. These findings suggest that bilateral testicular enlargement in patients with CAH may occur after excessive adrenocorticotrophic hormone stimulation of cells differentiated from unknown origin. Orchiectomy is not required but bilateral testicular biopsy must be performed.

Diaphragmatic injuries in childhood

Abstract The early detection and surgical repair of diaphragmatic injury is vital for saving the life of symptomatic children suffering from trauma. Furthermore, an accurate diagnosis may be difficult, particularly in right-sided diaphragmatic injuries. Fifteen children with diaphragmatic injury treated at our department between 1977 and 1998 were evaluated retrospectively. They included 9 boys and 6 girls, and consisted of 8 left- and 6 right-sided injuries, and 1 midline retrosternal injury, due to a blunt (n = 13) or penetrating (n = 2) trauma. The most frequent symptoms were dyspnea (86.6%), and abdominal pain and vomiting (13.4%). The diagnosis was confirmed preoperatively in 13 patients based on chest X-ray (n = 7), gastrointestinal series (n = 3), barium enema (n = 1), and computed tomography and/or ultrasonography findings (n = 2). Among these, a diagnostic delay occurred in 3 patients with right-sided injuries. A primary repair was performed through a laparotomy (n = 14) or thoracotomy (n = 1). Postoperative intussusception was the most frequent complication (n = 2). Diaphragmatic injury must be considered in any child who has sustained a thoracoabdominal trauma. Serial chest X-rays should be taken especially in right-sided injuries in which a considerable diagnostic delay may occur. Further radiological methods may be necessary to confirm the diagnosis. In addition, postoperative intussusception may be encountered following diaphragmatic repair.