Osman Faruk Senyuz

Pyloric atresia: 15-year review from a single institution

BACKGROUND Pyloric atresia (PA) is a rare pathology. Calder presented the first pyloric atresia case in 1749 and Touroff, Sussman, Meltz, and their colleagues presented the first successful operation in 1940. PA has 3 types of anatomic variations: (1) type A, pyloric membrane or web; (2) type B, the pyloric channel is a solid cord; and (3) type C, in which there is a gap between the stomach and duodenum. Associated anomalies also have been described. Epidermolysis bullosa (EB) and intestinal anomalies occur most often with this condition. METHODS Charts of 16 cases of congenital PA, aged 1 to 30 days and admitted to our department between 1986 and 2001, were studied retrospectively in regard to sex, prenatal diagnosis, presence of polyhydramnios, time of admission, pathology, type of operation, associated anomalies, and mortality rate. RESULTS In the study group, the male to female ratio was 5:3, the mean birth weight was 2,312 g and the mean age of admission was 6.5 days. The distribution of the anatomic variations was type A in 9 (56.3%) and type B in 7 (43.7%) of cases. Associated anomalies were present in 7 cases (43.8%). Familial occurrence was a prominent feature of our series. Seven of 16 cases occurred in 3 families. We performed 9 web excisions together with Heineke Mikulicz (H-M) pyloroplasty, 5 atresia excisions and gastro-duodenostomy, and 2 H-M pyloroplasties alone. Stamm gastrostomy was supplemented in 3 cases. The overall mortality rate (n = 9) was 56.3%. CONCLUSIONS Pyloric atresia can be managed successfully if it is diagnosed early. In this group of patients, congenital anomalies or septicemia are the main causes of mortality.

Bronchoscopic removal of tracheobroncheal foreign bodies: value of patient history and timing.

Abstract Between 1990 and 1999, 741 bronchoscopic procedures were performed in 698 children, 594 of whom were evaluated for foreign-body aspiration (FBA) (mean age 3.9 years, male:female 287/307). Based on the presenting symptoms, clinical outcome, and complications, two major groups were identified. Group 1 consisted of 438 patients with a definitive history of FBA. Most were admitted soon after the aspiration with sudden onset of symptoms such as coughing, choking, wheezing, and respiratory distress. Group 2 comprised 156 patients with chronic pulmonary infections and/or atelectasis without a definitive history of FBA. The most common radiographic finding was emphysema of one lung in group 1 (61.1%) and pneumonia in group 2 (70%). Among the patients in whom a FB was removed, the percentage of normal radiography was 17%. The FB was identified and removed in 83% of cases in group 1. The complication rate in this group was 9.8%, and all the complications were treated medically. Only 2 patients required intercostal drainage. In group 2, a FB was identified in 25% of bronchoscopic examinations and 17% of the patients developed complications. One of these patients underwent an urgent thoracotomy due to bilateral tension pneumothoraces and 2 required tracheostomies. Patients with a definitive history of FBA, even with a normal physical examination and radiographic findings, must undergo bronchoscopic investigation. Cases with late presentation and chronic pulmonary infection are at high risk. In this group care should be take in determining the indication and timing of bronchoscopy in order to prevent life-threatening complications.

Splenic artery ligation: A ten-year experience in the treatment of selected cases of splenic injuries in children

Splenic artery ligation (SAL) combined with either splenorrhaphy or partial splenectomy has been used as a spleen saving procedure in the management of massively bleeding splenic injuries. During the last 10 years, 37 children have been submitted to SAL following a selective management schedule. This study was jointly undertaken by two separate Pediatric Surgical Units in two different countries, in order to evaluate some preliminary observations published previously, with regard to; 1) the percentage of splenic injuries requiring ligation of the splenic artery; 2) the effect of this procedure on the arrest of bleeding; 3) the postoperative complications related to dearterialization of the spleen; 4) the immunological status after the operation and; 5) the postoperative imaging of the spleen using radioscintigrams and ultrasonograms. The mean age of the patients was 6.9 years and the follow up period ranged from 1 to 10 years. Thus, SAL was concluded to be an effective mode of treatment for rare cases of splenic injury unable to be treated nonoperatively or by splenorrhaphy alone. No postoperative complications were recorded in this series, while the immunological status remained undisturbed postoperatively and imaging of the spleen revealed intact and functional tissue with adequate healing.

Extensive intestinal aganglionosis

Extensive aganglionosis very close to the ligament of Treitz and total intestinal aganglionosis are rare forms of Hirschsprungs disease. In these cases, nutrition, fluid, and electrolyte balance are a problem. Although the myectomy-myotomy technique of Ziegler appears hopeful, no effective surgical method has been put into the practice for the treatment of these rare forms of Hirschsprungs disease. We report four cases with extensive intestinal aganglionosis, one of which was total intestinal aganglionosis with involvement of the stomach.

Intestinal rotation anomalies in childhood: review of 22 years' experience.

PurposeWe review our experience of treating intestinal rotation anomalies in infants and children in the 22-year period between 1978 and 2000.MethodsThe type of operation performed, postoperative complications, and mortality were compared in three age groups. Group 1 consisted of neonates ≪1 month old, Group 2 consisted of infants aged ≪1 year old, and Group 3 consisted of children aged ≫1 year old.ResultsThere were 101 infants and children, with a female : male ratio of 2 : 1. Of the 101 patients, 72 (71%) were neonates, with a mean age of 11.8 days (range 1–28 days); 20 (19.8%) were under the age of 1 year, with a mean age of 6.7 months (range 1–12 months); and 9 (8.9%) were ≫1 year of age, with a mean age of 6 years (range 1–9 years). Eighty-five (84%) patients underwent emergency procedures. Ladd’s operation was performed in all patients, with various additional procedures. The most frequent postoperative complications were adhesive intestinal obstruction, stoma necrosis, evisceration, and short bowel syndrome. The mortality rate was 36% in Group 1, 20% in Group 2, and 0% in Group 3.ConclusionsIn this series surgery was usually performed as an emergency procedure, with higher morbidity and mortality in newborns than in older infants and children.

Arterial carbon dioxide markedly increases during diagnostic laparoscopy in portal hypertensive children.

Several factors are responsible for hypercarbia during laparoscopic procedures. This study was undertaken because we observed a sudden increase in Paco2 in children with portal hypertension (PHT), which was unusual in healthy children undergoing laparoscopic procedures. Fifty-seven children underwent laparoscopic procedures under general anesthesia and were mechanically ventilated. Arterial blood samples were obtained 5 min after intubation (T0), 15 min and 30 min after CO2 pneumoperitoneum (T15 and T30), 5 min after desufflation (Tend), and 10 min after extubation (Text) for blood gas analysis. The changes in Paco2, pH, and ETco2 were statistically significant during the study periods in both groups (P < 0.05). The percentage of Paco2 increase between T0 and T15 was 11.5% and 20.1%, respectively, in the control group and the PHT group (P < 0.05). This increase reached 36.8% at T30 in the PHT group, whereas the control group had a 17.2% increase (P < 0.05). ETco2 presented similar changes. The variability in base excess, bicarbonate, Pao2, arterial oxygen saturation, and Spo2 was not significant in either group (P > 0.05). The Paco2 increased remarkably in children with PHT undergoing laparoscopy, with no difference in intrahepatic or extrahepatic origin. Limiting the duration of CO2 pneumoperitoneum and intraabdominal pressure and adjusting ventilatory variables to accommodate hypercarbia are of the utmost importance for such cases.

Esophageal Variceal Bleeding Secondary to Portal Hypertension: Endoscopic Sclerotherapy as the First-Step Treatment

BACKGROUND Variceal bleeding from the esophagus is an important cause of mortality and morbidity in children with portal hypertension (PHT). PATIENTS AND METHODS A series of 69 PHT cases (41 intrahepatic, 28 extrahepatic) have been evaluated in our department since 1990. According to the Child-Pugh classification, 49 cases were in class A, 16 cases were in class B, and 4 cases were in class C at admission. In our protocol, endoscopic sclerotherapy is performed in all patients, and the diagnosis is achieved directly by diagnostic laparoscopy and fine-needle liver biopsy. The procedure is applied under general anesthesia, and 1% aethoxysclerol (polidocanol) is injected paravariceally and intravariceally with the use of a flexible endoscope. RESULTS The Sugiura procedure was performed in nine patients who presented with recurrent bleeding episodes despite the strict sclerotherapy protocol. Liver transplantation was performed in two patients who were in Child class C. The total mortality rate in this series was 7% (5/69). CONCLUSION Endoscopic sclerotherapy, as presented herein, decreases the need for additional surgical interventions in children with PHT.

Congenital lobar emphysema ― A report of 5 cases

Five infants with congenital lobar emphysema whose main symptoms included dyspnea, cyanosis and recurrent respiratory infections, are presented herein. The most reliable diagnostic tool was plain chest X-ray films with antero-posterior and lateral views, while radio-isotopic investigation of the lung perfusion state took second place in the diagnosis of this disease. The affected lobes were the left upper lobe in four patients and the right middle lobe in one. Lobectomy was performed with good results in 4 patients, however, 1 infant was lost following an emergency thoracotomy.

Successful management of hepatic mucormycosis in an acute lymphoblastic leukaemia patient: a case report and review of the literature.

We present a case of hepatic mucormycosis in a 9‐year‐old boy with acute lymphoblastic leukaemia. Despite long‐term use of combined liposomal amphotericin B and posaconazole therapy, the lesion persisted and could only be treated by surgical excision. After surgery, antifungal treatment was continued with posaconazole. On follow‐up, the patient had two episodes of ascending cholangitis which were responsive to intravenous antibiotics. He is doing well at the moment in remission for 2.5 years. Mucormycosis was long regarded as a fatal infection with poor prognosis. With early medical and surgical management, survival rates increase. Isolated hepatic mucormycosis is rare and only seven cases were reported in the literature up to now. We wanted to emphasise the role of early surgery in patients with hepatic mucormycosis in view of the literature.

Radiological evaluation with Doppler sonography and multidetector CT angiography in congenital hepatic arteriovenous malformation in a newborn

Although hepatic arteriovenous malformations are rarely reported, they frequently have life-threatening complications such as cardiac failure and are associated with a high mortality rate. Consequently, accurate prenatal and early postnatal diagnosis is important and therapeutic procedures depend on the imaging features. We report the early postnatal sonographic, Doppler sonographic, multidetector CT and CT angiography findings of a congenital hepatic arteriovenous malformation in a newborn.