Is acute appendicitis different in patients with sickle cell disease?

Nine patients with sickle cell disease (SCD) were operated upon at our hospital for acute appendicitis, comprising only 0.43% of the total appendicectomies performed at our institution. Three appendices were acutely inflamed and six (66.7%) were perforated. Histologic evaluation of the six perforated specimens revealed congestion and haemorrhage by sickled erythrocytes (RBCs) in addition to acute transmural inflammatory cell infiltrates. The mucosa was extensively ulcerated, with haemorrhage both within the lumen and in the appendiceal wall. The blood vessels were dilated and packed with sickled RBCs. Two of the three acutely inflamed appendices showed features of acute transmural appendicitis, with marked congestion and haemorrhage by sickled RBCs. The third did not show any acute inflammatory cell infiltrate, however, the mucosa was partly ulcerated with both mucosal and intraluminal haemorrhage. These findings suggest that acute appendicitis is different in patients with SCD: while it is not common, when it does develop it has a rapid course with a high incidence of perforation due to blockage of appendiceal vessels by sickled RBCs, leading to transmural necrosis.

Concurrent left congenital diaphragmatic hernia and esophageal atresia: case report and review of the literature.

Coexisting congenital diaphragmatic hernia and esophageal atresia is an extremely rare phenomenon. Details of one infant with such a combination is presented, and the literature on the subject is reviewed.

Laparoscopic versus open cholecystectomy in children.

Twenty-one consecutive laparoscopic cholecystectomies (LC) were compared with 29 consecutive open cholecystectomies (OC). Sickle-cell disease (SCD) was the most common reason for cholecystectomy in both groups. The average length of operative time for LC was significantly longer than that of OC (P=0.0149). In 1 patient there was conversion from LC to OC due to severe adhesions. Common bile duct (CBD) stones were diagnosed in 8 (27.6%) of the OC group; in 4 of them the diagnosis was made preoperatively by ultrasound, in 4 by intraoperative cholangiogram. All 8 patients required CBD exploration, and 2 had additional transduodenal sphincteroplasties. In the LC group 5 patients (23.8%) had CBD stones. All had (ERCP) endoscopic retrograde cholangiopancreatography sphincterotomy, and stone extraction followed by LC. ERCP is a necessary adjunct to treatment if LC is to be contemplated. Six patients in the OC group developed complications, while only 4 patients in the LC group developed minor complications. The length of hospitalization after LC was significantly shorter than after OC (P=0.0150). LC is the procedure of choice in the management of cholelithiasis in children, especially those with SCD.

Incidence of urological anomalies in children with urinary tract infections in India.

A total of 2,970 infants and children with symptoms suggestive of urinary tract infection (UTI) were investigated. Diagnosis was based on clinical, microbiological, radiological, and sometimes endoscopic investigations. Quantitative culture of an uncontaminated sample of urine is the most sensitive screening test. An additional 548 children had positive physical findings such as enlarged kidneys and/or bladder and were excluded from this study. There were 2,970 children with urinary symptoms but no definite positive physical findings, and this group is discussed in detail; 73% (2,168) were boys and 27% (802) were girls. The commonest presenting symptoms were frequency of micturition, enuresis, and straining on voiding. One hundred sixty-six had significant bacteriuria and were fully investigated. Only 148 children completed the investigations and treatment; 130 had an underlying structural abnormality causing stasis of urine. It is well known that removal of the cause of the stasis will help to prevent further infections in most patients, and also reduces the effects of back-pressure on the upper tracts. This study illustrates: (1) the importance of proper urine culture technique; (2) the high incidence (88%) of structural abnormalities causing UTI in children; and (3) the importance of investigating all children with proven UTI to determine the cause of stasis. In India, the patterns of UTIs and their causes are markedly different from those published in the English literature.

Acute intestinal obstruction.

Nine hundred and four children with intestinal obstruction were studied. Necrotising enteritis was the commonest cause of intestinal obstruction in children. Acute intussusception was the second commonest cause in the whole group and the commonest cause in children under 1 year of age. Less common causes included band obstruction, sub acute intestinal obstruction and remnants of vitello-intestinal duct. A steady and significant improvement in the results of treatment was noted in children suffering from necrotising enteritis in the study period.

Experience in the diagnosis of Hirschsprung's disease using the anomanometer.

Results of using electromanometry as the sole preoperative diagnostic test for Hirschsprung's disease are presented. Out of a total of 113 infants and children diagnosed to be suffering from the disease, manometry was the initial diagnostic test in 92 infants and children. In nine, manometry confirmed the diagnosis after a colostomy has been done. In 11, barium enema had diagnosed the condition at a peripheral hospital and manometry confirmed the diagnosis. In one, manometry was interpreted as normal, but laparotomy revealed Hirschsprung's disease. Histologic corroboration has been obtained in 97 instances. In 14, no operative treatment was undertaken and, hence, no histologic examination was made; in 2, histologic examination is still pending.

Neuromuscular Reflexes of Rectal Continence.

The purpose of this paper is to describe the nerve pathways, reflex arcs and sphincter muscles responsible for the sensory and motor mechanisms of rectal continence. The data from which the reflex arcs were deduced were obtained from anal and rectal pressure profiles in children with normal and malformed rectums, and in children with injuries and deformities of the spinal cord which affect the neuromuscular control. These profiles were correlated with the clinical state of continence and with the known anatomy of both normal and abnormal states.