Congenital intrinsic duodenal obstruction associated with rotational abnormalities of the gut

The causes of congenital duodenal obstruction are divided into extrinsic and intrinsic. Intrinsic causes include atresia, stenosis and diaphragm while the commonest extrinsic cause is congenital bands in association with malrotation. The mere presence of malrotation and congenital bands should not be taken for granted that these bands are the cause of obstruction and intrinsic causes should also be excluded. We describe our experience with six children who had rotational abnormalities associated with intrinsic duodenal obstruction. The medical records of 35 children with the diagnosis of intrinsic duodenal obstruction were retrospectively reviewed for age at diagnosis, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment and outcome. The operative findings and procedures were obtained from the operative notes. Six [17.1%] of the 35 children had rotational abnormalities associated with intrinsic duodenal obstruction. All were females. Their age at presentation ranged from 2 days to 1.5 years. Two of them had Down's syndrome and two had congenital heart disease. Two had situs inversus which was diagnosed preoperatively. The remaining 4 had malrotation with congenital bands. The diagnosis in these 4 was made intraoperatively. Intraoperatively, two had duodenal atresia while the other 4 had duodenal diaphragm. In addition to the definitive operative procedure, all had appenedecectomy. Postoperatively, all did well. Rotational abnormalities including situs inversus are among the rare but interesting anomalies associated with intrinsic congenital duodenal obstruction. The presence of malrotation with congenital bands and congenital duodenal obstruction does not exclude the possibility of associated intrinsic cause which should be looked for intraoperatively. This to obviate the possibility of further operative therapy. Appendecectomy should also form part of the initial operative procedure

Unusual presentation of congenital asplenia

Congenital asplenia is a very rare condition that may be discovered incidentally or cause thrombocytosis simulating essential thrombocythemia. Pneumococcal sepsis however is often the first sign of the disease and if not recognized and treated promptly it may be fatal. We report a case of congenital asplenia in a child with alpha-thalassaemia and hereditary spherocytosis presenting unusually as an abdominal tumour

Unusual Mesoblastic nephroma: a report of two cases

Congenital mesoblastic nephroma [CMN] is a very rare tumour of early infancy with 80% of the cases being diagnosed within the first month of life. Complete surgical excision is almost always curative. We report two unusual large renal tumours in a 22-month-old boy and a 4-year-old girl who were diagnosed preoperatively as rhabdoid tumour and Wilms' tumour respectively. One of them was subjected unnecessarily to preoperative chemotherapy. Histological evaluation after complete radical excision demonstrated CMN

Iliopsoas abscess in infants and children

This report describes 3 patients [22-day-old, 40-day-old and 1.5-year-old] with a diagnosis of iliopsoas abscess. Aspects of diagnosis and management are discussed

Intestinal malrotation and volvulus beyond the neonatal period

Malrotation is a rare congenital malformation, commonly discovered in the first month of life. The diagnosis is however missed or delayed in a significant number of cases. This is especially so in children presenting beyond the neonatal period were they often present with a variety of atypical and frequently non-specific symptoms that may cause delay in diagnosis and treatment. This is a retrospective review of our experience with 11 infants and children with the diagnosis of mal-rotation presenting after the neonatal period treated at our hospital between 1990 and 2000. Sixteen infants and children were treated at our hospital during the study period. Three presented with intrauterine volvulus. Two presented within the first week of life with malrotation and volvulus. The remaining 11 were diagnosed late. In the majority, their symptoms dated back to birth. Six were malnourished and a large number were subjected to a variety of investigations including repeated upper and lower contrast studies and some received a number of unnecessary treatment modalities. All were operated on and underwent Ladd procedures and appendecectomies. In this group, there was no mortality but one of the patient required bowel resection. Malrotation represents a spectrum of anomalies that can present from as early as intrauterine to as late as adulthood. To obviate delay in diagnosis of malrotation with its catastrophic sequalae, physicians caring for these children should be aware of this and the possibility of malrotation should be suspected in any child who presents atypically with recurrent abdominal pain, vomiting especially if bile-stained, abdominal distension, and malnutrition. Once malrotation is diagnosed, the patient should be treated with a Ladd procedure. We advocate this even in asymptomatic children with incidentally diagnosed malrotation as it is difficult to predict the timing of midgut volvulus which is variable and unpredictable. We also advocate adding an appendecectomy as this will simplify their future management in case they present with abdominal emergency

Spontaneous central venous catheter fracture and the intracardiac migration in an infant: Successful percutaneous retrieval

The use of central venous lines including totally implantable venous devices has revolutionized the care and management of infants and children in general and oncology patients in particular and, although considered to be generally safe, they are however associated with significant morbidity. One of the rare but potentially life-threatening complications is catheter fracture and migration, which is commonly seen in adults but rarely in children. We report here the case of an infant with catheter fracture and migration to the right ventricle, which was successfully retrieved by a percutaneous transfemoral endovascular technique

Intussusception in children: factors affecting the final outcome

Fifty-nine infants and children [40 males and 19 females] with 60 episodes of intussusceptions were treated at our hospital over a period of 10 years. Their medical records were reviewed for age at diagnosis, sex, clinical features, duration of symptoms, method of treatment, morbidity, mortality and outcome. Their ages at diagnosis ranged from 5 days - 11 years, but the majority [79.7%] were below 1 year of age, clinical features included abdominal pain [72.9%] vomiting [91.5%] bleeding per rectum [78%] diarrhea [33.9%] and clinically 35 [39.3%] had a palpable abdominal mass, 22 [37.3%] had abdominal distension and 22 [37.3%] were dehydrated. The classic triad of abdominal pain, abdominal mass and bleeding per rectum was seen in only 21 [35.6%]. Twenty seven [45.8%] presented with in 24 hours of symptoms 17 [28.8%] had symptoms for 24-48 hours, while 15 [25.4%] had symptoms for more than 48 hours. Barium enema reduction was attempted in 45 with success in 18 [40%]. laparotomy was done in 41,9 underwent operative reduction only, 14 had reduction and appendicectomy, 6 had reduction and resection of Meckel's diverticulum and 12 had bowel resection. The majority had ileo-colic intussusceptions [84.7%] but 6 had ileo-ileal intussusceptions. A lead point was identified in 11 [18.6%]. Meckel's diverticulum was seen in 6 while 2 had non-Hodgkin's lymphoma. Excluding those with a lead point, 10 required bowel resection and all of them presented late with a mean duration of symptoms of 75 hours [30 hours-120 hours] Only one [1.7%] had a recurrence four days after a successful barium enema reduction. This was reduced by a repeat enema. There was no mortality but 10 [24.4%] had postoperative complications including chest infection in 2 [4.9%] wound infection in 5 [12.2%] and adhesive intestinal obstruction in 3 [7.3%]. The average hospital stay was 2.5 days after barium enema reduction, 6 days after manual reduction and 9 days after bowel resection. Intussusception although not a very common condition at our hospital, nevertheless it is an important cause of acute abdomen in children. Hydrostatic reduction using barium or air enema remains the treatment of choice but the success of this depends on several factors mainly early diagnosis. Operative reduction of intussusceptions is an invasive procedure known to be associated with an increased length of hospital stay. Hospital cost and increased morbidity. This is specially so when it is associated with bowel resection. This calls for early diagnosis of intussusception and referral to specialized centers with experienced paediatric surgeons and radiologists

Salmonella as a causative organism of various infections in patients with sickle cell disease

Patients with sickle cell disease [SCD] are susceptible to a variety of bacterial infections, including infections caused by Salmonella species. We sought to identify the most common infections caused by Salmonella spp. in SCD patients at our hospital, and to determine their mortality rates and susceptibility to commonly used antimicrobial agents. Patients and We conducted a retrospective review of all Salmonella infections in SCD patients admitted to Qatif Central Hospital during the last 11 years. During the 11 years of the study, 331 episodes of Salmonella infection occurred in 319 patients. The incidence of Salmonella infection in SCD patients was 11.5% compared to only 0.65% in the general hospital population. The commonest sites of Salmonella infection were blood [42% of isolates], followed by gastrointestinal tract [23.5%] and the skeletal system [22.5%]. Salmonella infection was commonly seen in patients less than 15 years of age. The case fatality rate in patients with Salmonella sepsis was 2.2% [3/139], but there were no deaths associated with other types of Salmonella infections. Ampicillin and cotrimoxazole resistance occurred in 29% and 24% of the isolates, respectively, but only 0.6% were resistant to ceftriaxone and 1.8% had intermediate resistance to ciprofloxacin. Conclusions: Salmonella is a common cause of sepsis and osteomyelitis in patients with SCD in our locality. Empirical antimicrobial therapy should cover the likely pathogens, including Salmonella species