Ahmed H. Al-Salem

Herniation through the foramen of Morgagni: early diagnosis and treatment

Abstract The majority of congenital diaphragmatic hernias (CDH) occur through the foramen of Bochdalek; herniation through the foramen of Morgagni (MH) is rare. Fifteen children (12 males and 3 females) with congenital MH (7 right, 3 left, 5 bilateral) were treated over a period of 15 years, comprising 11% of a total of 135 children with different types of CDH. The majority (12, 80%) had repeated chest infections. In 1 the hernia was discovered accidentally during evaluation of trauma, and another presented in the neonatal period with acute respiratory distress (ARD). The diagnosis was made on plain lateral chest radiograph when there was anterior herniation of bowel loops, and in these cases the diagnosis was confirmed by barium enema. Computed tomography was useful in preoperative diagnosis when the hernia contents were solid. All patients were operated upon transabdominally except 1, and in all cases there was a hernia sac. Associated anomalies were present in 10 (66.7%) patients: 4 (26.7%) had malrotation, 4 (26.7%) congenital heart disease, and 3 (20%) Downs syndrome. Our study shows a relatively high frequency of MH in our patients. MH rarely presents in the neonatal period, but when it does, it causes ARD. The majority of patients with MH present beyond the neonatal period with repeated attacks of chest infection, and although late-presenting MH is relatively benign, it nevertheless causes significant morbidity. Thus, clinical awareness and early diagnosis and surgical treatment are important factors.

Fistula-in-ano in infancy and childhood

The authors report the results of a retrospective study of 36 patients with fistula-in-ano (FIA) and/or perianal abscess (PA) presenting during a 3-year period. In 76.2% of the 21 patients with FIA, the fistulae developed in the first 2 years of life. For eight of the 16 patients who underwent fistulectomy, histological examination of the excised fistulae showed an epithelial lining of the tract mixed with stratified squamous, transitional and columnar epithelium. The early onset of FIA, the high percentage of bilateral and multiple fistulae, and the presence of these types of epithelium lining support a congenital etiology of FIA in children. In boys, a causal relationship exists between PA and FIA.

Sequential endoscopic/laparoscopic management of cholelithiasis and choledocholithiasis in children who have sickle cell disease

BACKGROUND/PURPOSE Cholelithiasis and choledocholithiasis are common complications of sickle cell disease (SCD). With the recent advances in laparoscopic cholecystectomy (LC), which has been used successfully for the management of cholelithiasis in children who have SCD, exclusion of choledocholithiasis before LC is of great importance. METHODS Eighteen children who had SCD, cholelithiasis, and choledocholithiasis were treated at our hospital. Seven were treated with open cholecystectomy (OC) and common bile duct (CBD) exploration, and two were treated with transduodenal sphincteroplasty. The remaining 11 patients underwent endoscopic retrograde cholangiopancreatography (ERCP), sphincterotomy, and stone extraction followed by laparoscopic cholecystectomy (LC). RESULTS A dilated CBD noted on ultrasound, elevated alkaline phosphatase, elevated total bilirubin of more than 5 mg/dL, history of pancreatitis, either singly or in combination, should raise suspicion of choledocholithiasis, and these patients together with those who have choledocholithiasis detected on ultrasound should undergo ERCP to confirm and extract the stones before LC. CONCLUSION This sequential approach of endoscopic sphincterotomy and stone extraction followed by LC is a safe and effective approach for the management of cholelithiasis and choledocholithiasis in children who have SCD.

Congenital spigelian hernia and cryptorchidism: cause or coincidence?

Abstract Congenital spigelian hernia (SH) is very rare in the pediatric age group. This is a report of two cases of SH in 1-week and 3-month-old male infants. A review of the literature revealed only 35 cases of SH in children younger than 17 years of age, bringing the total including our 2 cases to 37. There were 25 males and 12 females, a ratio of 2.1:1. Their ages ranged from 6 days to 17 years (mean 4.52 years). The hernia was situated on the right side in 13, the left side in 19, and was bilateral in 4. In one case the side of the hernia was not mentioned. In 29 cases the hernia was spontaneous while in 5 it was caused by trauma. In 3 children the hernia developed postoperatively, in 2 following repair of a congenital diaphragmatic hernia and in 1 following excision of a mediastinal neuroblastoma. Two children presented with a strangulated SH. Eleven of the 35 previously reported children had associated conditions; in 5 there was an ipsilateral undescended testis (UDT). Our two infants with SH also had an ipsilateral UDT. The significance of this association is discussed.

Congenital Morgagni's hernia: A national multicenter study

BACKGROUND Congenital Morgagnis hernia (CMH) is rare and represents less than 5% of all congenital diaphragmatic hernias. This is a national review of our experience with CMH outlining clinical presentation, methods of diagnosis, associated anomalies, treatment, and outcome. PATIENTS AND METHODS The medical records of all patients with the diagnosis of CMH treated at four pediatric surgery units in Saudi Arabia were retrospectively reviewed for age at diagnosis, sex, presenting symptoms, associated anomalies, diagnosis, operative findings, treatment, and outcome. RESULTS During a 20-year period (January 1990-December 2010), 53 infants and children with CMH were treated. There were 38 males and 15 females. Their age at diagnosis ranged from 1 month to 9 years (mean 22.2 months). Forty-three (81%) presented with recurrent chest infection. Twenty-two (44.5%) had right CMH, 15 (28.3%) had left-sided hernia and 16 (30.2%) had bilateral hernia. In 7, the diagnosis of bilaterality was made at the time of surgery. Associated anomalies were seen in 38 (71.7%). Twenty-one (39.6%) had congenital heart disease, 8 (15%) had malrotation, and 15 (28.3%) had Down syndrome. All were operated on. Twenty-nine (54.7%) underwent repair via an open approach. The remaining 24 (45.3%) underwent repair using minimal invasive surgery, laparoscopic-assisted hernia repair (19 patients) or totally laparoscopic approach (5 patients). At the time of surgery, the hernia sac content included the colon in 33 (62.3%), part of the left lobe of the liver in 13 (24.5%), the small intestines in 11 (20.75%), the omentum in 5 (9.4%), and the stomach in 4 (7.5%). In 12 (22.6%), the hernia sac was empty. When compared to the open repair, the laparoscopic-assisted approach was associated with a shorter operative time, an earlier commencement of feeds, less requirement for postoperative analgesia, a shorter hospital stay, and better cosmetic appearance. There was no mortality. On follow-up, 2 (7%) of the open surgical group developed recurrence. CONCLUSIONS CMH is rare and in the pediatric age group commonly presents with recurrent chest infection and has a high incidence of associated anomalies, commonly congenital heart disease and Down syndrome. We advocate a laparoscopic-assisted approach to repair CMH. This is a simple technique that produces a sound repair, and when compared with the open approach it takes less operative time, requires less analgesia, allows earlier commencement of feeds, is associated with a shorter hospital stay, and has a better cosmetic outcome.

Splenic abscess and sickle cell disease

This is a report of our experience with 10 cases of splenic abscess in patients with sickle cell disease (SCD). All presented with fever and abdominal pain and were found to have a tender enlarged spleen. Two were found to have a ruptured spleen and five of them were septicemic on presentation. Although both ultrasound and CT‐scan of the abdomen were of diagnostic value, we found CT‐scan more accurate and reliable in the diagnosis of splenic abscess. Ultrasound and/or CT‐scan should be used routinely in the evaluation of SCD patients who present with fever and abdominal pain, especially if they have a tender enlarged spleen. Diagnostic aspiration under CT‐scan or ultrasound guidance should be used in doubtful cases to differentiate between splenic abscess and a large splenic infarct. All our patients were managed by peri operative antibiotics and splenectomy with no mortality. Salmonella was the commonest causative organism. Although CT‐guided aspiration of splenic abscess is being advocated recently, we feel splenectomy should be the treatment of choice in patients with SCD as there is no point in preserving a non‐functioning spleen that is present in the majority of patients. CT‐guided aspiration may be employed as a temporary measure for those patients who are at high surgical risk with unilocular abscess. Am. J. Hematol. 58:100–104, 1998.

Congenital pyloric atresia and junctional epidermolysis bullosa: a report of two cases

Abstract The association between epidermolysis bullosa (EB) and congenital pyloric atresia (CPA) is rare, but is known distinct clinical entity with autosomal recessive inheritance. The outcome of such an association was universally fatal. This is a report of two newborns with EB and CPA, associated with additional aplasia cutis congenita in one case. One patient was treated postoperatively with phenytoin and survived. Aspects of the diagnosis, pathogenesis, and management are also discussed.

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 (FRCP) endoscopic retrograde cholangiopancreatography sphincterotomy, and stone extraction followed by LC. FRCP 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.

Focal nodular hyperplasia of the liver with the lumbo-costovertebral syndrome

Congenital lumbar hernia is uncommon in children; only 42 cases have been reported. A newborn girl with congenital superior lumbar hernia associated with lumbo-costovertebral syndrome is described. Associated features include focal nodular hyperplasia of the liver, absent right kidney and hydrocephalus.

Cholecystectomy in children with sickle-cell disease

Cholelithiasis is a common complication of sickle-cell disease (SCD); its frequency is variable, ranging from 4% to 55%. Twenty-two children with SCD (Hb SS) underwent cholecystectomy for cholelithiasis. All were managed with a preoperative transfusion regimen to achieve a hemoglobin concentration of 10–12 g/dl and a hematocrit of 30%–40%. On the evening before surgery, each patient was hydrated with IV fluids (5% dextrose in 1/2 or 1/4 normal saline) at 1.5 times the maintenance rate and postoperatively they received IV morphine infusions for analgesia. Intraoperative cholangiograms revealed common bile duct (CBD) stones in 7 patients (30%), only 4 of which were detected preoperatively by ultrasound (US). Two patients also had a splenectomy together with the cholecystectomy, 1 for a splenic abscess and the other for recurrent sequestration crises. There was no mortality, but 2 patients developed acute chest syndrome, 1 developed a minor wound infection, and 1 had postoperative bleeding from the splenic bed. Careful perioperative management permits safe cholecystectomy in children with SCD. Routine intraoperative cholangiography is recommended in these cases, as while 7 of our patients had CBD stones, only 4 of them were detected preoperatively by US.