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Biomedical subjects

Ahmed H Al-Salem

Publications and source records attributed to Ahmed H Al-Salem.

At least 19 recordsLinked to original sources

Diagnostic and therapeutic ERCP in the pediatric age group.

The role and value of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group is not well established, because pancreatic and biliary diseases are less common in children. This however is not the case in areas like the Eastern Province of Saudi Arabia where sickle cell disease (SCD) and other hemoglobinopathies are common, with increased frequency of cholelithiasis and choledocholithiasis. The purpose of this study was to evaluate the indications, findings, safety and therapies of ERCP in children. One hundred and twenty five children had diagnostic and/or therapeutic ERCP as part of their management at our hospital. Their medical records were reviewed for: age at diagnosis, sex, Hb electrophoresis, indication for ERCP, findings, therapy and complications. There were 77 males and 48 females. Their age at presentation ranged from 5-18 year (mean 13.25 year). The majority of them had sickle cell disease (77.6%). The indications for ERCP were: obstructive jaundice (67.2%), recurrent biliary colic with or without jaundice (10.4%), acute and chronic pancreatitis (7.2%), postoperative bile leak (2.4%), cholangitis with obstructive jaundice (2.4%), hepatitis of unknown etiology (3.2%), cirrhosis of unknown etiology (4%), thalassemia with jaundice (0.8%), hemobilia (0.8%), acute cholecystitis with jaundice (0.8%), and sickle cell disease with ulcerative colitis and obstructive jaundice (0.8%). In six children, ERCP was done following laparoscopic cholecystectomy. ERCP was carried out under sedation in 91 (72.8%) children and under general anesthesia in 34. It was successful in 121 (96.8%) children while cannulation of the Ampulla failed in four. ERCP was normal in 43 children, but eight of them showed evidence of recent stone passage and in six, there were gallstones. In the remaining children, ERCP revealed: normal CBD with stones (18 patients), dilated CBD with stones (17 patients), dilated CBD without stones (19 patients), dilated biliary tree with stones (10 patients), dilated biliary tree without stones (six patients), bile leak (two patients), dilated biliary tree with stones and choledocho-duodenal fistula (one patient), choledochal cyst (two patients), septate gallbladder (one patient), normal ERCP with multiple pancreatic cysts (one patient) and biliary stricture (one patient). The following procedures were carried out: 35 had endoscopic sphincterotomy and stone extraction, 20 had endoscopic sphincterotomy, four had CBD stenting, one underwent removal of a stent, two had insertion of a nasobiliary tube and one had biliary endoprosethesis. There was no mortality. One had bleeding from the site of sphincterotomy which stopped after adrenaline injection. Four patients (3.2%) developed transient mild pancreatitis which settled conservatively. ERCP in the pediatric age group is safe both as a diagnostic and therapeutic procedure. ERCP can provide valuable information which aid in the diagnosis of biliary and pancreatic diseases in children as well as therapy with the technical feasibility of endoscopic sphincterotomy. This is specially so in the era of laparoscopic cholecystectomy, where ERCP should be the treatment of choice in children with CBD stones who are going or have previously undergone laparoscopic cholecystectomy.

Adolescent↗

Successful separation of pygopagus conjoined twins and primary skin closure using V-shaped flaps.

Pygopagus conjoined twins were born by caesarean section at 34 weeks of gestation. Initial evaluation revealed no other abnormalities apart from their attachment at lower back, buttocks and perineum. They had two separate anal and urogenital openings. Their investigations included roentgenograms, ultrasound, barium enema, cystourethrogram, CT scan and MRI. There were two separate spinal columns fused distally at the lower sacral level. Lower gastrointestinal studies showed two separate rectums and cystourethrogram showed normal and separate urinary tracts. MRI showed a single dural sac between two fused sacral bones with no other vertebral anomalies. They were successfully separated with primary skin closure using V-shaped flaps.

Dermatologic Surgical Procedures↗

Successful separation of pygopagus conjoined twins.

Conjoined pygopagus twins are very rare. They represent about 17% of all conjoined twins and commonly share the gluteal region, terminal spine, and lower gastrointestinal, urological, and reproductive tracts. Meticulous preoperative investigations and planning as well as a multispecialty team approach contribute to the success of surgical separation. We report a case of successful surgical separation of pygopagus conjoined twins.

Anal Canal↗

Indications and complications of splenectomy for children with sickle cell disease.

BACKGROUND: Sickle cell anemia (SCA), which is characterized by high hemoglobin (Hb) F level and persistent splenomegaly into the older age group (up to 18 years of age) or even adults, is one of the commonest hemoglobinopathies in the Eastern Province of Saudi Arabia. This makes them liable to develop splenic complications requiring splenectomy. This is a review of our experience in the management of 134 children with SCA who had splenectomy as part of their management at our hospital, with emphasis given to the indications and complications of splenectomy. PATIENTS AND METHODS: The medical records of all children who had splenectomy at our hospital were retrospectively reviewed for the following: age at splenectomy, sex, Hb electrophoresis, indication for splenectomy, preoperative investigations, type of surgery, spleen weight, histology, perioperative management, and postoperative complications. RESULTS: From 1990 to 2004, 170 children with various hematologic disorders had splenectomy at our hospital. Of these, 134 had SCA (118 had sickle cell disease and 16 had sickle-beta-thalassemia). Recurrent acute splenic sequestration crisis (ASSC) was the commonest indication for splenectomy in 103 (76.9%) patients, followed by hypersplenism in 18 (13.4%). Seven (5.2%) of our patients had splenectomy for splenic abscess (SA) and 2 had splenectomy for massive splenic infarction; 103 (61 boys, 42 girls) patients with a mean age of 7.6 years (range, 1.8-13 years) had splenectomy for ASSC. Their mean Hb F level was 20.5% (range, 9.2%-39.6%). Thirty-two of them had major attacks. Their Hb levels at the time of admission ranged from 1.4 to 4.1 g/dL (mean, 2.5 g/dL). The remaining 71 had minor recurrent attacks. Eighteen had splenectomy for hypersplenism and all had a significant increase in their blood parameters after splenectomy. Seven had splenectomy for SA. In 5 patients, Salmonella was the causative organism; in 1, it was Enterobacter sakazaki, whereas in 1, no organisms were identified. Two of our patients had splenectomy for massive splenic infarction because of persistent left upper quadrant abdominal pain, and 1 had splenectomy for splenomegaly with a nonfunctioning spleen. Twenty-eight (21%) of our patients had splenectomy and cholecystectomy. In 4 patients, this was because of symptomatic gallstones, whereas in the other 24, it was because of asymptomatic gallstones discovered on ultrasound. There was no mortality, but 8 (6%) developed postoperative complications. CONCLUSIONS: With good perioperative management, splenectomy in children with SCA is not only safe, but also beneficial in treating SA, reducing the patients' transfusion requirements, eliminating the risks of ASSC, and eliminating the discomfort and mechanical pressure of the enlarged spleen. Abdominal ultrasound should be done routinely preoperatively for all children with SCA undergoing splenectomy, and if gallstones are discovered, they should undergo concomitant cholecystectomy. This is even so for asymptomatic gallstones. The addition of cholecystectomy to splenectomy does not increase the morbidity, but eliminates the subsequent complications of gallstones and simplifies their future management in case of abdominal crisis as the possibility of cholecystitis is eliminated.

Adolescent↗

Predictors of gangrenous necrotizing enterocolitis and extent of disease. Early laparotomy versus peritoneal drainage.

OBJECTIVE: Evaluation of known predictors of gangrene in neonates with necrotizing enterocolitis (NEC) and identification of those suggestive of severe disease, requiring expeditious laparotomy rather than primary peritoneal drainage as a definitive treatment. METHODS: This is a retrospective review of data collected from the medical records of newborns with confirmed NEC, treated at the Maternity and Children's Hospital, Dammam, Kingdom of Saudi Arabia, from May 1993 to May 2004. Fifty-five cases were selected for the study, 23 had successful medical management and 32 underwent laparotomy. Of this group, 15 had peritoneal drainage prior to laparotomy. Nine known clinical, radiological and laboratory features suspicious of bowel perforation or gangrene were evaluated. The operated group was classified according to the extent of disease into isolated, multifocal or pan intestinal and the distribution of these 9 criteria was calculated for each of the 3 groups. Comparison was then carried out between the group with isolated NEC and those with extensive disease. RESULTS: Isolated NEC was present in 8 (25%), multifocal NEC in 19 (59%) and pan intestinal NEC in 5 (16%) of the operated cases. Pneumoperitoneum and palpable abdominal mass were the most specific and predictive signs of perforated or gangrenous bowel in NEC. Severe pneumatosis intestinalis and gasless abdomen were also highly specific and predictive of the same but had a low prevalence. Abdominal wall erythema, persistent metabolic acidosis, portal vein air, gasless abdomen and severe pneumatosis intestinalis were found to be associated with severe or extensive gangrene. Palpable abdominal mass and fixed dilated loops were increased in cases of isolated NEC. Portal vein air was associated with the highest mortality. CONCLUSION: Pneumoperitoneum, though the only absolute evidence of bowel perforation, cannot predict the extent of disease. Peritoneal drainage is a useful stabilizing procedure but the presence of any of the above mentioned criteria which are associated with severe disease necessitate a quick decision in favor of laparotomy. The absence of these signs, however, cannot rule out extensive or progressive NEC and failure to improve after peritoneal drainage also requires an emergency laparotomy, regardless of birth weight or gestational age.

Abdomen↗

An unusual cause of pelvi-ureteric junction obstruction.

Congenital lumbar hernia is rare in the pediatric age group. This report describes an infant with a superior lumbar hernia with herniation of the kidney that resulted in pelvi-ureteric junction obstruction. Surgical repair of the hernia and reduction of the herniated kidney resulted in resolution of the pelvi-ureteric junction obstruction. The literature on the subject is also reviewed.

Humans↗

Choledochal cyst.

Choledochal cyst, which is characterized by dilatation of the biliary ducts, is common in Asian countries, mainly Japan, but relatively rare worldwide. This report describes 2 Saudi female children with choledochal cysts, with emphasis on long term follow-up.

Child, Preschool↗

Lymphangiomas in infancy and childhood.

OBJECTIVE: Lymphangiomas are rare congenital malformations, commonly seen in the head and neck. This is a review of our experience in the management of 22 children with lymphangiomas. METHODS: The medical records of children with lymphangioma admitted to Qatif Central Hospital, Qatif, Kingdom of Saudi Arabia over a period of 10 years from August 1989 to July 2000 were retrospectively reviewed for age at diagnosis, gender, mode of presentation, site of lymphangioma, method of treatment and outcome. RESULTS: We treated 22 children (12 females and 10 males) with lymphangioma. Their ages ranged from birth to 12 years, but majority (73%) were 4 years of age or younger. In 10 (45.5%), the lymphangioma involved the neck, 5 of them presented with sudden neck swelling as a result of hemorrhage into a lymphangioma, which caused diagnostic confusion. One patient had extensive lymphangioma involving the floor of the mouth, tongue, and left parotid gland. The remaining 11 patients had lymphangioma involving the parotid gland in 2, floor of the mouth in 3, and one each in the abdominal wall, above the right knee, mediastinum, breast, scrotum, and mesentery. All were treated surgically except 3 who were treated with intralesional bleomycin and showed complete disappearance of their lesions. There was recurrence in the child with mediastinal lymphangioma and a small recurrence in the child with bilateral lesions in the floor of the mouth. CONCLUSION: Lymphangiomas are relatively rare, involving mainly the head and neck, but they can be rarely seen at other sites. An important observation is the sudden appearance of cervical lymphangioma as a result of hemorrhage, which should be kept in mind. Our experience in the treatment of lymphangiomas using bleomycin is limited to draw any conclusions. We therefore considered surgery as treatment of choice for lymphangiomas. However, sclerotherapy can be used when there is a risk of damaging surrounding structures, and also to obviate the poor cosmetic results.

Child↗

Should cholecystectomy be performed concomitantly with splenectomy in children with sickle-cell disease?

Splenectomy and cholecystectomy are among the common surgical procedures required to treat complications of sickle-cell disease (SCD), and when performed separately have been shown to be safe and effective. To determine whether cholecystectomy be performed concomitantly with splenectomy (CSC) in these children, we studied a total of 130 children who underwent splenectomy for various hematologic diseases at our hospital. The most common indication was SCD. Ninety-nine patients (86 SCD and 13 sickle-B-thalassemia) underwent splenectomy and 19 (19.2%) (12 males and 7 females, mean age 13.4 years [7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18]) underwent CSC due to concomitant gallstones, which were asymptomatic in 13 cases. Those with an admission hemoglobin (Hb) of less than 10 g/dl were transfused with packed erythrocytes to increase their Hb to 10-12 g/dl and their hematocrit to 30%-40%. All patients received intravenous hydration the night before surgery at a rate of 1(1/2) times their maintenance rate, which was continued postoperatively until they were able to tolerate adequate oral fluid intake. The indications for splenectomy in those who had CSC were: acute splenic sequestration crisis in 12, splenic abscess in 3, hypersplenism in 2, and massive splenic infarction in 2. Eight patients had massive splenomegaly (spleen weight >1 kg). In 7 cases CSC was done through a left-upper-quadrant (LUQ) transverse incision, in the remaining 12 through an upper midline incision. There was no mortality and only 2 patients developed postoperative complications; a wound infection in 1 and a hematoma in the splenic bed in another. With good perioperative management, CSC is both safe and effective for children with SCD. Both procedures can be performed safely through an upper midline or a LUQ transverse incision, even in the presence of massive splenomegaly.

Adolescent↗

Acute appendicitis in patients with sickle cell disease.

OBJECTIVE: Patients with sickle cell disease (SCD) often present with abdominal pain, usually attributed to vaso-occlusive crisis, but not rarely, it may be caused by other surgical conditions. Acute appendicitis although common in patients with SCD, it is rare and has a rapid course with a high incidence of perforation. METHODS: Over a period of 7 years from 1995 to 2001, only 8 patients with SCD out of 1563 (0.5%) patients with acute appendicitis underwent operation at Qatif Central Hospital, Qatif, Kingdom of Saudi Arabia. Their histological slides were reviewed and the findings were compared to those with sickle cell trait (9 patients) and control group (28 patients). RESULTS: All patients with SCD and in spite of a short duration of symptoms had a moderate to severe inflammation and the vessels were packed with sickle red blood cells (RBCs) except one who had an intact mucosa, extensive transmural hemorrhage and congested blood vessels with sickled RBCs without inflammatory cell infiltrate. The mucosa was intact in only one patient with SCD when compared to 5 (55.6%) in those with sickle cell trait and 6 (21.4%) in the control group and in the majority (87.5%) of those with SCD there were moderate to severe mucosal ulcerations when compared to those with sickle cell trait (44.4%) or controls (64.3%). CONCLUSION: In patients with SCD, acute appendicitis is rare, and these appendicular changes were a sequelae of blockage of appendiceal vessels by sickled RBCs leading to congestion, edema, and ischemia with subsequent mucosal ulceration and marked inflammatory cell infiltrate.

Abdominal Pain↗

Herniation through the foramen of Morgagni: early diagnosis and treatment.

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%) Down's 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.

Child↗

Delayed presentation of bilateral Morgagni's hernia in a child with Down's Syndrome.

This case report describes the delayed presentation of bilateral Morgagni's hernia in a 13-month-old girl with Down's Syndrome. The report emphasizes the fact that a previously normal chest x-ray should not preclude the diagnosis of Morgagni's hernia even when bilateral. The various presentations and the association between Morgagni's hernia and Down's Syndrome are also discussed.

Barium Sulfate↗

Congenital lobar emphysema.

Congenital lobar emphysema is a very rare congenital cystic malformation of the lung that can cause acute respiratory distress in early life. This paper reviews 6 cases of congenital lobar emphysema seen over a period of 10 years. The medical records of children with the diagnosis of congenital lobar emphysema were retrospectively reviewed for age at diagnosis, sex, presenting symptoms, investigations, treatment and outcome. There were 4 males and 2 females, and all of them presented before 6 months of age. Three presented with recurrent chest infection, while the other 3 had acute respiratory distress soon after birth. In all, the diagnosis was confirmed by chest x-ray, and the left upper lobe was affected in all of them. Although congenital lobar emphysema is rare, clinical awareness of this condition is important for early diagnosis and effective surgical treatment.

Female↗

Kikuchi[corrected]-Fujimoto [corrected] disease.

OBJECTIVE: Kukuchi-Fujimito disease is a rare, benign and self limiting condition, which usually presents with lymphadenopathy or fever of an unknown etiology, or both. Its rarity, as well as the similarity of its clinical features to other more common conditions, contribute to overlooking it in the differential diagnosis of patients presenting with lymphadenopathy or fever of an unknown etiology. METHODS: The study was carried out at Qatif Central Hospital, Qatif, Kingdom of Saudi Arabia. All lymph node excisional biopsies received in the histopathology laboratory between 1989 and 1999 were evaluated and those diagnosed as Kukuchi-Fujimito disease were reviewed for clinical data and histological findings. RESULTS: A total of 6 cases were diagnosed as Kukuchi-Fujimito disease out of 390 lymph node biopsies. All patients were young with an average age of 21.5 years and equal sex distribution. Enlarged cervical lymph nodes with or without fever were the most familiar presenting symptoms. Anemia and leukopenia were observed in 3 patients. There was no recurrence of the lymphadenopathy over a period of 1-12 years follow up. CONCLUSION: Kukuchi-Fujimito disease although rare should be included in the differential diagnosis of patients presenting with lymphadenopathy as well as fever of an unknown origin. To obviate unnecessary investigations and therapeutic trials, these patients should undergo early lymph node biopsy which must be interpreted by an experienced pathologist.

Adolescent↗