Maternity and Children Hospital

Medina, Saudi Arabia

Maternity and Children Hospital

Medina, Saudi Arabia
SEARCH FILTERS
Time filter
Source Type

Habeb A.M.,Maternity and Children Hospital
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia | Year: 2012

Hereditary hypomagnesemia with secondary hypocalcemia (HSH) is a rare condition caused by mutations in the transient receptor potential melastatin 6 (TRPM6) gene. Patients usually present during early infancy with symptomatic hypocalcemia; however, intracranial calcification has not been previously reported in HSH. We report on a three-month-old Saudi girl who presented with hypocalcemic convulsions and was initially treated as nutritional rickets. However, further biochemical analysis of blood and urine were suggestive of HSH. This diagnosis was confirmed by mutation analysis, which identified a novel homozygous frame shift mutation (ins 2999T) of the TRPM6 gene. A computed tomography brain scan, done around the time of diagnosis, identified bilateral basal ganglia calcification (BGC). Her serum calcium and the BGC improved with magnesium replacement. BGC can be added as a new feature of HSH and the case highlights the importance of measuring serum Mg in patients with hypocalcemic convulsions, particularly in children of consanguineous parents.


Al-Salem A.H.,Maternity and Children Hospital
Pediatric Surgery International | Year: 2013

Background: Massive splenic infarction (MSI) is a very rare condition. Few reports of splenic infarction of various etiologies including hematological and non-hematological causes have been published. On the other hand, MSI in patients with sickle cell anemia (SCA) is extremely rare. This report describes our experience with 15 children with SCA and MSI outlining aspects of presentation, diagnosis and management. Patients and methods: The records of all children with MSI were retrospectively reviewed for age at diagnosis, sex, clinical features, precipitating factors, investigations, management and outcome. Results: 15 children (11 M: 4 F) with SCA were treated for MSI. Their mean age was 10.9 years (6-17 years). All presented with severe left upper quadrant abdominal pain. In nine, this was associated with nausea and vomiting. Three were febrile and all had a tender splenomegaly. Their mean hemoglobin was 8.2 g/dl (5.7-11.3 g/dl), mean WBC was 10.97 × 103 mm-3 (3.6 × 103-22.3 × 103 mm-3) and mean platelet count was 263.3 × 103 mm-3 (40 × 10 3-660 × 103 mm-3). In seven, there was a precipitating cause including high altitude in two, acute chest syndrome in two, septicemia in two and severe vasooclusive crisis in one. Abdominal ultrasound and CT scan confirmed the diagnosis of MSI which involved more than half of the spleen in 12 and whole spleen in 3. All were treated with IV fluids, analgesia and blood transfusion where appropriate. Eleven had splenectomy because of persistent abdominal pain, three developed splenic abscess and underwent splenectomy and one settled on conservative treatment. Histology confirmed the diagnosis of splenic infarction in 11 and infarction with abscess in the remaining 3. Conclusion: MSI is extremely rare in children with SCA. It can develop spontaneously or precipitated by other factors namely high altitude, acute chest syndrome and severe stress. Most reported cases of splenic infarction are small in size, focal and can be treated conservatively. MSI, on the other hand, may necessitate splenectomy for persistent symptoms or in case of complications, such as abscess formation. © 2012 Springer-Verlag Berlin Heidelberg.


Alqahtani A.,King Saud University | Al-Salem A.H.,Maternity and Children Hospital
Surgical Laparoscopy, Endoscopy and Percutaneous Techniques | Year: 2011

Background: Morgagni hernia is rare and represents less than 5% of all congenital diaphragmatic hernias. The defect is repaired either by conventional open surgery or laparoscopically. The aim of this study was to compare the conventional open technique with the laparoscopic-assisted repair of Morgagni hernia. Patients and Methods: The medical records of all infants and children with the diagnosis of Morgagni hernia were retrospectively reviewed and divided into 2 groups according to the method of repair, the laparoscopic-assisted group and the open surgery group. The 2 groups were compared according to the operative time, hospital stay, time to commencement of full feeds, requirement for postoperative analgesia, complications, and outcome. Results: Twenty-three (16 male and 7 female) patients had open surgery repair. Their age ranged from 1 to 60 months (median 9 mo). Nine patients had right-sided, 5 had left-sided hernia, and 9 (39%) had bilateral hernias. Associated anomalies were seen in 18 patients (78.3%). Eight patients (34.8%) had congenital heart disease, 5 (21.7%) had malrotation of bowel, and 6 (26%) had Down syndrome. All were operated transabdominally (14 upper midline and 8 upper transverse) except for 1 patient who had a right thoracotomy. In all patients, there was a hernia sac that was excised and the defect was repaired using nonabsorbable sutures. On follow-up, 2 patients (8.7%) developed recurrence. Sixteen patients (10 boys and 6 girls) had laparoscopic-assisted repair of Morgagni hernia. Their age ranged from 8 to 42 months (median 18.5 mo). Only 1 patient had Down syndrome and congenital heart disease. Seven patients had right-sided hernia, 7 had left-sided hernia, and 2 had bilateral hernia. On follow-up, 1 patient had a small residual sac 6 months postoperatively, without clinical significance. When the 2 groups were compared, the laparoscopic-assisted group was better in terms of shorter operative time, shorter time to commencement of full feeds, less postoperative analgesia, shorter hospital stay, and better cosmetic results. Conclusions: Laparoscopic-assisted repair of Morgagni hernia is an excellent and simple alternative to open surgery. It is, however, superior over conventional open surgery and the benefits are shorter operative time, early commencement of full feeds, less postoperative analgesia, rapid recovery, shorter hospital stay, and better cosmetic results. Laparoscopic-assisted repair of Morgagni hernia should be considered as the procedure of choice for the treatment of Morgagni hernia in infants and children. Leaving the hernia sac plicated in place has no adverse effects. © 2011 by Lippincott Williams & Wilkins.


Al-Salem A.H.,Maternity and Children Hospital
Journal of Pediatric Hematology/Oncology | Year: 2016

Splenomegaly and hypersplenism are common complications among children with thalassemia necessitating splenectomy. Thirty-six children (27 β-thalassemia major, 3 Hb H disease, and 6 thalassemia intermediate) had total splenectomy (11 laparoscopic and 13 open splenectomy) or partial splenectomy (12 patients). In the partial splenectomy group, 2 with Hb H required no transfusions. For those with β-thalassemia major who had partial splenectomy (9 patients), there was a reduction in the number of transfusions from a preoperative mean of 15.2 transfusions per year to a postoperative mean of 8.2 transfusions per year. Subsequently and as a result of increase in the size of splenic remnant, their transfusions increased, but none required total splenectomy. Twenty-four patients had total splenectomy (13 open and 11 laparoscopic splenectomy). Their postsplenectomy transfusions decreased from a preoperative mean of 17.8 transfusions per year to a postoperative mean of 10 transfusions per year. There was no mortality, and none developed postoperative sepsis or thrombotic complications. Total splenectomy is beneficial for children with β-thalassemia major and hypersplenism by reducing their transfusion requirements. Laparoscopic splenectomy is however more beneficial. Partial splenectomy reduces their transfusion requirements, but only as a temporary measure, and so it is recommended for children younger than 5 years of age. © Copyright 2015 Wolters Kluwer Health, Inc. All rights reserved.


Al-Salem A.H.,Maternity and Children Hospital
World Journal of Pediatrics | Year: 2010

Background: Congenital Morgagni-Larrey's hernia (CMLH) is rare and known to be associated with a high incidence of bilaterality and associated anomalies. This study aimed to review our patients with bilateral CMLH and evaluate their presentation, associated anomalies, diagnostic difficulties and therapy. Methods: From January 1989 to December 2007, we treated 8 children with bilateral CMLH at our hospital. Their medical records were retrospectively reviewed for age, sex, symptoms, associated anomalies, diagnosis, operative findings, treatment and outcome. Results: Among the 8 children, 4 were male and 4 female, with a mean age of 22.74 months (range: 0.93-108 months). Six of them sustained repeated attacks of pneumonia. In 5 children, bilaterality was diagnosed at surgery. Associated anomalies were observed in all children, including congenital heart disease in 5, Down's syndrome in 4, malrotation in 3, inguinal hernia in 2, umbilical hernia in 1, and pyloric stenosis in 1. All of these children were operated on transabdominally. All of them did well postoperatively and on follow-up ranging from 1.5 years to 5 years (mean: 3 years), one had recurrence of the hernia as well as an incisional hernia. Conclusions: The diagnosis of bilateral CMLH may be difficult preoperatively, especially if one of the hernial sacs is empty. CT scan is valuable to diagnose bilateral hernias. To repair these hernias, we advocate a transabdominal approach, which allows easy reduction and inspection of contents, access and repair of bilateral hernias, and correction of associated malrotation if present. © 2010 World J Pediatr. All rights reserved.


Issa H.,King Fahad Specialist Hospital | Al-Salem A.H.,Maternity and Children Hospital
World Journal of Gastroenterology | Year: 2011

AIM: To evaluate the role of endoscopic retrograde cholangiopancreatography (ERCP) for choledocholithia-sis in patients with sickle cell anemia (SCA) in the era of laparoscopic cholecystectomy (LC). METHODS: Two hundred and twenty four patients (144 male, 80 female; mean age, 22.4 years; range, 5-70 years) with SCA underwent ERCP as part of their evaluation for cholestatic jaundice (CJ). The indications for ERCP were: CJ only in 97, CJ and dilated bile ducts on ultrasound in 103, and CJ and common bile duct (CBD) stones on ultrasound in 42. RESULTS: In total, CBD stones were found in 88 (39.3%) patients and there was evidence of recent stone passage in 16. Fifteen were post-LC patients. These had endoscopic sphincterotomy and stone extraction. The remaining 73 had endoscopic sphincterotomy and stone extraction followed by LC without an intraopera-tive cholangiogram. CONCLUSION: In patients with SCA and cholelithiasis, ERCP is valuable whether preoperative or postoperative, and in none was there a need to perform intraoperative cholangiography. Sequential endoscopic sphincterotomy and stone extraction followed by LC is benefcial in these patients. Endoscopic sphincterotomy may also prove to be useful in these patients as it may prevent the future development of biliary sludge and bile duct stones. © 2011 Baishideng. All rights reserved.


Habeb A.M.,Maternity and Children Hospital
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia | Year: 2014

Neonatal adrenal hematoma is a rare finding that can be discovered incidentally or presents with various symptoms. However, urinary tract infection (UTI) has not been reported in association with this condition. We report on a 4-week old child with massive unilateral adrenal hematoma discovered incidentally during a routine abdominal ultrasound scan for UTI. The mass resolved spontaneously after several months with no complications. The diagnosis and management of infantile suprarenal mass and the possible link between this child's UTI and the adrenal hematoma are discussed.


Al-Salem A.H.,Maternity and Children Hospital
Journal of Pediatric Surgery | Year: 2011

Infantile fibrosarcoma is rare and represents less than 1% of all childhood cancers. Commonly, it arises in the limbs followed by the trunk and head and neck. We report a rare case of infantile fibrosarcoma masquerading as sacrococcygeal teratoma in a newborn. The literature on the subject is also reviewed. © 2011 Elsevier Inc. All rights reserved.


Al-Salem A.H.,Maternity and Children Hospital
Pediatric Surgery International | Year: 2012

Background Traumatic diaphragmatic hernia (TDH) is very rare in the pediatric age group. Because of its rarity and its coexistence with more serious injuries, the diagnosis is often missed or delayed. Patients and methods All children with the diagnosis of TDH were retrospectively reviewed for age at diagnosis, sex, mechanism of injury, site of diaphragmatic rupture, associated injuries, injury severity score, investigations, treatment and outcome. Results Over a period of 15 years (1992-2007), we treated seven children with TDH. All were males except one. Their mean age was 7.4 years (3.75-14 years). The site of TDH was on the left in three, on the right in three and one had bilateral TDH. The mechanism of injury was blunt trauma in six and penetrating injury from a stab in one. Three had right TDH and in all three, the diagnosis of TDH was delayed. All sustained severe injuries as reflected by the ISS score (mean 38.6). The time from presentation to diagnosis was variable ranging from 3 to 240 h (mean 65.7 h). The three patients with left TDH were repaired trans-abdominally while the three with right TDH were repaired via a right thoracotomy. The patient with bilateral TDH required laparotomy and thoracotomy. All our patients survived and the hospital stay was variable ranging from 7 to 24 days (mean 17 days). Conclusions TDH is very rare in children and often associated with severe more serious injuries. These two factors contribute to the delay in diagnosis. This is specially so for right TDH. To obviate delay in diagnosis, it is important to have a high index of suspicion as well as careful interpretation of the initial and follow-up radiological investigations including CT-scan of the chest and abdomen. This is important to detect a slowly increasing TDH that may not be apparent initially. © 2012 Springer-Verlag.


Habeb A.M.,Maternity and Children Hospital
Libyan Journal of Medicine | Year: 2013

Background: Wolcott-Rallison syndrome (WRS) is caused by recessive EIF2AK3 gene mutations and characterized by permanent neonatal diabetes (PNDM), skeletal dysplasia, and recurrent hepatitis. The frequency of this rare syndrome is largely unknown. Objectives: To define the frequency and spectrum of WRS in the Kingdom of Saudi Arabia (KSA) based on published data. Methods: The Medline database was searched for published articles on WRS. The number of reported cases from KSA was compared to the total number of WRS cases reported worldwide. The genotype and phenotype of WRS patients from KSA were reviewed. Results: Ten articles describing 23 WRS patients from 12 Saudi families from 1995 to 2012 were identified. This figure accounts for 27.7% (23/83) of the patients and 22.2% (12/54) of the families with WRS reported worldwide until January 2013. All Saudi patients with WRS presented with PNDM, and they represent 59% of all PNDM cases from WRS. At reporting, 73% of patients experienced recurrent hepatitis, 56.5% had skeletal abnormalities, and 39.1% of them were dead. There was a variation in the phenotype even between affected siblings. Genetic diagnosis was confirmed in all 12 families with no correlation between the genotype and phenotype. Eight of the nine EIF2AK3 mutations were only reported in these families, and one was shared with a patient from Qatar, a neighboring Arab state. Conclusions: No study on the frequency of WRS has been published. However, the available data indicate that KSA has the largest collection of patients with WRS worldwide, and nine of the identifiable EIF2AK3 mutations appear to be confined to Arabs. Establishing a national or international registry for WRS would provide more reliable data on this rare condition. © 2013 Abdelhadi M. Habeb.

Loading Maternity and Children Hospital collaborators
Loading Maternity and Children Hospital collaborators