Splenectomy for Children With Thalassemia: Total or Partial Splenectomy, Open or Laparoscopic Splenectomy.

Splenomegaly and hypersplenism are common complications among children with thalassemia necessitating splenectomy. Thirty-six children (27 ß-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 ß-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 ß-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.

Congenital H-type tracheoesophageal fistula: a national multicenter study.

BACKGROUND: Congenital H-type tracheoesophageal fistula (TEF) is very rare and represents <5 % of all congenital tracheoesophageal malformations. This is a national, multicenter review of our experience with isolated H-type TEF outlining clinical presentation, methods of diagnosis, associated anomalies, treatment and outcome PATIENTS AND METHODS: The medical records of all patients with the diagnosis of congenital H-type TEF treated at four pediatric surgery units in Saudi Arabia were retrospectively reviewed for: age at diagnosis, sex, presenting symptoms, associated anomalies, method of diagnosis, treatment and outcome. RESULTS: During the study period (January 1998-December 2013), 435 infants and children with the diagnosis of esophageal atresia with or without TEF were treated. Among these, 23 (5.3 %) had isolated TEF. There were 11 males and 12 females. Their age at presentation ranged from 5 days to 3 years and 7 months but the majority (90 %) were diagnosed during their first year of life. Their clinical presentation included: chocking and coughing during feeds in 12 (52.2 %), recurrent chest infection in 16 (69.6 %) and cyanosis in 10 (43.5 %). One presented with abdominal distension also. The diagnosis was made using esophagogram. In 11 (47.8 %), a single study confirmed the diagnosis, 8 (34.8 %) required two studies while 4 (17.4 %) required three studies. Nineteen (82.6 %) had preoperative bronchoscopy and in 13 (56.5 %), a catheter was used to cannulate the fistula. All were operated through a right cervical incision except one who underwent thoracoscopic ligation and division of the fistula. In one, the fistula was only transfixed and tied without being divided. This patient developed a recurrent fistula. Two patients developed postoperative stridor secondary to recurrent laryngeal nerve palsy. In both of them, there was complete recovery. CONCLUSIONS: H-type TEF is very rare and commonly presents with recurrent chest infection, chocking and coughing during feeds and cyanosis. Physicians caring for these patients should be aware of this and a high index of suspicion is of paramount importance to avoid delay in diagnosis with its associated morbidity. A contrast esophagogram is valuable in confirming the diagnosis. The study however may need to be repeated. Preoperative bronchoscopy is valuable to localize and cannulate the fistula for easier access during surgery. Surgical repair is the treatment of choice and this should be performed through a right cervical incision or thoracotomy for low fistulae. Thoracoscopic ligation and division of a low H-type fistula is an alternative and less invasive approach when compared to thoracotomy.

Massive splenic infarction in children with sickle cell anemia and the role of splenectomy.

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 × 10(3) mm(-3) (3.6 × 10(3)-22.3 × 10(3) mm(-3)) and mean platelet count was 263.3 × 10(3) mm(-3) (40 × 10(3)-660 × 10(3) 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.

Traumatic diaphragmatic hernia in children.

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.

Familial achalasia in children.

Achalasia is rare in the pediatric age group and in most cases it is idiopathic with no family history. Familial achalasia is very rare. This report describes two families with achalasia: in one, six children were affected while in the other a brother and a sister had Allgrove's syndrome (triple-A syndrome consisting of achalasia, adrenal insufficiency, and alacrima). Familial achalasia suggests that it is hereditary and may be transmitted as an autosomal recessive trait. The management of achalasia in children is still controversial. With the recent advances in minimal invasive surgery, laparoscopic Heller's myotomy is the procedure of choice in the management of achalasia in children.

Coexisting congenital diaphragmatic hernia, esophageal atresia, and tracheoesophageal fistula: a case report and review of the literature.

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) and congenital diaphragmatic hernia (CDH) are two relatively common neonatal conditions. The coexistence of the two conditions in the same newborn is extremely rare. This is a case report of a newborn with CDH and concurrent EA and TEF. Aspects of diagnosis and the literature on the subject are also reviewed.

Esophageal atresia with or without tracheoesophageal fistula: success and failure in 94 cases.

BACKGROUND: The management of newborns with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) has evolved considerably over the years. Currently an overall survival of 85% to 90% has been reported from developed countries. In developing countries, several factors contribute to higher mortality rates. We describe our experience with 94 consecutive cases of EA with or without TEF. PATIENTS AND METHODS: We retrospectively studied 94 patients with EA with or without TEF treated at our hospital over a period of 15 years. Medical records were reviewed for age at diagnosis, sex, birth weight, associated anomalies, aspiration pneumonia, method of diagnosis, treatment, postoperative complications and outcome. RESULTS: Ninety-four newborns (55 males and 39 females) with EA/TEF were treated at our hospital. Their mean birth weight was 2.2 kg (700 g to 3800 g). Age at diagnosis ranged from birth to 7 days. At the time of admission 37 (39.4%) had aspiration pneumonia. Associated anomalies were seen in 46 (49%) patients. Thirteen patients had major associated anomalies that contributed to mortality. Postoperative complications were similar to those from developed countries but overall operative mortality (30.8%) was high. CONCLUSIONS: The overall mortality was high but excluding major congenital malformations, sepsis was the most frequent cause of death. Factors contributing to mortality included prematurity, delay in diagnosis with an increased incidence of aspiration pneumonia and a shortage of qualified nurses. To improve overall outcome, factors contributing to sepsis should be evaluated and efforts should be made to overcome them.

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.

Splenectomy for children with thalassemia.

Twenty-three children with thalassemia (18 with beta-thalassemia major, 3 with Hb H disease, and 2 with thalassemia intermediate) had total splenectomy (nine beta-thalassemia major patients and two thalassemia intermediate patients) or partial splenectomy (nine beta-thalassemia patients and three Hb H disease patients) as part of their management at our hospital. There were 10 males and 1 female in the total splenectomy group (mean age, 7.8 years; range, 4.5-12 years), and 4 males and 8 females in the partial splenectomy group (mean age, 6.9 years; range, 3-10 years). In all, the indication for splenectomy was hypersplenism. In the partial splenectomy group, two children with Hb H disease required no further blood transfusions. The transfusion requirements of the third patient with Hb H disease decreased from 15 to 11 transfusions per year (from 1.2 g/week Hb drop preoperatively to 0.7 g/week postoperatively), but subsequently his transfusion requirements increased as a result of an increase in the size of splenic remnant. He underwent total splenectomy 1.5 years post-partial splenectomy. For those with beta-thalassemia major who had partial splenectomy, there was a reduction in the number of blood transfusions from a preoperative mean of 15.2 transfusions per year (range, 11-22 transfusions per year) to a postoperative mean of 8.2 transfusions per year (range, 2-11 transfusions per year). Their Hb drop decreased from a preoperative mean of 1.6 g/week (range, 0.8-3.5 g/week) to a postoperative mean of 0.5 g/week (range, 0.2-0.75 g/week). Subsequently and as a result of increase in the size of splenic remnant, their transfusion requirements increased, but none of them to this point have required total splenectomy. Eleven children had total splenectomy. Their postsplenectomy transfusion requirements decreased from a preoperative mean of 17.8 transfusions per year (range, 12-23 transfusions per year) to a postoperative mean of 10 transfusions per year (range, 8-12 transfusions per year), and their Hb drop decreased from a preoperative mean of 1.8 g/week (range, 0.5-2.3 g/week) to a postoperative mean of 0.45 g/week (range, 0.3-0.65 g/week). In conclusion, total splenectomy is beneficial for children with thalassemia and hypersplenism because it reduces their transfusion requirements. Partial splenectomy may be beneficial for those with Hb H disease. However, for those with beta-thalassemia, partial splenectomy is beneficial in reducing their transfusion requirements only as a temporary measure, and it is recommended for children who are less than 5 years of age.

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.

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.

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.

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.

Iatrogenic perforation of the esophagus.

Iatrogenic perforation of the esophagus occurs rarely, and is most frequently seen in preterm and low birth weight infants. This is a report of 2 cases of iatrogenic perforation of the esophagus, outlining aspects of diagnosis and management.

Principles of blood transfusion in sickle cell anemia.

Sickle cell anemia (SCA) is one of the commonly inherited hemoglobinopathies in the Kingdom of Saudi Arabia. It is characterized by periods of remissions and exacerbations called crises as well as certain pathological phenomenon such as acute chest syndrome, priapism, hepatopathy, and cerebrovascular stroke. Blood transfusion (BT) as therapy and prophylaxis in SCA, although was advocated as early as the 1940's, there are still debates regarding its benefits and risks. This is a review of the value of BT in patients with SCA with emphasis on the risks and benefits as well as guidelines towards safe BT.

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.

Volvulus of the sigmoid colon in a child.

Sigmoid volvulus although a common cause of large bowel obstruction in the elderly, is considered rare in the pediatric age group. We report a case of sigmoid volvulus in a 10-year-old child with mental retardation and myopathy. The various predisposing factors for sigmoid volvulus in children are discussed, and the literature on the subject is also reviewed.

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.

Congenital duodenal diaphragm in eight children.

BACKGROUND: Congenital duodenal obstruction (CDO) is a common and usually easy to diagnose cause of intestinal obstruction in the newborn, except when the cause of the obstruction is a duodenal diaphragm. We describe our experience with eight children who had intrinsic duodenal obstruction secondary to a duodenal diaphragm. METHODS: The medical records of 22 children with the diagnosis of congenital intrinsic duodenal obstruction were reviewed for age at diagnosis, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment and outcome. Operative findings and procedures were obtained from the operative notes. RESULTS: Eight of the 22 children (36.4%) had congenital duodenal diaphragm (CDD). In all children, the diagnosis was made from plain abdominal X-ray, which showed the classic double-bubble appearance, and barium meal, which showed duodenal obstruction. Four patients had associated anomalies, including two with Down's syndrome. Intraoperatively, five patients were found to have duodenal diaphragm with a central hole, while the other three had complete duodenal diaphragms. Postoperatively, all patients did well. Six required total parenteral nutrition. CONCLUSIONS: The 100% survival rate among these children is comparable to that in Western countries, and can be attributed to the lack of major associated abnormalities, good perioperative management, and the availability of total parenteral nutrition.