Is splenectomy for massive splenomegaly safe in children?

PURPOSE: To study and analyze the causes, etiology, morbidity, mortality and therapeutic value of splenectomy performed for massive splenomegaly in children. METHODS: The medical records of 115 children less than 18 years old who had splenectomy for various hematological disorders were reviewed. Twenty of them had splenectomy for massive splenomegaly (spleen weight > or =1,000 g). The records of these were reviewed for age at operation, gender, hematological diagnosis, indication for splenectomy, operative procedures, postoperative complications, and outcome. RESULTS: Twenty children had splenectomy for massive splenomegaly. There were 16 males and 4 females. Their ages ranged from 4 to 15 years (mean 11.2). Twelve had sickle cell disease, 5 had sickle-beta-thalassemia, 1 had beta-thalassemia major, 1 had thalassemia intermediate, and 1 had chronic myeloid leukemia. The indications for splenectomy were hypersplenism in 11, recurrent splenic sequestration crisis in 8, and splenic abscess in 1. The transfusion requirements in the patient with beta-thalassemia major decreased markedly postoperatively from 18 transfusions/year to only 4 transfusions/year; and for those with hypersplenism, there was a marked improvement in their blood parameters following splenectomy. The patient with thalassemia intermediate required no more blood transfusions. There was no mortality. The immediate postoperative morbidity was 10% for those with massive splenomegaly compared with 6.3% for those with splenomegaly <1,000 g. CONCLUSIONS: With good perioperative management, splenectomy in children with massive splenomegaly is both safe and effective.

Splenectomy in patients with sickle-cell disease.

BACKGROUND: Splenic complications of sickle-cell disease (SCD) are associated with morbidity, and in some it may lead to mortality. This paper presents our experience with 43 patients with SCD who had splenectomy as part of their management. PATIENTS AND METHODS: The records of 43 patients with SCD who had splenectomy were examined for age at operation, sex, hemoglobin (Hb) electrophoresis, indication for splenectomy, pre- and postoperative medications, operative procedures, and postoperative complications. RESULTS: The indications for splenectomy were acute splenic sequestration crisis (ASSC) in 21 patients, hypersplenism in 15, and splenic abscess in 7. In 17 patients, the spleen was also found to be massively enlarged causing discomfort and intervening with everyday activity. For those with hypersplenism, there was a significant postoperative increase in total Hb (P < 0.0001), hematocrit (P < 0.0001), white blood cells (P < 0.0001), and platelet count (P < 0.0001). CONCLUSIONS: With careful perioperative management and proper follow-up, splenectomy in patients with SCD is beneficial in reducing their transfusion requirements and its attendant risks, eliminating the discomfort from mechanical pressure of the enlarged spleen, avoiding the risks of ASSC, and managing splenic abscess.

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.

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.

Infantile hypertrophic pyloric stenosis and congenital diaphragmatic hernia.

The association of infantile hypertrophic pyloric stenosis with congenital diaphragmatic hernia is rare. We report three cases of infantile hypertrophic pyloric stenosis who had concomitant congenital diaphragmatic hernia.

An unusual case of esophageal atresia and double distal tracheoesophageal fistula.

This report describes a neonate with a very rare and an unusual variety of esophageal atresia and tracheoesophageal fistula. The anomaly consisted of esophageal atresia and double distal tracheoesophageal fistula. The two fistulae as well as part of the distal esophagus were made up of tracheobronchial tissues. The embryology of the anomaly is also discussed.

Concurrent left congenital diaphragmatic hernia and esophageal atresia: case report and review of the literature.

Coexisting congenital diaphragmatic hernia and esophageal atresia is an extremely rare phenomenon. Details of one infant with such a combination is presented, and the literature on the subject is reviewed.

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.

Congenital intrinsic duodenal obstruction: problems in the diagnosis and management.

Nineteen infants with intrinsic duodenal obstruction are analyzed. Atresia was the most common lesion. An exceptionally high rate of associated anomalies (73.7%) were present; Down's syndrome, the single most common anomaly, was seen in 47% of the infants. In seven infants, the diagnosis was delayed and in another three it was made intraoperatively while establishing a gastrostomy for esophageal atresia. Three infants died without operation because of gross prematurity and multiple anomalies. Of the 16 operated on, three died, one due to peritonitis and the other two because of metabolic derangements. Of the various operative procedures used, no significant difference was found in the final outcome of treatment. A schematic approach to the diagnosis and management is proposed.

Intrauterine torsion of a wandering spleen presenting as an abdominal cystic swelling.

Wandering spleen is a rare clinical condition that presents commonly with splenic infarction secondary to torsion. Intrauterine torsion of a wandering spleen, however, is extremely rare. An unusual case of intrauterine torsion of a wandering spleen presenting as an abdominal mass is reported.

Ectopic thymic tissue simulating--a posterior mediastinal mass.

A 4-month-old male child underwent a thoracotomy for a suspected posterior mediastinal mass. Ectopic thymic tissue was found. Ectopic thymic tissue should be included in the differential diagnosis of a posterior mediastinal mass in a child.

The role of partial splenectomy in children with thalassemia.

Partial splenectomy was performed on 12 patients with thalassemia (9 beta-thalassemia major and 3 Hb H disease) to reduce blood transfusion requirements. The indication for partial splenectomy was the presence of splenomegaly and increased blood transfusion requirements (i.e. Hb drop > 0.5 g per week). Their ages ranged from 3 to 10 years (mean 6.9 years). On follow-up, ranging from 1.1-5.5 years (mean 2.6 years), two of the three patients with Hb H disease required no more blood transfusions while the third continued to receive blood transfusions, but at a lower frequency. For those with beta-thalassemia major, the transfusion requirements and Hb drop per week decreased in the majority of patients. This is specially so during the first 1-2 years following partial splenectomy. In all, about 1/3 of the size of the normal spleen was preserved (either upper or lower pole) which was judged functional as there has been no significant infection in any of the patients, no change in IgM level, no Howell-Jolly bodies and visualization on scintigraphy. Partial splenectomy is recommended to start with for those with Hb H disease. For patients with beta-thalassemia major, partial splenectomy is beneficial as a temporary measure and in those children who are less than 5 years of age, as they are at greater risk of post splenectomy sepsis.

Colostomy complications in infants and children.

Seventy-seven colostomies were performed in 74 patients: 35 for high anorectal agenesis, 34 for Hirschsprung's disease, 2 for necrotizing enterocolitis, 2 for small left colon syndrome, and 1 for volvulus neonatorum with perforation. There were 55 boys and 19 girls with a mean age of 0.8 years. The different types of colostomies performed were: transverse loop in 48, sigmoid loop in 21, transverse end in 4, descending end in 2, sigmoid end in 1, and transverse double barrel in 1. Forty-seven patients developed stomal complications (74.6%). Eleven patients died, but only in 2 (2.7%) were the deaths directly related to colostomy formation. Five patients required stomal revision (6.8%). The incidence of complications was neither related to the age nor to the primary indication for the colostomy, but sigmoid colostomy was associated with a lower complication rate compared to transverse colostomy (52% versus 81% 0.02 greater than p greater than 0.01). A sigmoid loop colostomy should be used whenever possible.

Three faces of midgut duplication.

This is an analysis of three unusual cases of gastrointestinal duplications seen over a two year period. They were all males aged eight and 48 hours, and 4 1/2 years. Two had cystic duplications; in one of them it was associated with a partially fixed cecum resulting in a volvulus, while in the second the duplication occurred in association with duodenal atresia and jejunal deletion. The third patient had a tubular malformation involving distal jejunum, whole of the ileum, and right side of the colon as far as its transverse level. It was also associated with duodenal stenosis and rectal atresia. Two cystic duplications had muscular coats separate from the normal bowel permitting complete excision. Clinical presentation and pathological anatomy are briefly considered and aspects of management are discussed.

Congenital Morgagni's hernia in infants and children.

Morgagni's hernia is an uncommon type of diaphragmatic hernia in the pediatric age group. Out of 52 children with different types of congenital diaphragmatic hernia that we have treated, 5 (9.6%) had Morgagni's hernia. There were 2 infants and 3 children including one with Down's syndrome. All suffered from repeated attacks of chest infection, and only after a chest X-ray was the diagnosis of Morgagni's hernia suspected. In 2 cases this appeared as an opacity in the anterior mediastinum adjacent to the pericardium; diagnosis was confirmed by barium enema in one and a CT-scan in the other. The remaining 3 cases showed anterior herniation of bowel loops on chest X-ray which was bilateral in one. This bilaterality was confirmed pre-operatively by CT scan. Associated anomalies were present in all cases, including 2 with malrotation. All patients were treated surgically via a transabdominal approach. Our study shows a relative high frequency of Morgagni's hernia in our patients and, although late-presenting Morgagni hernias are relatively benign, it can cause significant morbidity. This calls for early diagnosis and early referral for surgery. Chest X-ray is to be strongly advocated in children with repeated attacks of chest infection.

Splenectomy for hematological disorders in the Eastern Province of Saudi Arabia.

Splenomegaly is a common clinical disorder in the Kingdom of Saudi Arabia. Its aetiology is variable and includes portal hypertension due to schistosomal periportal hepatic fibrosis, haemoglobinopathies and lymphoproliferative disorders. At King Fahd Hospital of the University, Al-Khobar in the Eastern Province of the Kingdom, splenectomy is performed frequently for various reasons on patients drawn from all the Provinces. This is a report of 104 patients (age range eight months to 65 years) who underwent splenectomy between 1982 and 1987. We reviewed the indications, operative findings, outcome, and complications of the procedure. Patients with hypersplenism formed the largest group (50%) subjected to splenectomy followed by those suffering from the haemoglobinopathies. Chest infection as the most common complication and it mainly affected patients with portal hypertension. Post-operative septicaemia occurred in four cases within six months from the date of splenectomy.

Splenectomy in children with sickle cell disease and thalassemia.

A number of Saudi children (31) with sickle cell disease and thalassemia underwent splenectomy: 12 for frequent blood transfusions, 15 for chronic hypersplenism (most of whom were also the recipients of periodic blood transfusion) and 4 for splenic abscess. The mean age of splenectomy was 8.8 years (8 months-18 years). Eight patients had sickle cell disease, 14 beta-thalassemia and 9 had sickle cell thalassemia. All patients received prophylaxis against pneumococcal infection. There was one postoperative death most probably due to sepsis. Sixteen of those who required frequent preoperative blood transfusions needed no more transfusions, while in 7 the need for transfusions decreased significantly (p less than 0.05). For those with hypersplenism, there was a significant postoperative increase in total hemoglobin (P less than 0.001), RBC (P less than 0.001) and platelet counts (p less than 0.02); and a substantial decrease in reticulocyte counts (p less than 0.05). The common post splenectomy complications were chest infection and a brief episode of pyrexia, but without undue morbidity. The study establishes a definite place for splenectomy in a selected population of children with sickle cell disease and thalassemia.

Gastric volvulus in infancy and childhood.

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