Stanley Crankson

The immediate and long-term outcomes of newborns with congenital diaphragmatic hernia

In spite of the innovations in the management of newborns with congenital diaphragmatic hernia (CDH) presenting with respiratory distress at birth, mortality and ongoing morbidity still remain high. This is a retrospective analysis of newborns with CDH to determine the immediate and long-term outcomes among survivors. Medical records of newborns with CDH and respiratory distress at birth between January 1993 and March 2002 were reviewed retrospectively. There were 45 newborns, 29 males and 16 females. Eleven newborns (24%) died during the period of preoperative stabilization, 9 from pulmonary hypoplasia and 2 with complex anomalies who were not resuscitated. Surgery was performed in 34 newborns (76%). Three died postoperatively from severe pulmonary hypoplasia and pulmonary hypertension. Eleven newborns (24%) had sepsis from coagulative-negative staphylococci. Thirty-one of 43 newborns (72%) with isolated CDH were discharged home. Twenty-seven of 31 survivors (87%) had adverse long-term outcome and 2 late deaths were from pulmonary complications. Twenty-nine of 43 newborns (67%) with isolated CDH survived. The principal determinant of survival was pulmonary hypoplasia. Eighty-seven percent of survivors have associated morbidity including ongoing pulmonary, nutritional and neuro-developmental problems. Nevertheless preoperative stabilization and delayed surgery have been a satisfactory mode of management.

Non-Hodgkin lymphoma manifesting as thyroid nodules and cardiac involvement.

To the Editor: A 6-year-old boy presented with a month-long history of progressive painless thyroid enlargement, anorexia, weight loss, and shortness of breath. Physical examination revealed elevated jugular venous pressure, clear lungs, normal heart sounds, multinodular thyroid enlargement, and a mass in the left side of the abdomen. There was no hepatosplenomegaly or peripheral lymphadenopathy. Complete blood count and blood chemistry findings were normal except for elevated values for lactic dehydrogenase (4,190 u/L; normal 240– 615 u/L) and uric acid (422 umol/L; normal 120–320 umol/L). A chest radiograph revealed cardiomegaly with clear lung fields. Two-dimensional echocardiography showed a mass in the right atrium, one in the right ventricle that caused obstruction to right ventricular outflow. Computed tomography scans of the neck, chest, abdomen, and pelvis were performed. The thyroid gland appeared to be replaced by a large, lobulated, hypodense mass (Fig. 1). There was a hypodense irregular mass occupying the whole of the right atrium and right ventricle and extending into the lower part of the pericardium. There was no lymphadenopathy noted in the mediastinum (Fig. 2). There were multiple masses in the abdomen. Spleen, liver, and pelvis appeared normal. A diagnosis of Burkitt lymphoma was made on fineneedle aspiration of the thyroid nodules and a Tru-Cut biopsy of the left suprarenal mass. The cerebrospinal fluid was positive for malignant cells. Bone marrow biopsy was deferred because of the patient’s rapidly worsening clinical condition. The patient was determined to have stage IV Burkitt lymphoma and received induction consisting of intravenous vincristine, doxorubicin, and cyclophosphamide (VAdriaC), high-dose cytarabine (HD Ara-C), and intermediatedose methotrexate (ID MTX), along with intrathecal methotrexate, hydrocortisone, and cytarabine (IT MTX/HC/AraC). During therapy the patient was treated for congestive heart failure, tumor lysis syndrome, superior vena cava syndrome, seizures, and Pseudomonas and Staphylococcus epidermidis sepsis. Intensification chemotherapy consisted of intravenous etoposide and ifosfamide. By the end of intensification reevaluation revealed complete disappearance of the thyroid nodules and the cardiac tumor. However, the patient had

Rhabdoid tumour of the kidney with caval and cardiac extension

Rhabdoid tumour of the kidney is a rare paediatric neoplasm usually associated with a grave prognosis. We report a case of left renal rhabdoid tumour with involvement of the inferior vena cava (IVC) and right atrium in a 13-year-old girl. Left nephrectomy together with removal of the IVC and atrial extension was undertaken on cardiopulmonary bypass and hypothermia. She remained disease-free and in good health for 1 year, however, recent review has shown recurrence in the abdominal lymph nodes.

Management of inguinal hernia in premature infants: 10-year experience

Aim: Debatable issues in the management of inguinal hernia in premature infants remain unresolved. This study reviews our experience in the management of inguinal hernia in premature infants. Materials and Methods: Retrospective chart review of premature infants with inguinal hernia from 1999 to 2009. Infants were grouped into 2: Group 1 had repair (HR) just before discharge from the neonatal intensive care unit (NICU) and Group 2 after discharge. Results: Eighty four premature infants were identified. None of 23 infants in Group 1 developed incarcerated hernia while waiting for repair. Of the 61 infants in Group 2, 47 (77%) underwent day surgery repair and 14 were admitted for repair. At repair mean postconceptional age (PCA) in Group1 was 39.5 ± 3.05 weeks. Mean PCA in Group 2 was 66.5 ± 42.73 weeks for day surgery infants and 47.03 ± 8.87 weeks for admitted infants. None of the 84 infants had an episode of postoperative apnea. Five (5.9%) infants presented subsequently with metachronous contralateral hernia and the same number of infants had hernia recurrence. Conclusions: Delaying HR in premature infants until ready for discharge from the NICU allows for repair closer to term without increasing the risk of incarceration. Because of low occurrence of metachronous hernia contralateral inguinal exploration is not justified. Day surgery HR can be performed in former premature infant if PCA is >47 weeks without increasing postoperative complications.

Management of blunt hepatic and splenic trauma in children.

Trauma is a major cause of morbidity and mortality in children. With blunt abdominal trauma, the most commonly injured organs are the liver and the spleen.1 The management of blunt hepatic and splenic injuries has evolved over the past three decades from an emergency operation to a nonoperative approach. The nonoperative management of splenic injuries in children became acceptable because of the significant incidence and seriousness of postsplenectomy sepsis, and the frequent complications associated with non-therapeutic laparotomies.2 Subsequently, nonoperative management of hepatic injuries showed similar success to the results of splenic injuries.3,4 Thus, in the hemodynamically stable child, nonoperative management of hepatic and/or splenic injuries has become the current standard of care. However, in the hemodynamically unstable child or the child with signs or symptoms of peritonitis, an immediate operation is necessary. This report is a retrospective review of children with blunt hepatic and/ or splenic injuries managed at King Fahad National Guard Hospital, Riyadh, Saudi Arabia.

Cryopreserved recombinant tissue plasminogen activator for the restoration of occluded central venous access devices in pediatric oncology patients.

BACKGROUND Thrombolytic therapy with urokinase 5000 units has been the standard therapy for the restoration of thrombosed central catheters. However, with the decreased availability of urokinase, alternatives needed to be sought. The aim of the study was to determine the efficacy, bioactivity, dwell time and cost of cryopreserved recombinant tissue plasminogen activator (rTPA) in the restoration of occluded central venous access devices. MATERIALS AND METHODS For children < 10 kg, a dose of 0.5 mg, and for children >10 kg, a dose of 1mg was used. The dwell time was 1-2 hours. RESULTS Of the 40 courses of rTPA, 39 fully restored central venous line patency (97%). Successful courses were instilled for an average of 1 hour. CONCLUSION Cryopreserved rTPA appears to be safe and effective in the dose used to restore the patency of occluded central venous access devices in pediatric oncology patients.

A minimally invasive option for the treatment of persistent postoperative esophageal leak

Persistent postoperative leaks in esophageal surgery in children are highly morbid. They usually require technically difficult major reoperations. A less invasive approach is always desirable. During the period 2003 to 2007, we used a minimally invasive approach for the treatment of persistent postoperative esophageal leaks in two children by injection of fibrin glue into the leak site through a small catheter that was introduced through the chest tube. The procedure was successful in one case. We introduce this approach as an alternative to surgery. We found it to be a safe and viable option for the treatment of persistent postoperative esophageal leaks.

Extrinsic duodenal obstruction from anomalous congenital band.

Case Report A nine-day-old male infant was transferred from a local hospital to our institution with persistent bilious vomiting since birth. He had been delivered full term per vaginam to a 28-year-old gravida 5, para 4, and weighed 3280 g. During the pregnancy, maternal ultrasound scan revealed polyhydramnios. At presentation, clinical examination was unremarkable. A plain abdominal radiograph was normal. An upper gastrointestinal (GI) study showed a mild dilatation of the proximal duodenum, a hold-up at the third part of the duodenum, with passage of contrast into the proximal jejunum. The ligament of Treiz was in the normal position (Figures 1A-1D). The patient was kept on conservative management of intravenous fluids and nasogastric tube decompression. He underwent laparotomy, which revealed an ACB arising from the antimesenteric wall of the third and fourth parts of duodenum as far as the ligament of Treiz, and extending across the proximal jejunum to the root of the mesentery (Figure 2). The band which contained tiny vessels was lysed. At follow-up 10 months later, the infant was asymptomatic and thriving.