BackgroundPentalogy of Cantrell is a rare syndrome, first described by Cantrell and co-workers in 1958. The syndrome is characterized by the presence of five major congenital defects involving the diaphragm, abdominal wall, the diaphragmatic pericardium, lower sternum and various congenital intra-cardiac abnormalities. The syndrome has never been reported in Tanzania, although may have been reported from other African countries. Survival rate of the complete form of pentalogy of Cantrell is as low as 20%, but recent studies have reported normal growth achieved by 6 years of age where corrective surgeries were done; showing that surgical repair early in life is essential for survival.Case presentationThe African baby residing in Tanzania was referred from a district hospital on the second day of life. She was noted to have a huge omphalocele and ectopia cordis covered by a thin membrane, with bowels visible through the membrane and the cardiac impulse visible just below the epigastrium. Despite the physical anomaly, she appeared to saturate well in room air and had stable vitals. Her chest X-ray revealed the absence of the lower segments of the sternum and echocardiography showed multiple intra-cardiac defects. Based on these findings, the diagnosis of pentalogy of Cantrell was reached. On her fifth day of life, the neonate was noted to have signs of cardiac failure characterized by easy fatigability and restlessness during feeding. Cardiac failure treatment was initiated and she was discharged on parents’ request on the second week of life. Due to inadequate facilities to undertake this complex corrective surgery, arrangements were being made to refer her abroad. In the meantime, her growth and development was satisfactory until the age of 9 months, when she ran out of the medications and succumbed to death. Her parents could no longer afford transport cost to attend the monthly clinic visits, where the infant was getting free medication refill.ConclusionsThe case reported here highlights that in resource limited settings; poor outcome in infants with complex congenital anomalies is a function of multiple factors. However, we believe that surgery would have averted mortality in this 9-month-old female infant. We hope to be able to manage these cases better in future following the recent establishment of cardiac surgery facilities at Muhimbili National Hospital.

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Introduction. Hepatopulmonary fusion is a very rare finding associated with right-sided congenital diaphragmatic hernia. With less than 50 reported cases, management and outcomes of hepatopulmonary fusion are poorly understood. This report highlights that clinical presentation is not a reliable indicator of outcomes in this rare disease. Case Presentation. A term neonate admitted for tachypnea and complete opacification of the right hemithorax was diagnosed with right-sided congenital diaphragmatic hernia. Preoperative respiratory support was minimal, and the only symptom exhibited was tachypnea. During surgical repair, fusion of the lung and liver were noted, consistent with a diagnosis of hepatopulmonary fusion. Postoperatively, the patient’s pulmonary hypertension worsened and required extracorporeal membrane oxygenation. Conclusions. Many patients with hepatopulmonary fusion and only mild symptoms die postoperatively from severe pulmonary hypertension and progressive respiratory failure. Preoperative clinical status is not indicative of postoperative outcomes, and literature suggests that patients who require less support preoperatively have high mortality rates. The availability of ECMO for postoperative complications may be necessary in patients requiring repair of hepatopulmonary fusion.

While relatively common in the skeletal system, cartilaginous tumors are rarely seen originating from the orbit. Here, we report a rare case of an orbital chondroma. A 27-year-old male patient presented with a painless hard mass in the superonasal quadrant (SNQ) of left orbit since 3 months. On examination, best-corrected visual acuity of both eyes was 20/20, with normal anterior and posterior segment with full movements of eyeballs and normal intraocular pressure. Computerized tomography scan revealed well defined soft tissue density lesion in SNQ of left orbit. Patient was operated for anteromedial orbitotomy under general anesthesia. Mass was excised intact and sent for histopathological examination (HPE). HPE report showed lobular aggregates of benign cartilaginous cells with mild atypia suggesting of benign cartilaginous tumor - chondroma. Very few cases of orbital chondroma have been reported in literature so far.