Spontaneous pneumothorax in early pregnancy: Successful management by thoracoscopy

Discussion Beckwith–Wiedemann syndrome (BWS) is a congenital overgrowth syndrome also known as the EMG syndrome because its most prominent features include exomphalos, macroglossia and gigantism. Beckwith reported the first cases in three children at postmortem (Beckwith, 1963) in 1963 and in 1964 Wiedemann reported three siblings with exomphalos, macroglossia and overgrowth (Wiedemann, 1964). Over the next 30 years 300 cases of Beckwith– Wiedemann (BWS) syndrome have been reported in the literature (Elliott and Maher, 1994). There are numerous clinical features but exomphalos, macroglossia and gigantism are almost universal. Other major findings are posterior helical ear pits, nephromegaly and hemihypertrophy. Less common (minor) features include cardiac malformations, neoplasia, polydactyly and cleft palate. The diagnosis is often made on the finding of three major or two major plus three minor features. Over half of the cases are complicated by omphalocele and as this can be detected, during routine ultrasonography the condition may be suspected antenatally (Elliott and Maher, 1994). In this case the patient was booked for antenatal care at 17 weeks and no structural anomaly was seen. Routine anomaly scanning was not carried out at Altnagelvin Hospital at that time, otherwise it is likely that the omphalocele would have been noticed earlier. The presence of polyhydramnios will often lead to preterm labour or premature rupture of the membranes, as in this case, where gross polyhydramnios caused both to occur spontaneously at 22 weeks. In a series of 76 affected fetuses, all those complicated by polyhydramnios delivered prematurely and there was felt to be no association between fetal overgrowth and preterm labour (Elliott et al., 1994). To date, pathognomonic pathological placental lesions have not been documented for BWS (Lage, 1991, McCowan and Becroft, 1994). Lage (1991) reported on four placentas collected over a 3-year period, which had massive villous hydrops. The pathology showed large fluid filled vesicles dispersed throughout the placentas. In all four placentas a diagnosis of hydatidiform mole was first excluded microscopically. In three of the four cases there was associated fetal omphalocele which had been diagnosed antenatally. One of these fetuses was later confirmed to have BWS and the other two had features suggestive of the syndrome. One of the four pregnancies was terminated for severe pre-eclampsia at 19 weeks and the fetus had features of BWS. McCowan and Becroft (1994) reported the only other two cases in the literature of massive hydrops of the placental stem villi and severe pre-eclampsia associated with fetal BWS. This case may be only the fourth reported case of massive placental hydrops and severe early onset pre-eclampsia associated with BWS. Of particular interest is the fact that in the other three cases the placentas, respectively, weighed 700 g at 19 weeks, 370 g at 22 weeks and 670 g at 31 weeks. In this case the placenta was heavier than any other reported in the literature, weighing 1300 g at 22 weeks gestation and containing a huge number of clear fluid-filled, large vesicles, the biggest measuring 3 cm diameter. Table I, shows the comparisons of the findings in our case with those of the three other reported cases. It has been suggested that the distinctive placental lesion of stem villous hydrops may be specific for Beckwith–Wiedemann syndrome and when present this could predispose to severe, early-onset preeclampsia (McCowan and Becroft, 1994). However, this is only the fourth reported case and many more placentas will need to be examined in order to determine if an association exists. The preeclampsia in these cases almost certainly resulted from the hyperplacentosis as it does in severe rhesus disease, diabetes and hydatidiform mole. Beckwith–Wiedemann syndrome is usually diagnosed at birth or in early childhood. However, where severe, early-onset preeclampsia is diagnosed and placentomegaly is demonstrated on ultrasound, a detailed anomaly scan should be performed with the possibility of Beckwith–Wiedemann syndrome considered in the differential diagnosis.

Spontaneous pneumothorax and febrile neutropenia in pregnancy.

The thoracotomy is the most invasive and most effective method for treatment of spontaneous pneumothorax. This method is indicated not only for recurrent pneumothoraces but also if conservative treatment does not induce expansion of lung. The primary operation is indicated absolutely in only some cases (spontaneous hemopneumothorax, pneumothorax in pregnancy). Today it is possible to operate for relative indications: Surgery has a small rate of complications, mortality and relapse (below 1%). The operation removes the causes of pneumothorax and protect for relapse of the pneumothorax via pleurodesis with mechanical irritation of the pleural surface.

Surgical indications and technique of persistent and recurrent spontaneous pneumothorax

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Surgical indications and technique of persistent and recurrent spontaneous pneumothorax