Obstetric Case Reports 527
(Nohara et al. 2003). Th is case supports the acceptability of trying for vaginal delivery before resorting to caesarean section. Arguably, at caesarean section, the inadvertent cutting of the septum could result in an increased risk of bleeding and diffi culty in repair. Th ere is a lack of strong evidence for either option. Th e circumstances of the pregnancies, opinion of the medical team and importantly the mother ’ s choice would therefore be the most crucial factors in determining which option is chosen.
Declaration of interest: Th e authors report no confl icts of interest. Th e authors alone are responsible for the content and writing of the paper.
References Erez O , Dukler D , Novack L et al . 2007 . Trial of labor and vaginal birth aft er cesar-
ean section in patients with uterine M ü llerian anomalies: a population-based study . American Journal of Obstetrics and Gynecology 196 : 537.e1 – e11 .
Fayad S , Bongain A , Holhfeld P et al . 2003 . Delayed delivery of second twin: a multicentre study of 35 cases . European Journal of Obstetrics and Gynecology and Reproductive Biology 109 : 16 – 20 .
Green QL , Schanck GP , Smith JR 1961 . Normal, living twins in uterus didelphys with 38 day interval between deliveries . American Journal of Obstetrics and Gynecology 82 : 340 – 342 .
Kekkonen R , Nuutila M , Laatikainen T . 1991 . Twin pregnancy with a fetus in each half of a uterus didelphys . Acta Obstetricia et Gynecologica Scandinavica 70 : 373 – 374 .
Neville MC , Morton J . 2001 . Physiology and endocrine changes underlying human lactogenesis II . Journal of Nutrition 131 : 3005S – 3008S .
Nh â n VQ , Huisjes HJ . 1983 . Double uterus with a pregnancy in each half . Obstet-rics and Gynecology 61 : 115 – 117 .
Nohara M , Nakayama M , Masamoto H et al . 2003 . Twin pregnancy in each half of a uterus didelphys with a delivery interval of 66 days . British Journal of Obstetrics and Gynaecology 110 : 331 – 332 .
Petocchi T , Golfi eri G . 1969 . [A rare case of double pregnancy in didelphic uterus with a 3-day interval in delivery] . Minerva Ginecologica 21 : 843 – 848 .
Steer P , Flint C . 1999 . ABC of labour care: physiology and management of normal labour . British Medical Journal: Clinical Research 318 : 793 – 796 .
Taylor E , Gomel V . 2008 . Th e uterus and fertility . Fertility and Sterility 89 : 1 – 16 . Tyagi A , Minocha B , Prateek S . 2001 . Delayed delivery of second twin in uterus
didelphys . International Journal of Gynaecology and Obstetrics 73 : 259 – 260 . Woolf RB . 1965 . Coincident congenital anomalies of the reproductive and uro-
logic tract-didelphys with 58 days between births of viable twins . Revista Cl í nica. Lisbon. Instituto Maternal 16 : 39 – 44 .
Dehydrated hereditary stomatocytosis and recurrent prenatal ascites
H. Poret 1,2,3 , E. G. Simon 1,2,3 , P. Herv é 2 & F. Perrotin 1,2,3
1 UMR INSERM Unit 930, Tours, France, 2 CHRU de Tours, Service de gyn é cologie-obst é trique, Centre Olympe de gouges, CHU Bretonneau and 3 Universit é Fran ç ois Rabelais de Tours, Tours, France
DOI: 10.3109/01443615.2013.781142
Correspondence: E. G. Simon, Service de Gyn é cologie-obst é trique, Centre Olympe de Gouges, CHU Bretonneau, 2 Boulevard Tonnelle, 37044 TOURS Cedex 01, France. E-mail:[email protected]
Case report Dehydrated hereditary stomatocytosis (DHS), also known as ‘ heredi-tary xerocytosis ’ , is a rare form of hereditary haemolytic anaemia with autosomal dominant transmission. Th is genetic disorder is provoked by a red cell membranopathy, leading to a well-compensated haemo-lytic anaemia. Approximately 30 families with DHS were described in the literature (Carella et al. 1998), but the exact prevalence is not yet known (Beaurain et al. 2007). Rare patients with DHS develop prena-tal oedema. It resolves spontaneously during the last weeks of gesta-tion or within the weeks following birth (Grootenboer et al. 2001).
Here, we report a case of DHS associated with recurrent fetal ascites in two successive pregnancies. Th e mother showed haemo-lytic anaemia. Compensated hyperhaemolysis, high reticulocytosis,
macrocytosis, presence of stomatocytes and gradient ektacytometric curves led to the diagnosis of DHS. Fetal ascites in the two pregnan-cies resolved spontaneously.
A 32-year-old woman, gravida 2, para 1 was referred to our Prena-tal Diagnosis Unit for fetal ascites. Her past history mentioned hepa-titis at the age of 23. No aetiology had been found. Antenatal tests for hepatitis B, hepatitis C and human immunodefi ciency viruses, as well as for syphilis and cytomegalic virus were negative. She was immune to rubella. At 18 weeks, the routine scan showed severe ascites and minimal pleural eff usion. No polyhydramnios or placental oedema was present, and all organs appeared normal. Values for peak sys-tolic fl ow velocity in the middle cerebral artery remained within the normal range. An amniocentesis was performed. Th e karyotype was normal. Tests for storage diseases, digestive enzymes and infections were negative. Close ultrasound monitoring was decided. Pleural eff usion disappeared and fetal ascites increased during the 27th week of gestation. Ascites remained stable until the 31 weeks ’ gestation and then resolved slightly. A complete resorption of ascites was noticed at 34 weeks ’ gestation with magnetic resonance imaging. Natural vagi-nal delivery occurred at 38 weeks ’ gestation, inducing the delivery of a female infant weighing 3,275 g with Apgar scores of 10 and 10 at 1 and 5 min, respectively.
Th e mother ’ s haemoglobin aft er delivery was 15 g/dl and her haematocrit NH48%, she had a macrocytosis. She refused further investigations. Th e baby did well. At the age of 5 months there was no splenomegaly, all biological tests were normal.
Th e third pregnancy occurred 4 years later. At 14 weeks ’ gestation, an ultrasound examination revealed a recurrence of fetal ascites with pleural eff usion. At 24 weeks ’ gestation, another ultra-sound examination showed increased fetal ascites. Pleural eff usion had disappeared, and all organs appeared normal. Th ere was no polyhydramnios or placental oedema. Values for peak fl ow veloc-ity of the middle cerebral artery remained in the normal range. An amniocentesis was performed revealing normal karyotype. Tests for storage diseases, digestive enzymes and infections were negative. At 30 weeks ’ gestation, the patient was hospitalised for preterm labour. Betamethasone was administered to induce pulmonary matura-tion. At this time, the patient showed an elevated serum glutamate oxaloacetate transaminase (SGOT) and serum glutamate pyruvate transaminase (SGPT) concentration. A hepatic biopsy was then per-formed under suspicion of acute fatty liver of pregnancy. Th is biopsy was normal.
During hospitalisation, an ultrasound examination revealed increased ascites compared to the examination at 24 weeks. Th is ascites was more pronounced than in the previous pregnancy. A caesarean section was performed at 31 weeks ’ gestation due to prema-ture rupture of membranes. A male infant with markedly increased abdominal circumference was born. Apgar scores were 6 and 6 at 1 and 5 min, respectively. He showed a mild respiratory distress as well as hypotonia and cyanosis. Th e abdomen was soft and distended with poorly formed abdominal musculature. Initial respiratory distress progressively disappeared and blood gases became normal with a CPAP for 13 days. A paracentesis was performed on his fi rst day of life; 300 ml of pale yellow coloured ascitic fl uid were removed (protein 28 g/l, glucose 4.3 mmol/l, LDH 188 UI/l; amylase 2 UI/l). No recurrence was reported aft erwards. Haemoglobin was 15.4 g/dl, macrocytosis was observed, white cells and platelets were normal. Th e patient ’ s course was favourable and he was transferred to the neonatal intermediate care unit. Presently, this infant is doing well.
Aft er this third pregnancy, the mother presented with spleno-megaly. A blood fi lm showed a compensated haemolysis and a macrocytosis with signifi cant reticulocytosis (271.5 10 9 /l), normal haemoglobin (14.9 g/dl), and increased mean corpuscular volume (99.9 fl ). Th en extensive investigations were performed. Th ere was no evidence of thalassaemia. But, it seemed highly likely that mother and children had the same dominantly inherited problem of intrauterine ascites, possibly associated with the mother ’ s mild haemolysis. An ektacytometry was performed, the ektacytometric curve was shift ed left ward, as typically found in dehydrated hereditary stomatocytosis. It was concluded as DHS. Ektacytometry was performed on all children and they had DHS similar to their mother.
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528 Obstetric Case Reports
Discussion Fetal ascites is not a frequent ultrasound fi nding. An underlying pathology or associated disorder occurs in 92% of cases. A care-ful search for the aetiology of ascites should be undertaken. It is frequently associated with several pathologies such as parvovirus, cardiac malformation or dysfunction and aneuploidy. DHS is a rare cause of ascites with a relatively benign prognosis (Ami et al. 2009). It is defi ned by mild anaemia, increased mean corpuscular volume and increased mean corpuscular haemoglobin concentration. DHS is caused by a defect in the ionic permeability of the red blood cell membrane (S á nchez et al. 2005).
Th e pathophysiology of fetal oedema in DHS is unclear. It is likely that it would result from the dysfunction of an ion channel, or an ion channel regulating protein in red cells (Ogburn et al. 2001). Th is dysfunction would be expressed, in the prenatal or perinatal period, in endothelial cells. As a result, fl uid may be extruded in diff erent cavities. But the exact red cell membrane defect and the way the ascites is produced remain unknown.
Th e exact prevalence of DHS is not yet known. Th e responsible gene maps to 16q23-q24 but the mutant gene responsible is unknown (Carella et al. 1998).
Commonly, symptomatology includes a mild and well-compensated haemolytic anaemia associated with a high reticulocyte count. Splenectomy is not indicated in DHS due to increased risk of thrombosis. Th e osmotic gradient ektacytometric curve shows a left ward shift (Vicente-Guti é rrez et al. 2005). Some patients with DHS develop prenatal oedema. Eleven cases of prenatal oedema due to DHS have been described in the literature (Ami et al. 2009; Basu et al. 2003; Entezami et al. 1996; Grootenboer et al. 1998; Grootenboer-Mignot et al. 2003; Vicente-Guti é rrez et al. 2005). Fetal ascites have been detected with ultrasound examination in all cases between 15 and 32 weeks ’ gestation. No predictive factor exists for severity of fetal oedema in DHS families. Th e severity of the eff usions may vary from ascites to severe hydrops. Five cases of fetal hydrops have been described and only one case of ascites and pleural eff usion is reported (Grootenboer-Mignot et al. 2003). Th ere is no evidence in the literature suggesting that the variable expressivity of the fetal disease depends on the sex of the fetus. Diagnosis was confi rmed for all newborns by ektacytometry. Th e degree of fetal anaemia is not related to the intensity of oedema.
Oedema commonly resolves in late gestation or within weeks of birth and never recurs (Basu et al. 2003, Grootenboer-Mignot et al. 2003). It does not change substantially aft er blood transfusion or drainage of ascites and tends to resolve spontaneously. In these cases, intrauterine transfusions would not be justifi ed by the intensity of anaemia and would not reduce the oedema (Grootenboer-Mignot et al. 2003). Non-invasive management is reasonable (Entezami et al. 1996). Because fetal oedema will resolve spontaneously, both family and medical teams should be confi dent.
To conclude, fetal oedema without obvious cause should raise the possibility of DHS even in the absence of any known family history. Th e prognosis of this pathology is less severe than other fetal ascites causes.
Declaration of interest: Th e authors report no confl icts of interest. Th e authors alone are responsible for the content and writing of the paper.
References Ami O , Picone O , Gar ç on L et al . 2009 . First-trimester nuchal abnormalities
secondary to dehydrated hereditary stomatocytosis . Prenatal Diagnosis 29 : 1071 – 1074 .
Basu AP , Carey P , Cynober T et al . 2003 . Dehydrated hereditary stomatocytosis with transient perinatal a sc ites. Archives of Disease in Childhood. Fetal and Neonatal Edition 88 : F438 – F439 .
Beaurain G , Mathieu F , Grootenboer S et al . 2007 . Dehydrated hereditary stoma-tocytosis mimicking familial hyperkalaemic hypertension: clinical and genetic investigation . European Journal of Haematology 78 : 253 – 259 .
Carella M , Stewart G , Ajetunmobi JF et al . 1998 . Genomewide search for dehy-drated hereditary stomatocytosis (hereditary xerocytosis): mapping of locus to chromosome 16 (16q23-qter) . American Journal of Human Genetics 63 : 810 – 816 .
Entezami M , Becker R , Menssen HD et al . 1996 . Xerocytosis with concomi-tant intrauterine ascites: fi rst description and therapeutic approach . Blood 87 : 5392 – 5393 .
Grootenboer S , Barro C , Cynober T et al . 2001 . Dehydrated hereditary stomatocy-tosis: a cause of prenatal ascites . Prenatal Diagnosis 21 : 1114 – 1118 .
Grootenboer S , Schischmanoff PO , Cynober T et al . 1998 . A genetic syndrome associating dehydrated hereditary stomatocytosis, pseudohyperkalaemia and perinatal oedema . British Journal of Haematology 103 : 383 – 386 .
Grootenboer-Mignot S , Cr é tien A , Laurendeau I et al . 2003 . Sublethal hydrops as a manifestation of dehydrated hereditary stomatocytosis in two consecutive pregnancies . Prenatal Diagnosis 23 : 380 – 384 .
Ogburn PL Jr, Ramin KD , Danilenko-Dixon D et al . 2001 . In utero erythrocyte transfusion for fetal xerocytosis associated with severe anemia and non-immune hydrops fetalis . American Journal of Obstetrics and Gynecology 185 : 238 – 239 .
S á nchez M , Palacio M , Borrell A et al . 2005 . Prenatal diagnosis and manage-ment of fetal xerocytosis associated with ascites . Fetal Diagnosis and Th erapy 20 : 402 – 405 .
Vicente-Guti é rrez MP , Castell ó -Almaz á n I , Salv í a-Roiges MD et al . 2005 . Nonim-mune hydrops fetalis due to congenital xerocytosis . Journal of Perinatology 25 : 63 – 65 .
An unusual cause of postpartum collapse: Undiagnosed myasthenia gravis
K. W . Cheung 1 , N. W. M. Shek 1 & K. H. Chan 2
Departments of 1 Obstetrics and Gynaecology and 2 Medicine, Queen Mary Hospital, University of Hong Kong, Hong Kong SAR, China
DOI: 10.3109/01443615.2013.782274
Correspondence: K. W. Cheung, Department of Obstetrics and Gynaecology, Queen Mary Hospital, University of Hong Kong, 102 Pokfulam Road, Hong Kong SAR, China. E-mail: [email protected]
Introduction Maternal weariness is common in the early puerperium. It is oft en perceived as normal and women are reassured. However, persistent or progressive weakness during pregnancy is unusual and clinicians should be alert to possible causes. We report a case of undiagnosed myasthenia gravis presenting with postpartum collapse.
Case report A 37-year-old primigravida presented with respiratory failure and postpartum collapse one day aft er an uneventful elective lower seg-ment caesarean section (LSCS). Th is was a dichorionic-diamniotic twin pregnancy conceived with the help of in-vitro fertilisation because of primary subfertility. She had gestational diabetes and her glycaemic control remained satisfactory throughout the pregnancy. She was seen regularly in the antenatal clinic and remained well. Elec-tive LSCS was performed uneventfully under spinal anaesthesia aft er term. Th e babies were transferred to the neonatal unit for observa-tion due to meconium-stained liquor. She complained of shortness of breath 28 h aft er delivery. Physical examination revealed drowsiness, bilateral ptosis, general weakness with limb power grade 2/5, hypore-fl exia, poor respiratory eff ort and cyanosis. She was desaturated on room air (SaO 2 50 – 60%) and hypotensive with a tachycardia (blood pressure 80/60 mmHg, pulse rate 110 b.p.m). Th e obstetrician and anaesthetist arrived immediately for resuscitation. Th ere was no sign of internal bleeding and vaginal bleeding was minimal. She lost consciousness later and was intubated by the anaesthetist. She was transferred to intensive care with an intravenous dopamine infusion. Electrocardiography, chest X-ray and computerised tomography brain scan were unremarkable. Bronchoscopy revealed thick copious yellowish sputum throughout her right upper lobe and suction was performed. Her husband recalled that the patient had generalised weakness throughout the pregnancy. He had to help her get changed and carry her back home aft er the clinic visit, especially in the later part of the gestation. A neurologist was consulted and neuromus-
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