A Hard Case to Swallow: Achalasia in Pregnancy

Sir,

Achalasia is a rare condition, and its’ complications in pregnancy are not commonly described. The effects of malnutrition on both the mother and fetus are significant, and can dictate the mode and timing of delivery. A 28 year old nulliparous woman with a long-standing history of achalasia presented at 32 weeks gestation with severe dehydration secondary to regurgitation and intolerance of oral fluids. The woman had been reviewed extensively by a dietitian, with monitoring of her weight gain and fetal growth. A diagnosis of malnutrition and hypovolaemia secondary to achalasia was made, and she was referred to her upper gastrointestinal surgeon.

Oesophageal dilatation under general anaesthetic was offered. She declined this option due to concerns for her fetus. A naso-jejunal (NJ) tube was sited with difficulty at 33 weeks and slow enteral feeding was used to avoid re-feeding syndrome. At 35 weeks, her symptoms deteriorated, and required her tube to be re-sited by endoscopy. Induction of labour was planned for 38 weeks. Repeated blockages of the NJ tube secondary to spasm led to its removal at 37 weeks, and she was admitted for rehydration. Total parenteral nutrition was considered, but deemed unnecessary at this point.

Given the severity of the woman’s condition, and the woman’ own wishes, a decision was made to deliver the fetus. The patient was unsuitable for induction of labour, and a healthy female infant was delivered by lower segment caesarean section. She underwent a pneumatic dilatation three days post-partum, and was discharged home on day five. The woman made a good recovery and was well six weeks postpartum and eating normally. Achalasia is a motor disorder of the oesophageal smooth muscle, where the lower oesophageal sphincter does not relax normally, and the oesophagus itself undergoes non-peristaltic contractions1. The American College of Gastroenterology recommends oesophageal motility testing, and fluoroscopic imaging, such as barium swallow to confirm the diagnosis1. A dilated oespohagus with tapering of the inferior aspect, the “bird-beak” sign, is typically pathognomonic for achalasia, as seen in figure 1.

Achalasia is a rare condition, with a prevalence of approximately 8 per million1.  Data is limited regarding achalasia in pregnancy, with the largest review in the literature describing only 35 cases3. Maternal and fetal outcomes were generally good; however the majority required expedited delivery3.

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Figure 1: Barium swallow showing ‘bird-beak’ sign. From Farrokhi et al2.

Calcium channel blockers and nitrates have been used to reduce lower oesophageal pressures; though are contraindicated in pregnancy1. Similarly, surgical myotomy has been used with good results, but requires general anaesthesia. Pneumatic dilatation is the most effective non-surgical treatment option1, and can be performed under sedation, preferable in the pregnant patient. Endoscopic injection of Botulinum toxin is recommended in patients who are unsuitable for other treatments1Malnutrition secondary to poor oral intake is the most common complication of achalasia in pregnancy3. Fetal growth trajectory is sensitive to the effects of maternal nutrition during the entire antepartum period4.

Given its rarity, there are no conclusive guidelines for treating achalasia in pregnancy, and so each case must be managed individually with senior multidisciplinary input.

B D. O’Leary 1 S Curran2  V Broderick1                                     

1 Fetal Medicine Unit, National Maternity Hospital, Dublin, Ireland.

2 Department of Dietetics, National Maternity Hospital, Dublin, Ireland.

Conflict of Interest

The authors report no conflict of interest.

Correspondence: Dr. Bobby O’Leary, Fetal Medicine Unit, National Maternity Hospital, Dublin 2.

Tel: +353 (1) 637 3217

Email: roleary@ucd.ie

References

  1. Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: diagnosis and management of achalasia. Am J Gastroenterol. 2013; 108: 1238-49.
  2. Farrokhi F, Vaezi MF. Idiopathic (primary) achalasia. Orphanet J Rare Dis. 2007; 2: 38.
  3. Khudyak V, Lysy J, Mankuta D. Achalasia in pregnancy. Obstet Gynecol Surv. 2006; 61: 207-11.
  4. Wu G, Imhoff-Kunsch B, Girard AW. Biological mechanisms for nutritional regulation of maternal health and fetal development. Paediatr Perinat Epidemiol 2012; 26: 4-26.

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