Incisional Hernia Complicating Pregnancy In Ehlers-Danlos Syndrome

BJ O’Daly (1), Karl J Sweeney  (1), ADK Hill (1), P Lenehan (2), EWM Mcdermott (1) 

(1) Surgical Professorial Unit, St. Vincent’s University Hospital, Dublin 4, IRELAND and

(2) Department of Obstetrics, National Maternity Hospital, Dublin 2, IRELAND 

Address for Correspondence:  Dr. Brendan J. O’Daly, 17 Merrion Woods, Blackrock, Co. Dublin, IRELAND 

e-mail: brendan.odaly@ucd.ie

 

ABSTRACT

Ehlers-Danlos syndrome (EDS) is a rare hereditary disorder of connective tissue.  Specific obstetric complications reported include preterm delivery, spontaneous abortion, stillbirth, pre- and peri-partal bleeding and rupture of the great vessels, bladder and vagina.  We report the first case of a large incarcerated incisional hernia containing small bowel complicating pregnancy at 34 weeks in a multiparous patient with Ehlers-Danlos syndrome (Type I) with favourable outcome.  A healthy male infant was delivered by caesarean section with concomitant tubal ligation and herniorraphy.  Despite favorable maternal and neonatal outcome in this case, patients with EDS who wish to get pregnant need to be alerted to the risk of these rare, but potentially serious complications.

Key Words:       Ehlers Danlos syndrome; Gravis type 1; Incarcerated incisional hernia; Pregnancy; Caesarean section

 

INTRODUCTION

Ehlers-Danlos syndrome (EDS) is a heterogeneous group of hereditary connective tissue disorders with a prevalence of 1:150,000 1 .  Ehlers-Dandles syndrome (classic) type I (gravis) is estimated to occur in 30% of cases and is characterised by cutaneous and articular hyperextensibility, easy bruising, atrophic scars and fetal prematurity 2 .  Inheritance is autosomal dominant.  It is most frequently seen in Caucasians of European origin.  Surgical and obstetric intervention in these patients requires special consideration and is associated with considerable morbidity. Incisional hernias in EDS present a unique management problem with a high risk of recurrence.  Special closure techniques have been described to reduce recurrence rate 3 . We describe a case of pregnancy in an EDS patient complicated by a large incisional hernia. 

 

CASE REPORT

A 35-year old woman, para 2, presented to the Emergency Department at an acute tertiary hospital following referral from a maternity hospital at 34 weeks gestation with a 10-hour history of central abdominal pain radiating to the back.  She had four episodes of vomiting and obstipation for 12 hours.  EDS classic type I (gravis) had been diagnosed at age 9 years based on clinical, biochemical and electron microscopic findings.  Past surgical history included corrective surgery for cervical spina bifida, trans-abdominal and trans-thoracic repair for recurrent diaphragmatic hernia, severe upper gastrointestinal haemorrhage and two lower-segment caesarean sections (LSCS).  In addition, she reported a history of a hiatus hernia, easy bruising, dextrocardia, asthma and poor scar healing.  Previous indications for caesarean section were pre-eclampsia toxaemia (at 38 weeks) in her first pregnancy and pre-term labour (at 31 weeks) in her second pregnancy. 

 

In her current pregnancy, she had postural hypotension.  Corticosteroids were administered at 26 weeks to accelerate foetal lung maturity.  At 28 weeks, she was admitted to hospital for management of an infective exacerbation of asthma.  This was treated with corticosteroids and oral clarithromycin.  Elective caesarean section was planned for 36 weeks gestation.  Features of EDS on physical examination, included kyphoscoliosis, skin hyperextensibility, joint hypermobility and widened atrophic scars.  On examination, the abdomen was consistent with 34 weeks gestation.  On palpation, the abdomen was tender over a large midline incisional hernia (Figure 1).  The fundus was palpable outside the hernia.  Bowel sounds were present in the hernia.  The hernia was not reducible.  Ultrasound scan demonstrated fluid-filled loops of small bowel of normal calibre.  Fetal heart-beat was normal.  A diagnosis of incarcerated incisional hernia was made.  Pulmonary function tests revealed a forced expiratory volume (FEV1) 32% of that predicted for age, consistent with significant respiratory compromise.  Chest x-ray confirmed dextrocardia.  

 

FIGURE 1 Incisional hernia. Note additional finding of hyperextensible skin (black arrowhead)

 

Following discussion with the patient, it was elected to perform LSCS as an emergency procedure and concomitant tubal ligation and incisional herniorraphy.  Epidural anaesthesia was used.  A healthy 1900 g male infant was delivered through a standard incision.  Liquor was clear.  Apgar scores were 8/ 9/ 10 at 1, 5 and 10 minutes respectively.  The placenta was delivered intact and the uterus closed in two layers.  Tubal ligation was performed without complication.  The hernia sac contained obstructed, but viable small bowel, which was freed from the abdominal wall.  An incidental finding of small bowel diverticulae was noted (Figure 2).  The defect in the abdominal wall was closed in one single layer using monofilament polyglyconate synthetic absorbable suture (1-MAXON, Tyco Healthcare, USA).  Peri- and post-partal bleeding was not observed.  She was transferred to the intensive care unit (ICU) post-operatively for 12 hours for observation.

 

FIGURE 2 Small bowel diverticulum (black arrowhead)

 

The puerperium was complicated by right-sided pneumothorax at day 10, requiring acute re-admission.  The incisional hernia has not recurred at 12 months follow-up.

 

DISCUSSION

Severe complications in pregnancy have long been reported in women with EDS. These include prematurity with rupture of foetal membranes, pre-term delivery, spontaneous abortion, still-birth, pre- and peri-partum haemorrhage, rupture of great vessels and hollow viscus 4 .  There is a paucity of data from large studies on which to base firm conclusions about the risks of pregnancy in EDS.  There are no previous reports of incarcerated incisional hernia complicating pregnancy in EDS.

 

Few case series and reports present data with specific reference to clinical form and subtype of EDS.  Consequently, incidence of complications in pregnancy in EDS Type I is difficult to assess.  In a study of 29 EDS type I patients miscarriage (60%), premature rupture of membranes (75%), caesarean section (25%) and postpartum complications (60%) are reported 5 .  Incidence of post-partum haemorrhage is reported at 33% in a smaller study 4 .  Patients with EDS who wish to get pregnant should be alerted to the potential risks.  Pre-conceptual counseling concerning specific possible complications and a multidisciplinary approach are recommended.  Guidance and management must be analyzed individually and according to the clinical form.

REFERENCES

1.             Taylor DJ, Wilcox I, Russell JK. Ehlers-Danlos syndrome during pregnancy: a case report and review of the literature. Obstet Gynecol Surv 1981; 36: 277-81.

2.             Munz W, Schlembach D, Beinder E et al. Ehlers-Danlos syndrome type I in pregnancy: a case report. Eur J Obstet Gynecol Reprod Biol 2001; 99: 126-8.

3.             Girotto JA, Malaisrie SC, Bulkely G et al. Recurrent ventral herniation in Ehlers-Danlos syndrome. Plast Reconstr Surg 2000; 106: 1520-6.

4.             Lind J, Wallenburg HC. Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population. Acta Obstet Gynecol Scand 2002; 81: 293-300.

5.             Ainsworth SR, Aulicino PL. A survey of patients with Ehlers-Danlos syndrome. Clin Orthop Relat Res 1993; 286: 250-6.

 
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