Bilateral thoracoscopic splanchnicectomy in chronic intestinal pseudo‐obstruction: report of two paediatric cases

Chronic intestinal pseudo‐obstruction (CIPO) is a rare, highly morbid disorder characterised by signs and symptoms of intestinal obstruction in the absence of mechanical obstruction.¹ With no specific aetiology to tackle, management of CIPO focuses on symptom control and maintenance of an adequate nutritional status. Intractable chronic abdominal pain is a frequent and distressing feature in CIPO.

Here we report on the effect of bilateral thoracoscopic splanchnicectomy (TS) in two boys, one mentally retarded, with severe CIPO refractory to conventional therapy. Their debilitating chronic abdominal pain and markedly impaired quality of life (QoL) was iteratively alleviated by transient bilateral coeliac blocks (CB). After 26 and 9 successful CB, respectively, TS was performed. The greater and lesser splanchnic nerves were coagulated and divided bilaterally in both patients (fig 1).

Figure 1 Thoracoscopic localisation and section of splanchnic nerves. Right pleural cavity, patient head on the right hand side. The greater (GS) and lesser (LS) splanchnic nerves were identified (A) and sectioned under visual control (B). D, diaphragm; V, intercostal vessels; L, lung.

The postoperative course was uneventful. Mild diarrhoea occurred on the first day after CB and was followed by a lasting improvement in bowel habits. Resolution of complaints and clinical signs, including a dramatic improvement in psychomotor and social behaviour of the mentally retarded boy, persisted over a follow up period of 30 and 25 months, respectively.

Management of CIPO remains challenging. Various therapeutic options have been proposed: dietary measures, total parenteral nutrition, prokinetic drugs, surgical procedures (for example, feeding gastrostomy or jejunostomy), gastrointestinal decompression procedures, gastric or intestinal pacing, bowel resections, and small bowel transplantation.²,³ Nevertheless, QoL often remains disappointingly poor. Chronic abdominal pain is a distressing feature in CIPO. Pain is especially difficult to assess in toddlers and in mentally retarded children. In teenagers, chronic pain is related to low self care and decreased participation in school and social activities.⁴

CB and TS both proved efficient for pain relief in cancer of the pancreas and in chronic pancreatitis.⁵,⁶,⁷ CB with local anaesthetic agents appears quite safe but offers only transient relief. Conversely, CB performed with destructive agents offers longer lasting relief but carries a risk of serious side effects, including paraplegia and circulatory arrest.⁸,⁹ We made the safe choice of anaesthetic CB for its reversibility as an unconventional therapeutic test. Transient diarrhoea, followed by improvement in bowel habits, was observed after CB and TS in both patients. This likely reflects relief of the gut from inhibitory sympathetic efferent signals, leading to an increase in motility and decrease in water absorption by excitatory cholinergic innervation.¹⁰ The role of the intrinsic enteric nervous system in the generation of excessive sympathetic reflex remains unclear. Improvement in gut motility after CB or TS suggests that, despite marked abnormalities (hyperganglionosis), the enteric nervous system was still able to organise adequate peristalsis after interruption of hyperactive sympathetic inhibitory reflexes. Furthermore, in both patients, immediate pain relief was observed after CB and TS, emphasising the prominent role of sympathetic afferent pathways in their painful symptoms. Altogether, these observations suggest that sympathetic reflexes played a central role in the physiopathology of painful CIPO in these two young patients.

The large number of procedures before TS (our patients underwent 26 and 9 CB, respectively) illustrates both the striking improvement provided by CB that strengthened treatment compliance and the reluctance of physicians to perform an irreversible procedure for an unconventional indication.

Based on our experience, we propose considering TS for children with refractory painful CIPO and poor QoL after a successful therapeutic trial with iterative CB. TS offers great promise for these patients and their families but still awaits dedicated investigations in larger series of patients to determine its place in the paediatric surgeon's armamentarium.

Acknowledgements

We are indebted to Huy Nguyen Tran for preparation of the figure.

This work was supported by grants from the National Fund for Scientific Research (Belgium), Fondation Médicale Reine Elisabeth, and Fondation Universitaire David et Alice Van Buuren (Belgium) to JM Vanderwinden.