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Original Article

S. Hosie 1

S. Loff 1

H. Wirth 1

H.-J. Rapp 2

C. von Buch 3

K.-L. Waag 1

Experience of 49 Longitudinal Intestinal Lengthening Procedures for Short Bowel Syndrome

Abstract

Patients, Methods and Results: Forty-nine patients with a mean age of 25 months underwent a longitudinal intestinal lengthen- ing procedure for short bowel syndrome (SBS) in our institution. Indications for the operation were dependence on parenteral nu- trition in spite of adequate conservative management. The small bowel was lengthened from a mean of 27 cm to a mean of 51 cm. There was no intraoperative mortality. The following early com- plications occurred in our early series: ischemia of a short bowel segment of 2 cm, requiring resection in two patients, insuffi- ciency of the longitudinal anastomosis in two patients and an intra-abdominal abscess in one. Four of 9 non-survivors died of liver failure and 3 of sepsis. Follow-up showed that 19 patients were weaned from parenteral nutrition after a mean of 9.1 months. Long-term complications encountered were dismotility with malabsorption due to bacterial overgrowth caused by pro- gressive dilatation of the bowel, d-lactic acidosis, cholelithiasis and urolithiasis. Conclusions: A longitudinal intestinal length- ening procedure is an effective and safe surgical approach for SBS, provided it is perfomed in time, the patient’s preoperative condition is optimized and technical surgical details are taken into account.

Key words Short bowel syndrome · bowel lengthening · surgical treatment

Résumé

Patients, Méthodes et Résultats: 49 patients avec un âge moyen de 25 mois ont subi un allongement longitudinal de l’intestin pour un grêle court dans notre Institution. Les indications de cette opération étaient la dépendance à la nutrition parentérale en dépit de la prise en charge. L’intestin était allongé de 25 cm à 51 cm. Il n’y a pas eu de mortalité intra-opératoire. Les complica- tions survenant précocement dans notre première série étaient:

une ischémie d’un segment intestinal de 2 cm nécessitant une résection chez deux patients, une insuffisance de l’anastomose longitudinale chez deux patients, et un abcès intra-abdominal chez un patient. Quatre sur 9 des patients décédés présentaient une défaillance hépatique et trois un sepsis. Le suivi montrait que chez 19 patients on pouvait arrêter la nutrition parentérale après une moyenne de 9,1 mois. Les complications à long terme rencontrées étaient une dysmotilité avec une malabsorption due à une pullulation microbienne causée par une dilatation progres- sive de l’intestin, une acidose lactique, une lithiase cholédo- cienne et une urolithiase. Conclusion: L’allongement longitudi- nal de l’intestin est une méthode efficace et sûre pour améliorer les grêles courts, dans la mesure où cette intervention est réali- sée au moment où les conditions pré-opératoires du patient sont optimales et que les détails de la technique chirurgicale sont bien pris en compte.

Mots-clés Grêle court · allongement du grêle · traitement chirurgical

171

Affiliation 1 Department of Pediatric Surgery, Mannheim University Hospital, Heidelberg University, Germany 2 Department of Anesthesia, Mannheim University Hospital, Heidelberg University, Germany 3 Department of Pediatrics, Mannheim University Hospital, Heidelberg University, Germany

Correspondence Prof. Dr. med. Stuart Hosie · Klinik für Kinderchirurgie · Klinikum München Schwabing · Kölner Platz 1 · 80804 München · Germany · E-mail: stuart.hosie@kms.mhn.de

Received: October 30, 2004 · Accepted after Revision: December 11, 2004

Bibliography Eur J Pediatr Surg 2006; 16: 171 – 175 © Georg Thieme Verlag KG Stuttgart · New York · DOI 10.1055/s-2006-924251 · ISSN 0939-7248

Original Article

Resumen

Pacientes, Métodos y Resultados: 49 pacientes con una edad media de 25 meses fueron sometidos a alargamiento intestinal

longitudinal por síndrome de intestino corto en nuestra institu- ción. La indicación para la operación fue la dependencia de ali- mentación parenteral a pesar del tratamiento conservador ade- cuado. En intestino delgado fue alargado de una media de 27 cm

a una media de 51 cm. No hubo mortalidad intraoperatoria. Las

siguientes complicaciones precoces ocurrieron en nuestras se- ries iniciales: Isquemia de un segmento de 2 cm que requirió re- sección en 2 pacientes, insuficiencia de la anastomosis longitudi-

nal en 2 pacientes y absceso intraabdominal en 1. Cuatro de 9 no sobrevivientes murieron de fallo hepático y 3 de sepsis. El segui- miento ha mostrado que 19 pacientes pudieron ser liberados de

la alimentación parenteral tras una media de 9,1 meses. Las com-

plicaciones encontradas a largo plazo fueron dismotilidad con malaabsorción debida a sobrecrecimiento bacteriano causado por dilatación progresiva del intestino, acidosis d-láctica, coleli- tiasis y urolitiasis. Conclusion: El alargamiento longitudinal in- testinal es efectivo y seguro para el síndrome de intestino corto siempre que se lleve a cabo a tiempo, que la condición preopera- toria del paciente sea buena y que los detalles quirúrgicos técni- cos sean tenidos en cuenta.

Palabras clave Síndrome del intestino corto · alargamiento intestinal · trata- miento quirúrgico

Zusammenfassung

Patienten, Methoden und Ergebnisse: 49 Patienten mit einem mittleren Alter von 25 Monaten wurden einer longitudinalen Darmverlängerung aufgrund eines Kurzdarmsyndroms unterzo- gen. Operationsindikation war die Notwendigkeit der parentera- len Ernährung trotz adäquater konservativer Therapie. Der Dünndarm wurde im Mittel von 27 cm auf 51 cm verlängert. Es trat keine intraoperative Mortalität auf. Folgende frühe Kompli- kationen wurden beobachtet: Ischämie eines 2 cm messenden Dünndarmsegmentes, welches reseziert werden musste, bei zwei Patienten, Insuffizienz der Längsnaht bei zwei Patienten und ein intraabdomineller Abszess bei einem weiteren. 9 Patien- ten sind verstorben, davon vier an Leberinsuffizienz und 3 an Sepsis. Die Nachuntersuchung hat gezeigt, dass 19 Patienten nach im Schnitt 9,1 Monaten keiner parenteralen Ernährung mehr bedurften. Beobachtete Spätkomplikationen waren Darm- transportstörung mit Malabsorption aufgrund bakterieller Über- wucherung durch eine erneute Darmdilatation, D-Laktat-Azido- se, Cholelithiasis und Urolithiasis. Schlussfolgerungen: Die lon- gitudinale Darmverlängerung ist eine effektive und sichere Ope- rationstechnik bei Kurzdarmsyndrom, vorausgesetzt die Opera- tionsindikation wird beizeiten gestellt, der präoperative Zustand des Patienten wird optimiert und operativ-technische Einzelhei- ten werden beachtet.

Schlüsselwörter Kurzdarmsyndrom · Darmverlängerung · chirurgische Therapie

172

Introduction

Short bowel syndrome (SBS) is defined as malabsorption after congenital or acquired loss of part of the small intestine [3]. The incidence of neonatal SBS in a Canadian population-based study was 24.5 per 100 000 live births, with a reported mortality esti- mate for children under the age of 4 years of 2/100 000 popula- tion/year [10].

Since the first description of a longitudinal intestinal lengthen- ing procedure by Bianchi in 1980, this technique has gained wide acceptance as the surgical therapy for short bowel syndrome in children [2]. With this technique, the dilated small bowel is divided longitudinally, two narrower loops are created and anas- tomosed in an isoperistaltic manner. This improves intestinal transport, prevents stasis of bowel contents and therefore pre- vents bacterial overgrowth. Furthermore, transit time is prolong- ed, and the contact area between intestinal mucosa and bowel contents is optimized [2, 7,11]. Nevertheless the indication to perform this procedure is rare, so that larger series are scarce.

The aim of this report is to describe our series of 49 patients, who underwent a longitudinal intestinal lengthening procedure for SBS, with particular focus on the preoperative evaluation, techni- cal surgical aspects and postoperative management.

Hosie S et al. Experience of 49 … Eur J Pediatr Surg 2006; 16: 171 – 175

Patients and Methods

Since 1982 49 patients underwent an intestinal lengthening pro- cedure in our institution. Table 1 shows the entities which led to the short bowel syndrome.

Indications for operation were: dependence on parenteral nutri- tion and impossibility of achieving at least 50% of the caloric re- quirement enterally, after 6 months of adequate conservative treatment. Further indications were the inclusion of all intestinal segments into bowel continuity by anastomoses of eventual en- terostomies.

Preoperative studies included a thorough clinical examination, complete blood cell count, serum electrolytes, C-reactive protein, transaminases, bilirubin, acetylcholinesterase, total protein and albumin, fat-soluble vitamins, trace elements and coagulation tests (prothombin time, thrombin time and activated partial

tests (prothombin time, thrombin time and activated partial Table 1 Entities leading to short bowel syndrome

Table 1 Entities leading to short bowel syndrome

Gastroschisis

18

Small bowel atresia

14

Midgut volvulus

10

Necrotizing enterocolitis

7

Total

49

Fig. 1 Small intestinal loop divided and anti-mesenteric sides. Note the branches of the mesenteric

Fig. 1 Small intestinal loop divided

and anti-mesenteric sides. Note the branches of the mesenteric ves- sels supplying each bowel strip.

longitudinally at the mesenteric

thromboplastin time). A gastrointestinal contrast study was per- formed in every case to evaluate the patency, length and dilata- tion of the remaining small bowel, and also the presence and length of the colon and, if clinically suspected, gastroesophageal reflux. Doppler imaging of the central vessels was performed when indicated, to assess patency and possible thrombosis. Seri- al blood culture as well as nasopharyngeal cultures were obtain- ed to search for potential pathogens, since most patients were re- ferred from other institutions and had spent most of their lives in hospital. In the last 5 patients, we included percutaneous liver biopsy and determination of single coagulation factors in the preoperative evaluation.

The operative technique has been previously described [8, 9]. Briefly, only small bowel dilated to a diameter of at least double the normal size (about 5 cm) was considered adequate for the lengthening procedure. The bowel loop was divided longitudi- nally along the antimesenteric border using bipolar scissors. Di- vision on the mesenteric side was accomplished in the same manner, after careful dissection of the small vessels supplying each half-circumference of the bowel loop (Fig. 1 ). Fashioning of the new bowel loops was accomplished by inverting running su- tures; anastomoses were performed in an isoperistaltic manner (Figs. 2 and 3 ). Oral feeding was started after return of bowel function, usually on the 5th postoperative day. Feedings were progressively advanced until achieving 10 bowel movements per day. Solid food was introduced when formula was tolerated by the patient.

Results

Patients’ mean age at the time of operation was 25 months with a range of 4 months to 12 years. Preoperatively the small bowel had a mean length of 27 cm (range, 12 to 60 cm). After the length- ening procedure, the mean small bowel length was increased to 51 cm (range 18 to 120 cm). There was no intraoperative mortal- ity. As early complications we observed ischemia of a short bow- el segment of 2 cm in two patients. This was noticed intraopera-

Original Article Fig. 2 A thinner loop was created by longitudinal suture of one bowel
Original Article
Fig. 2 A thinner loop was created by longitudinal suture of one bowel
strip. The proximal part of the loop corresponds to the right side of the
picture.
of the loop corresponds to the right side of the picture. 173 Fig. 3 Small bowel

173

Fig. 3 Small bowel loops after longitudinal intestinal lengthening. Longitudinal suture lines were covered with fibrin glue. After position- ing the loops in a circular manner, the most proximal part, in continuity with the duodenum, corresponds to the lower loop on the left side of the picture. The right side of the lower loop has to be anastomosed with the left part of the upper loop. The right end of the upper loop is anastomosed to the colon.

tively and required resection. Two patients developed a leakage along the longitudinal suture, and one patient developed an in- tra-abdominal abscess necessitating re-laparotomy and drain- age.

Nine patients have died so far, which amounts to a mortality rate of 18%. Causes of death are shown in Table 2 .

Follow-up of our patients showed that 19 patients were weaned from parenteral nutrition after a mean of 9.1 months (range 1 – 72 months). 5 patients still need parenteral nutrition at home 4 to 21 months postoperatively. 16 patients were lost to follow-up. Frequently encountered problems in the long term were bowel dysmotility secondary to recurrent dilatation of the lengthened bowel loops and bacterial overgrowth leading to steatorrhea, d-

Hosie S et al. Experience of 49 … Eur J Pediatr Surg 2006; 16: 171 – 175

Original Article

Original Article Table 2 Causes of mortality after longitudinal intestinal lengthen- ing Liver failure 4 Sepsis

Table 2 Causes of mortality after longitudinal intestinal lengthen- ing

Liver failure

4

Sepsis

3

Massive aspiration with gastroesophageal reflux

1

Cardiac failure, multiple thromboses

1

Total

9

lactic acidosis requiring dietary measures and bowel decontami- nation, cholelithiasis and urolithiasis.

Discussion

Previous to considering a lengthening procedure the following conditions must be fulfilled:

all intestinal segments must be included into bowel continu- ity by closure of enterostomies in order to recruit the entire resorption area available;

allowance must be made for adequate bowel adaptation by offering oral feedings over a sufficient time interval;

there must be a sufficient adaptive bowel dilatation of about 5 cm diameter;

it must previously have been proved to be impossible to in- crease the enteral caloric intake despite adequate conserva- tive treatment.

Since these patients are frequently multimorbid, a careful preop-

174 erative evaluation is essential. In the early series we operated on patients with end-stage liver disease as an ultima ratio. All of them died of liver failure in the early postoperative period, which led us to exclude such patients from surgical therapy.

It is imperative to optimize the patients’ preoperative condition,

for example to compensate nutritional deficits such as deficits of vitamins (vitamin B 12 , fat-soluble vitamins such as vitamin K which are important for synthesis of coagulation factors) and trace elements, and also to correct electrolyte and acid-base dis- orders, anemia, coagulation disorders, thrombocytopenia and hypoalbuminemia. It is also highly important to detect and treat thrombosis resulting from the central venous catheter. Thorough bacteriological screening allows early institution of specific anti- bacterial therapy, and adequate perioperative prophylaxis, in- cluding antifungal agents when required. Frequently liver func- tion will partially recover by reducing or cycling parenteral lipids.

Some patients have a compensated functional impairment of the liver in spite of normal or nearly normal laboratory findings. They may acutely decompensate after an insult such as an oper- ative procedure or a perioperative infection. One patient died in

the early postoperative period from liver failure after a postoper- ative sepsis, although she had had normal preoperative labora- tory tests. After this experience, we started to routinely perform

a percutaneous liver biopsy preoperatively in order to assess the

real liver status, and thus to be able to estimate risk and prog- nosis.

Hosie S et al. Experience of 49 … Eur J Pediatr Surg 2006; 16: 171 – 175

Liver failure caused by progressive cholestasis due to long-term total parenteral nutrition and recurrent septicemias is the main cause of death in patients with SBS. In our series 4 out of 9 pa- tients died of end-stage liver failure. Bianchi reported on a series of 20 patients who underwent a longitudinal lengthening proce- dure. Overall mortality was 55%, in all cases due to end-stage liv- er failure [1]. For this reason we strongly recommend early refer- ral to surgical therapy as soon as indicated. The prolongation of an unsuccessful conservative treatment with the risk of deterio- ration of liver function or other major complications is counter- productive.

Dilatation of the small bowel on preoperative X-ray studies is most probably secondary to the adaptive response after massive intestinal loss. It should not be misinterpreted as secondary dila- tation due to mechanical obstruction, since this could lead to fur- ther resection of vitally important bowel surface.

In contrast to other authors we do not routinely perform longitu- dinal lengthening using a conventional stapling device, since the branches are very big [6, 7,11]. Thompson et al. reported necrosis of one of the divided bowel limbs, requiring resection, after a lengthening procedure performed with a stapler [6]. In our opin- ion careful dissection and sparing of the small mesenteric vessels is best accomplished with fine scissors. Bleeding is markedly re- duced with the help of bipolar scissors. Inverted hand suturing allows the exact apposition of the intestinal wall, sparing the small mesenteric vessels. Furthermore this technique minimizes the loss of intestinal area due to the surgical procedure. Never- theless, on one occasion we used an endoscopic linear cutter (Ethicon, Nordestedt, Germany) with good results. The instru- ment’s branches are small enough to be inserted in the triangle between the mesenteric bowel wall and the vessels supplying each bowel hemicircumference. Sealing of the suture lines with fibrin glue seems to be effective in preventing fistula formation and avoids traction on the mesenteric vessels. We have not ob- served any insufficiency of the suture lines since we routinely use fibrin glue.

Like Bianchi, we consider an intestinal diameter of about 5 cm suitable for the lengthening procedure [1]. Weber considered a diameter of 3 cm adequate and used a stapling device in all cases except one. Five of his 16 patients developed bowel obstruction in the early postoperative period, which required re-operation [11]. We have not experienced this complication so far.

The most frequent complication in the long term is recurrent di- latation of the lengthened bowel segments. This phenomenon leads to dismotility, stasis, bacterial overgrowth and malabsorp- tion. In the past, these symptoms had to be treated conserva- tively with dietary measures and recurrent bowel decontamina- tion with antibiotics, eventually leading to further problems such as candidiasis. Kim and coworkers recently published a sur- gical technique they called serial transverse enteroplasty (STEP). The bowel is lengthened by serial transverse incisions with a GIA stapler from opposite directions, creating a zig-zag loop [4]. Am- ple and long-term experience with this technique is still not available, therefore we would be hesitant to use it as the primary procedure for the surgical therapy of SBS at present. Neverthe- less it might prove to be effective and safe in future, particularly

Original Article

as an option to treat recurrent dilatation after longitudinal intes-

tinal lengthening [5].

Our series, the largest published so far, shows that the longitudi-

nal intestinal lengthening procedure is a safe and effective surgi- cal therapy for children with SBS, provided that the surgical ther- apy is performed early enough, before liver function deteriorates.

A careful and extensive preoperative evaluation and therapy

leading to an optimal preoperative condition is of vital impor- tance. A careful surgical technique, which takes account of vari- ous surgical details, should reduce the complication rate and

thus improve outcomes.

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