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Diaphragmatic hernia with splenic infarction – Recurrence after thoracoscopic repair
J Ped Surg Case Reports 1 (2013) 413e415
Contents lists available at ScienceDirect
Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com
Diaphragmatic hernia with splenic infarction e Recurrence after thoracoscopic repairq C. Kujath a, D. Großmann b, C. Müller b, * a b
Hannoversche Kinderheilanstalt, Department of Pediatric Surgery, Janusz-Korczak-Allee 12, 30173 Hannover, Germany University Medicine Greifswald, Department of Pediatric Surgery, Ferdinand-Sauerbruch-Str. 1, 17475 Greifswald, Germany
a r t i c l e i n f o
a b s t r a c t
Article history: Received 10 April 2013 Received in revised form 29 May 2013 Accepted 30 May 2013
We describe the case of a 17 month old boy with recurrence of congenital diaphragmatic hernia accompanied by splenic infarction and incarceration of colon. Primary surgery had been carried out thoracoscopically. On recurrence we found parts of the mesentery attached to the thoracic side of the diaphragm. Torsion of the spleen and consecutive hemorrhagic infarction was caused by the shift of splenic vessels into the thorax. Splenectomy was therefore made necessary. Ó 2013 The Authors. Published by Elsevier Inc. All rights reserved.
Key words: Diaphragmatic hernia Splenic torsion Recurrence
Congenital diaphragmatic hernia (CDH) occurs in one in 2000e3000 newborns. It can be associated with pulmonary hypoplasia and persistent pulmonary hypertension [1,2]. In 70e80% of cases CDH consists of a posterolateral defect on the left side of the diaphragm [3]. 1. Case report A 17 months old boy was presented to the emergency department with vomiting. For two days he had not drunk or eaten anything. Onset of illness was sudden when the little boy had shown signs of abdominal cramps over a short period two days earlier. Five days earlier he had shown similar symptoms of abdominal spasms for a few minutes and had afterward seemed exhausted. The following day the child had seemed normal, a visit to the pediatrician had therefore not taken place. Past medical history consisted of a congenital diaphragmatic hernia. Thoracoscopic repair on the fifth day of life and follow-up q This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-No Derivative Works License, which permits non-commercial use, distribution, and reproduction in any medium, provided the original author and source are credited. * Corresponding author. Tel.: þ49 3834 867037; fax: þ49 3834 867038. E-mail address: [email protected] (C. Müller).
were carried out at another institution. According to the mother splenomegaly has been known since birth. The boy was treated as an in-patient because of airway infections at age 10 and 11 months. On admission the child seemed ill. The boy was apathetic but would not lie down. He kept himself in a sitting position with the head tilted toward the left shoulder. Skin turgor was decreased. Breath sounds were regular, but decreased on the base of the left chest as had been described before. Peripheral oxygen saturation was 96% and above at all times. The abdomen was distended but without abdominal guarding. The spleen was palpable. Bowel sounds were present, but not tinkly. Ultrasonographic examination showed splenomegaly. Because of the past medical history and the child’s respiratory distress we carried out an X-ray of the chest. This showed an elevated left diaphragm as well as pulmonary consolidation at the base of the left lung (Fig. 1). This picture was almost identical to another X-ray taken 7 months earlier. On further deterioration of the little boy’s condition repeated ultrasonographic examination showed massive herniation of bowel into the left thorax (Fig. 2). We therefore carried out emergency laparotomy. Intraoperatively we saw a posterolateral diaphragmatic defect with herniation of colon which was incarcerated (Fig. 4a/b). There was hemorrhagic splenic infarction; parts of the splenic vessels
2213-5766/$ e see front matter Ó 2013 The Authors. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.epsc.2013.05.020
414
C. Kujath et al. / J Ped Surg Case Reports 1 (2013) 413e415
Fig. 1. X-ray of the chest.
Fig. 3. Hemorrhagic splenic infarction.
were also herniated into the left thoracic cavity (Fig. 3). To reposition the colon into the abdomen careful enlargement of the diaphragmatic defect was necessary. The affected part of the colon was dilated and showed signs of decreased perfusion. Good recovery was observed intraoperatively. On close inspection parts of the mesentery could be found on the thoracic side of the diaphragm. They adhered to the diaphragm and could not be removed without risking further damage. Direct reconstruction of the diaphragm was carried out. Postoperatively the little patient recovered very well and could be discharged after eight days. The parents were informed repeatedly about the consequences of splenectomy. Due to a complete history of immunization further vaccination was not necessary. We started chemoprophylaxis to prevent OPSI (Overwhelming Post Splenectomy Infection Syndrome). The pathology report confirmed the diagnosis of hemorrhagic infarction of the spleen. 2. Discussion
Fig. 2. Ultrasonographic examination with enterothorax.
Patients with severe congenital diaphragmatic hernia (CDH) continue to have considerable mortality and morbidity [4]. These are mostly to due to underlying pulmonary hypoplasia and pulmonary hypertension [5]. Nevertheless, the risk of recurrence after surgical repair of the defect and its possible complications should not be neglected. Recurrence rates up to 15% for open surgery and up to 25% for minimal access surgery have been described [6,7]. In retrospect, it seems likely that recurrence in our case already took place several months earlier. On chest X-rays taken at the age of 11 months an elevated left diaphragm, mediastinal shift and
C. Kujath et al. / J Ped Surg Case Reports 1 (2013) 413e415
415
pulmonary consolidation at the base of the left lung were already described. These were not present on X-rays taken during the first and second month of life. The adherence of mesentery to the thoracic side of the diaphragm also suggests persistence of recurrence over several months. Apart from the two hospital stays described above the boy appeared healthy and showed excellent development. The parents therefore canceled an appointment for MR-imaging of the chest. However, loss of the spleen could have been prevented by further diagnostic imaging. There is a great need of standardized follow-up in CDH patients. Interdisciplinary follow-up programs have been shown to be beneficial [8]. Parents and pediatricians should be made aware of the possibility of asymptomatic recurrences. Pediatric surgeons need to discuss the possible diagnosis and treatment of these asymptomatic recurrences. 3. Conclusion The advantages of neonatal thoracoscopic CDH repair come with a price as the procedure has greater recurrence rates compared with open surgery. Parents and pediatricians should be well aware of the possibility of asymptomatic recurrences. Pediatric surgeons need to discuss the possible diagnosis and treatment of these asymptomatic recurrences. Conflict of interest No conflicts of interests. Sources of funding No funding them.
References
Fig. 4. a/b. Diaphragmatic defect with herniation of colon.
[1] Keijzer R, Puri P. Congenital diaphragmatic hernia. Semin Pediatr Surg 2010;19: 180e5. [2] Keijzer R, van de Ven C, Vlot J, Sloots C, Madern G, Tibboel D, et al. Thoracoscopic repair in congenital diaphragmatic hernia: patching is safe and reduces the recurrence rate. J Pediatr Surg 2010;45:953e7. [3] Tovar JA. Congenital diaphragmatic hernia. Orphanet J Rare Dis 2012;7:1. [4] Gander JW, Fisher JC, Gross ER, Reichstein AR, Cowles RA, Aspelund G, et al. Early recurrence of congenital diaphragmatic hernia is higher after thoracoscopic than open repair: a single institutional study. J Pediatr Surg 2011;46: 1303e8. [5] Lansdale N, Alam S, Losty PD, Jesudason EC. Neonatal endosurgical congenital diaphragmatic hernia repair: a systematic review and meta-analysis. Ann Surg 2010;252:20e6. [6] McHoney M, Giacomello L, Nah SA, De Coppi P, Kiely EM, Curry JI, et al. Thoracoscopic repair of congenital diaphragmatic hernia: intraoperative ventilation and recurrence. J Pediatr Surg 2010;45:355e9. [7] Vijfhuize S, Deden AC, Costerus SA, Sloots CE, Wijnen RM. Minimal access surgery for repair of congenital diaphragmatic hernia: is it advantageous? e an open review. Eur J Pediatr Surg 2012;22:364e73. [8] Chiu PPL, Ijsselstijn H. Morbidity and long-term follow-up in CDH patients. Eur J Pediatr Surg 2012;22:384e92.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com
Diaphragmatic hernia with splenic infarction e Recurrence after thoracoscopic repairq C. Kujath a, D. Großmann b, C. Müller b, * a b
Hannoversche Kinderheilanstalt, Department of Pediatric Surgery, Janusz-Korczak-Allee 12, 30173 Hannover, Germany University Medicine Greifswald, Department of Pediatric Surgery, Ferdinand-Sauerbruch-Str. 1, 17475 Greifswald, Germany
a r t i c l e i n f o
a b s t r a c t
Article history: Received 10 April 2013 Received in revised form 29 May 2013 Accepted 30 May 2013
We describe the case of a 17 month old boy with recurrence of congenital diaphragmatic hernia accompanied by splenic infarction and incarceration of colon. Primary surgery had been carried out thoracoscopically. On recurrence we found parts of the mesentery attached to the thoracic side of the diaphragm. Torsion of the spleen and consecutive hemorrhagic infarction was caused by the shift of splenic vessels into the thorax. Splenectomy was therefore made necessary. Ó 2013 The Authors. Published by Elsevier Inc. All rights reserved.
Key words: Diaphragmatic hernia Splenic torsion Recurrence
Congenital diaphragmatic hernia (CDH) occurs in one in 2000e3000 newborns. It can be associated with pulmonary hypoplasia and persistent pulmonary hypertension [1,2]. In 70e80% of cases CDH consists of a posterolateral defect on the left side of the diaphragm [3]. 1. Case report A 17 months old boy was presented to the emergency department with vomiting. For two days he had not drunk or eaten anything. Onset of illness was sudden when the little boy had shown signs of abdominal cramps over a short period two days earlier. Five days earlier he had shown similar symptoms of abdominal spasms for a few minutes and had afterward seemed exhausted. The following day the child had seemed normal, a visit to the pediatrician had therefore not taken place. Past medical history consisted of a congenital diaphragmatic hernia. Thoracoscopic repair on the fifth day of life and follow-up q This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-No Derivative Works License, which permits non-commercial use, distribution, and reproduction in any medium, provided the original author and source are credited. * Corresponding author. Tel.: þ49 3834 867037; fax: þ49 3834 867038. E-mail address: [email protected] (C. Müller).
were carried out at another institution. According to the mother splenomegaly has been known since birth. The boy was treated as an in-patient because of airway infections at age 10 and 11 months. On admission the child seemed ill. The boy was apathetic but would not lie down. He kept himself in a sitting position with the head tilted toward the left shoulder. Skin turgor was decreased. Breath sounds were regular, but decreased on the base of the left chest as had been described before. Peripheral oxygen saturation was 96% and above at all times. The abdomen was distended but without abdominal guarding. The spleen was palpable. Bowel sounds were present, but not tinkly. Ultrasonographic examination showed splenomegaly. Because of the past medical history and the child’s respiratory distress we carried out an X-ray of the chest. This showed an elevated left diaphragm as well as pulmonary consolidation at the base of the left lung (Fig. 1). This picture was almost identical to another X-ray taken 7 months earlier. On further deterioration of the little boy’s condition repeated ultrasonographic examination showed massive herniation of bowel into the left thorax (Fig. 2). We therefore carried out emergency laparotomy. Intraoperatively we saw a posterolateral diaphragmatic defect with herniation of colon which was incarcerated (Fig. 4a/b). There was hemorrhagic splenic infarction; parts of the splenic vessels
2213-5766/$ e see front matter Ó 2013 The Authors. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.epsc.2013.05.020
414
C. Kujath et al. / J Ped Surg Case Reports 1 (2013) 413e415
Fig. 1. X-ray of the chest.
Fig. 3. Hemorrhagic splenic infarction.
were also herniated into the left thoracic cavity (Fig. 3). To reposition the colon into the abdomen careful enlargement of the diaphragmatic defect was necessary. The affected part of the colon was dilated and showed signs of decreased perfusion. Good recovery was observed intraoperatively. On close inspection parts of the mesentery could be found on the thoracic side of the diaphragm. They adhered to the diaphragm and could not be removed without risking further damage. Direct reconstruction of the diaphragm was carried out. Postoperatively the little patient recovered very well and could be discharged after eight days. The parents were informed repeatedly about the consequences of splenectomy. Due to a complete history of immunization further vaccination was not necessary. We started chemoprophylaxis to prevent OPSI (Overwhelming Post Splenectomy Infection Syndrome). The pathology report confirmed the diagnosis of hemorrhagic infarction of the spleen. 2. Discussion
Fig. 2. Ultrasonographic examination with enterothorax.
Patients with severe congenital diaphragmatic hernia (CDH) continue to have considerable mortality and morbidity [4]. These are mostly to due to underlying pulmonary hypoplasia and pulmonary hypertension [5]. Nevertheless, the risk of recurrence after surgical repair of the defect and its possible complications should not be neglected. Recurrence rates up to 15% for open surgery and up to 25% for minimal access surgery have been described [6,7]. In retrospect, it seems likely that recurrence in our case already took place several months earlier. On chest X-rays taken at the age of 11 months an elevated left diaphragm, mediastinal shift and
C. Kujath et al. / J Ped Surg Case Reports 1 (2013) 413e415
415
pulmonary consolidation at the base of the left lung were already described. These were not present on X-rays taken during the first and second month of life. The adherence of mesentery to the thoracic side of the diaphragm also suggests persistence of recurrence over several months. Apart from the two hospital stays described above the boy appeared healthy and showed excellent development. The parents therefore canceled an appointment for MR-imaging of the chest. However, loss of the spleen could have been prevented by further diagnostic imaging. There is a great need of standardized follow-up in CDH patients. Interdisciplinary follow-up programs have been shown to be beneficial [8]. Parents and pediatricians should be made aware of the possibility of asymptomatic recurrences. Pediatric surgeons need to discuss the possible diagnosis and treatment of these asymptomatic recurrences. 3. Conclusion The advantages of neonatal thoracoscopic CDH repair come with a price as the procedure has greater recurrence rates compared with open surgery. Parents and pediatricians should be well aware of the possibility of asymptomatic recurrences. Pediatric surgeons need to discuss the possible diagnosis and treatment of these asymptomatic recurrences. Conflict of interest No conflicts of interests. Sources of funding No funding them.
References
Fig. 4. a/b. Diaphragmatic defect with herniation of colon.
[1] Keijzer R, Puri P. Congenital diaphragmatic hernia. Semin Pediatr Surg 2010;19: 180e5. [2] Keijzer R, van de Ven C, Vlot J, Sloots C, Madern G, Tibboel D, et al. Thoracoscopic repair in congenital diaphragmatic hernia: patching is safe and reduces the recurrence rate. J Pediatr Surg 2010;45:953e7. [3] Tovar JA. Congenital diaphragmatic hernia. Orphanet J Rare Dis 2012;7:1. [4] Gander JW, Fisher JC, Gross ER, Reichstein AR, Cowles RA, Aspelund G, et al. Early recurrence of congenital diaphragmatic hernia is higher after thoracoscopic than open repair: a single institutional study. J Pediatr Surg 2011;46: 1303e8. [5] Lansdale N, Alam S, Losty PD, Jesudason EC. Neonatal endosurgical congenital diaphragmatic hernia repair: a systematic review and meta-analysis. Ann Surg 2010;252:20e6. [6] McHoney M, Giacomello L, Nah SA, De Coppi P, Kiely EM, Curry JI, et al. Thoracoscopic repair of congenital diaphragmatic hernia: intraoperative ventilation and recurrence. J Pediatr Surg 2010;45:355e9. [7] Vijfhuize S, Deden AC, Costerus SA, Sloots CE, Wijnen RM. Minimal access surgery for repair of congenital diaphragmatic hernia: is it advantageous? e an open review. Eur J Pediatr Surg 2012;22:364e73. [8] Chiu PPL, Ijsselstijn H. Morbidity and long-term follow-up in CDH patients. Eur J Pediatr Surg 2012;22:384e92.