- Email: [email protected]
Peritoneal encapsulation in childhood
Peritoneal Encapsulation in Childhood Case Report, Embryologic Analysis, and Review of Literature
Joel Sayfan, MD, Zerifin, Israel Yehuda G. Adam, MD, FACS, Zerifin, Israel Renate Reif, MD, Zerifin, Israel
Peritoneal encapsulation is a very rare congenital malformation. Only seven accidentally diagnosed cases have been reported in the world literature, all of them in adults [I-5]. The subject of this report is a child in whom acute mechanical intestinal obstruction developed due to peritoneal encapsulation. Case Report A 12 year old Caucasian girl was admitted in January 1978 to the emergency ward of our hospital because of severe crampy abdominal pain, vomiting, and constipation of 24 hours’ duration. She had had intermittent abdominal discomfort, mostly epigastric, for 3 months before admission. Because of these complaints her family physician suspected a duodenal ulcer, and shortly before the recent episode a contrast study of the stomach and duodenum had been carried out with negative results. Except for the usual childhood illnesses, her past medical history was negative. Physical examination on admission revealed a well developed girl in acute distress. Her oral and rectal temperature was normal. Blood pressure was 130/90 mmHg and the pulse 95/min. The positive findings were confined to the abdomen, which was diffusely tender and moderately distended, and hyperactive metal-tinged bowel sounds were present. On rectal examination a hard, tender, lobulated, and fixed mass was felt in the midline. The mass was not palpable on abdominal examination. Blood glucose, urea nitrogen, serum electrolytes, and urine were normal. Hemoglobin was 12 g/100 ml and the white cell count 14,800. X-ray films of the chest were negative. Flat and upright film of the abdomen revealed markedly distended loops of small bowel with an obstructive pattern. From the Departments of Surgery ‘A’ and Pathology, Asaf Harofe Hospital, Tel-Aviv University Medical School, Zerifin, Israel. Reprint requests should be addressed to Joel Sayfan. MD, Department of Surgery ‘A,’ Asaf Harofe Hospital, Zerifin, Israel.
Volume 139, November 1979
A diagnosis of acute mechanical small bowel obstruction was made. After gastric decompression and a short period of rehydration, due to the presence of diffuse abdominal tenderness, laparotomy was decided on. Under general anesthesia the abdomen was entered through a midline incision. A small amount of free yellow serous fluid was found in the peritoneal cavity. The small bowel was markedly dilated to about 40 cm from the ileocecal valve. At that point a 15 X 20 X 15 cm mass, covered by a dense whitish membrane, was encountered. On incision of this capsule, a small amount of blood-tinged fluid escaped. Within the sac a lobulated mass consisting of several loops of matted ileum with spotty discoloration was present. The whole mass was fixed to the parietal peritoneum of the posterior abdominal wall. The last 10 cm of terminal ileum, distal to the capsule, was collapsed. The whole mass and its enveloping membrane was totally resected, and a two layer end-to-end enteroenterostomy was carried out to establish intestinal continuity. The postoperative course was uneventful and the patient was discharged on the seventh postoperative day. On follow-up examinations the patient was found to be totally asymptomatic. Pathology
Gross examination of the specimen revealed a conglomerate of small intestine about 25 cm in length, covered by a whitish, dense, glistening membrane. When this fibrous capsule was longitudinally opened, distended convoluted loops of small gut, compressed together under considerable tension, filled the sac-like structure. Due to the disparity between the size of the sac and the amount of bowel it contained, the bowel wall was found to be arranged in multiple folds. The length of the excised intestine was about 55 cm. On histologic examination the intestinal wall was found to be covered with a thick fibrous membrane instead of the normal serosa. This membrane was
725
Sayfan et al
Figure 1. External aspect of the resected specimen showing the thick covering capsule.
compatible with thickened peritoneum. The mesothelial cells were not prominent (Figures l-3). Comments
Peritoneal encapsulation was first described by Cleland [I] in 1868. Since his original report, an additional six cases have been described [2-51. The embryologic explanation of this rare congenital anomaly is not entirely clear. As a part of the normal developmental pattern, in the seventh gestational week the gastrointestinal trace begins its rotation and the extracelomic midgut withdraws to the abdominal cavity. This withdrawal is completed in the 10th week of embryonic life. Variations of this normal mechanism are probably responsible for the accessory peritoneal membrane resulting in encapsulation. One explanation of the anomaly is that
Figure 2. After kn@tudinal opening, the fokled intestinal wall is seen.
malrotation of the small gut or a part of it with displacement and subsequent fusion of its dorsal mesentery, which originally forms the transverse mesocolon, to the ascending and descending colon and the posterior peritoneum is responsible for this phenomenon [4]. The case presented herein speaks against this theory because of the lack of fixation of the accessory peritoneal sac to the colon and the presence of intact transverse mesocolon. Another tentative explanation, described by Estrada [6], is that the abnormal peritoneal capsule is a persisting part of the yolk sac membrane drawn into the abdominal cavity during the intestinal return to the peritoneal cavity proper. This theory better explains the findings in our case. Peritoneal encapsulation should not be confused with chronic encapsulating peritonitis, which has an
Figure 3. Section through the intestinal wal/ showsthe mucosalsurface on the right and the thick f/brous membrane instead of the norma/ servsa on the far Mt. (Magn&atkn X20, reduced 14 per cent.)
726
The American Journal of Surgery
Peritoneal Encapsulation
entirely different gross and microscopic picture. Despite its congenital nature, all the cases of peritoneal encapsulation reported so far have been described in adults. It should be further emphasized that all of the reported cases of peritoneal encapsulation were found accidentally, during unrelated surgery such as cholecystectomy or laparotomy for trauma, or at autopsy. In a few cases, a detailed retrospective history disclosed long-standing nonspecific abdominal discomfort, which might or might not have been related to the accidentally discovered encapsulation. Our case is the first one described in childhood. In contrast to the known instances of peritoneal encapsulation, the malformation in our patient caused mechanical small bowel obstruction requiring emergency surgery. It seems that a longstanding incomplete intestinal obstruction long preceded the acute episode and expressed itself by intermittent abdominal pain and occasional vomiting. These symptoms were interpreted as manifestations of a questionable duodenal ulcer. Although peritoneal encapsulation is an extremely rare congenital anomaly that may cause acute abdominal sympt,oms, awareness of this entity could
Volume 139. November 1979
in Childhood
help the surgeon to recognize it intraoperatively treat it properly.
and
Summary
The first reported case of peritoneal encapsulation causing mechanical small bowel obstruction in a child is described. So far, only seven asymptomatic adult patients with this anomaly have been reported on. The embryogenetic basis is discussed. Methods of operative recognition and surgical management are suggested. References 1. Cleland J: Abnormal peritoneal membrane. J Anat physiol2: 201, 1868 (quoted by Lickley and Cameron). 2. Lickley JD. Cameron J: Abnormal peritoneal membrane. J Anaf Physiol41: 88, 1907. 3. Papez JW: A rare intestinal anomaly of embryonic origin. Anat Ret 53: 197, 1932. 4. Thorlakson PHT, Monie IW, Thorlakson TK: Anomalous peritoneal encapsulation of the small intestine. f3r J Surg 40: 490. 1953. 5. Lewin K, McCarthy LJ: Peritoneal encapsulation of small intestine. Gasfroenferology 59: 2, 1970. 6. Estrada RL: Anomalies of Intestinal Rotations and Fixation. Springfield, IL, Charles C Thomas, 1958, p 24.
727
Joel Sayfan, MD, Zerifin, Israel Yehuda G. Adam, MD, FACS, Zerifin, Israel Renate Reif, MD, Zerifin, Israel
Peritoneal encapsulation is a very rare congenital malformation. Only seven accidentally diagnosed cases have been reported in the world literature, all of them in adults [I-5]. The subject of this report is a child in whom acute mechanical intestinal obstruction developed due to peritoneal encapsulation. Case Report A 12 year old Caucasian girl was admitted in January 1978 to the emergency ward of our hospital because of severe crampy abdominal pain, vomiting, and constipation of 24 hours’ duration. She had had intermittent abdominal discomfort, mostly epigastric, for 3 months before admission. Because of these complaints her family physician suspected a duodenal ulcer, and shortly before the recent episode a contrast study of the stomach and duodenum had been carried out with negative results. Except for the usual childhood illnesses, her past medical history was negative. Physical examination on admission revealed a well developed girl in acute distress. Her oral and rectal temperature was normal. Blood pressure was 130/90 mmHg and the pulse 95/min. The positive findings were confined to the abdomen, which was diffusely tender and moderately distended, and hyperactive metal-tinged bowel sounds were present. On rectal examination a hard, tender, lobulated, and fixed mass was felt in the midline. The mass was not palpable on abdominal examination. Blood glucose, urea nitrogen, serum electrolytes, and urine were normal. Hemoglobin was 12 g/100 ml and the white cell count 14,800. X-ray films of the chest were negative. Flat and upright film of the abdomen revealed markedly distended loops of small bowel with an obstructive pattern. From the Departments of Surgery ‘A’ and Pathology, Asaf Harofe Hospital, Tel-Aviv University Medical School, Zerifin, Israel. Reprint requests should be addressed to Joel Sayfan. MD, Department of Surgery ‘A,’ Asaf Harofe Hospital, Zerifin, Israel.
Volume 139, November 1979
A diagnosis of acute mechanical small bowel obstruction was made. After gastric decompression and a short period of rehydration, due to the presence of diffuse abdominal tenderness, laparotomy was decided on. Under general anesthesia the abdomen was entered through a midline incision. A small amount of free yellow serous fluid was found in the peritoneal cavity. The small bowel was markedly dilated to about 40 cm from the ileocecal valve. At that point a 15 X 20 X 15 cm mass, covered by a dense whitish membrane, was encountered. On incision of this capsule, a small amount of blood-tinged fluid escaped. Within the sac a lobulated mass consisting of several loops of matted ileum with spotty discoloration was present. The whole mass was fixed to the parietal peritoneum of the posterior abdominal wall. The last 10 cm of terminal ileum, distal to the capsule, was collapsed. The whole mass and its enveloping membrane was totally resected, and a two layer end-to-end enteroenterostomy was carried out to establish intestinal continuity. The postoperative course was uneventful and the patient was discharged on the seventh postoperative day. On follow-up examinations the patient was found to be totally asymptomatic. Pathology
Gross examination of the specimen revealed a conglomerate of small intestine about 25 cm in length, covered by a whitish, dense, glistening membrane. When this fibrous capsule was longitudinally opened, distended convoluted loops of small gut, compressed together under considerable tension, filled the sac-like structure. Due to the disparity between the size of the sac and the amount of bowel it contained, the bowel wall was found to be arranged in multiple folds. The length of the excised intestine was about 55 cm. On histologic examination the intestinal wall was found to be covered with a thick fibrous membrane instead of the normal serosa. This membrane was
725
Sayfan et al
Figure 1. External aspect of the resected specimen showing the thick covering capsule.
compatible with thickened peritoneum. The mesothelial cells were not prominent (Figures l-3). Comments
Peritoneal encapsulation was first described by Cleland [I] in 1868. Since his original report, an additional six cases have been described [2-51. The embryologic explanation of this rare congenital anomaly is not entirely clear. As a part of the normal developmental pattern, in the seventh gestational week the gastrointestinal trace begins its rotation and the extracelomic midgut withdraws to the abdominal cavity. This withdrawal is completed in the 10th week of embryonic life. Variations of this normal mechanism are probably responsible for the accessory peritoneal membrane resulting in encapsulation. One explanation of the anomaly is that
Figure 2. After kn@tudinal opening, the fokled intestinal wall is seen.
malrotation of the small gut or a part of it with displacement and subsequent fusion of its dorsal mesentery, which originally forms the transverse mesocolon, to the ascending and descending colon and the posterior peritoneum is responsible for this phenomenon [4]. The case presented herein speaks against this theory because of the lack of fixation of the accessory peritoneal sac to the colon and the presence of intact transverse mesocolon. Another tentative explanation, described by Estrada [6], is that the abnormal peritoneal capsule is a persisting part of the yolk sac membrane drawn into the abdominal cavity during the intestinal return to the peritoneal cavity proper. This theory better explains the findings in our case. Peritoneal encapsulation should not be confused with chronic encapsulating peritonitis, which has an
Figure 3. Section through the intestinal wal/ showsthe mucosalsurface on the right and the thick f/brous membrane instead of the norma/ servsa on the far Mt. (Magn&atkn X20, reduced 14 per cent.)
726
The American Journal of Surgery
Peritoneal Encapsulation
entirely different gross and microscopic picture. Despite its congenital nature, all the cases of peritoneal encapsulation reported so far have been described in adults. It should be further emphasized that all of the reported cases of peritoneal encapsulation were found accidentally, during unrelated surgery such as cholecystectomy or laparotomy for trauma, or at autopsy. In a few cases, a detailed retrospective history disclosed long-standing nonspecific abdominal discomfort, which might or might not have been related to the accidentally discovered encapsulation. Our case is the first one described in childhood. In contrast to the known instances of peritoneal encapsulation, the malformation in our patient caused mechanical small bowel obstruction requiring emergency surgery. It seems that a longstanding incomplete intestinal obstruction long preceded the acute episode and expressed itself by intermittent abdominal pain and occasional vomiting. These symptoms were interpreted as manifestations of a questionable duodenal ulcer. Although peritoneal encapsulation is an extremely rare congenital anomaly that may cause acute abdominal sympt,oms, awareness of this entity could
Volume 139. November 1979
in Childhood
help the surgeon to recognize it intraoperatively treat it properly.
and
Summary
The first reported case of peritoneal encapsulation causing mechanical small bowel obstruction in a child is described. So far, only seven asymptomatic adult patients with this anomaly have been reported on. The embryogenetic basis is discussed. Methods of operative recognition and surgical management are suggested. References 1. Cleland J: Abnormal peritoneal membrane. J Anat physiol2: 201, 1868 (quoted by Lickley and Cameron). 2. Lickley JD. Cameron J: Abnormal peritoneal membrane. J Anaf Physiol41: 88, 1907. 3. Papez JW: A rare intestinal anomaly of embryonic origin. Anat Ret 53: 197, 1932. 4. Thorlakson PHT, Monie IW, Thorlakson TK: Anomalous peritoneal encapsulation of the small intestine. f3r J Surg 40: 490. 1953. 5. Lewin K, McCarthy LJ: Peritoneal encapsulation of small intestine. Gasfroenferology 59: 2, 1970. 6. Estrada RL: Anomalies of Intestinal Rotations and Fixation. Springfield, IL, Charles C Thomas, 1958, p 24.
727